Abdulhafez Selim, MD, PhD

A progressively growing lesion on the left knee prompted a 35-year-old woman to visit our clinic. She reported that about 3 months earlier, she had developed a small ulceration on the knee following a fall. With local wound care, the ulceration healed with a scar. The scar, however, continued to grow and she developed distinct papules outside the original scar.

FIGURE
Scar develops into a dusky plaque

The scar subsequently became raised with violaceous discoloration. The patient reported having no history of excessive scarring or keloid forming after skin surgery or trauma. There was neither a personal nor family history of inflammatory or infectious granulomatous diseases.

On physical examination, there was an erythematous to dusky plaque with well-defined irregular borders. There were also discrete papules on the anterior and medial aspect of the knee. The plaque measured approximately 2.8 cm by 3.8 cm. There were no tender nodules on the shins, nor was lymphadenopathy present. A routine chest x-ray was normal.

To support our clinical diagnosis, we took a 4-mm punch biopsy from the center of the plaque. The histologic examination revealed changes in the dermis termed noncaseating “naked” granulomas.

What is your diagnosis?
How would you manage this condition?

Diagnosis: Scar sarcoidosis

Sarcoidosis is a systemic granulomatous disease that may affect any organ system, and therefore may present with various clinical manifestations.¹ Sarcoidosis can be an incidental finding on chest x-ray or be discovered in patients that present with respiratory or constitutional symptoms.²

Cutaneous sarcoidosis occurs in up to one third of patients with systemic sarcoidosis.² The classic skin lesions are “erythema nodosum”—an acute, nodular, erythematous eruption that usually is limited to the shins, and “lupus pernio”—red-to-purple or violaceous indurated nodules affecting the nose, cheeks, ears, and lips. There are other uncommon skin presentations of sarcoidosis ranging from scattered papules and annular lesions to erythrodermic skin manifestations.³

In scar sarcoidosis, there is spontaneous development of livid or reddish-brown plaques on scars that were previously atrophic for the most part. Scar sarcoidosis may be caused by:^4-6

• venipuncture
• tuberculin skin tests
• herpes zoster
• tattoos
• cosmetic fillers such as hyaluronic acid injection.

Infection and other factors may be at work

Although the precise cause of sarcoidosis remains unknown, various infectious, noninfectious, environmental, and genetic factors may be at work. Researchers have theorized that immune dysregulation may be involved. Contact with a persistent antigen that is poorly cleared by the immune system may lead to T lymphocytes and mononuclear phagocytes accumulating in the granulomas of sarcoidosis.⁷ Researchers have proposed that inoculation of foreign matter from minor trauma may be one type of pathogenic mechanism in cutaneous sarcoid.⁸

Granuloma annulare is part of the differential

A wide range of diseases comprise the differential diagnosis of sarcoidosis. These diseases include:⁹

• Granuloma annulare. It is also a granulomatous skin disease, but it appears as single or multiple rings.
• Rheumatoid nodules. These usually appear in the context of a diagnosis of rheumatoid arthritis.
• Granulomatous mycosis fungoides. This type of cutaneous lymphoma has many clinical forms, including granuloma formation.
• Syringoma. On inspection, you’ll see small, firm adnexal benign tumors that usually appear around the upper cheeks and lower eyelids
• Xanthelasma. These are benign, yellow macules, papules, or plaques that tend to appear on the eyelids. Patients with xanthelasma often have a lipid disorder.
• Lichen planus. This is a very pruritic skin involvement with pink to violaceous papules and plaques. It may present in different locations, but the most common areas are the wrists and ankles.
• Granulomatous rosacea. This is a variant of rosacea characterized by uniform papules on the face.

Clinical findings, biopsy clinch the diagnosis

The diagnosis of sarcoidosis is made by a combination of clinical and histologic findings.¹

• Clinical findings. Cutaneous involvement is either “specific” or “nonspecific.”
• With specific cutaneous involvement, which our patient had, you’ll see typical noncaseating granulomas, with no evidence of infection or a foreign body. It may be disfiguring, but it’s almost always nontender and it is rarely ulcerative.
• With nonspecific cutaneous involvement, you’ll see erythema nodosum lesions, especially on the legs. The serum angiotensin-converting enzyme (ACE) level is elevated in many of these patients.

Histologic findings. Skin biopsy demonstrating noncaseating granulomas provides definitive evidence of skin involvement. Typical sarcoid lesions are characterized by circumscribed granulomas of epithelioid cells with little or no necrosis. (The term “naked” granuloma refers to the absence, or small number, of surrounding lymphocytes.)

Other granulomatous diseases, such as berylliosis and tuberculosis, must be excluded since they often present the same way as scar sarcoidosis.⁷

Steroids control symptoms, slow disease progression

Topical, intralesional, and systemic corticosteroids are used to treat scar sarcoidosis, as are systemic medications such as chloroquine¹⁰ and allopurinol.¹¹ Corticosteroids (local and systemic) are effective in controlling all sarcoid symptoms; they also slow disease progression.¹

For localized skin involvement, intralesional corticosteroids are typically more effective than topical steroids. Systemic corticosteroids are reserved for widespread, progressive lesions or those that impair function.^1,12,13 A starting dose of 1 mg/kg of prednisone is appropriate.

In general, the prognosis of cutaneous sarcoidosis is good.² The course is variable, ranging from self-limited acute episodes to a chronic debilitating disease that may result in death.² Spontaneous remissions occur in nearly two thirds of patients, but 10% to 30% have a more chronic or progressive course.^1,2,13

Our patient responds to treatment

Our patient declined intralesional corticosteroid injections, so we started her on potent topical corticosteroid tapes (Cordran). She had significant improvement 6 weeks later.

Correspondence
Amor Khachemoune, MD, CWS, 450 Clarkson Avenue Box 46, Brooklyn, NY 11203; amorkh@pol.net

A progressively growing lesion on the left knee prompted a 35-year-old woman to visit our clinic. She reported that about 3 months earlier, she had developed a small ulceration on the knee following a fall. With local wound care, the ulceration healed with a scar. The scar, however, continued to grow and she developed distinct papules outside the original scar.

FIGURE
Scar develops into a dusky plaque

The scar subsequently became raised with violaceous discoloration. The patient reported having no history of excessive scarring or keloid forming after skin surgery or trauma. There was neither a personal nor family history of inflammatory or infectious granulomatous diseases.

On physical examination, there was an erythematous to dusky plaque with well-defined irregular borders. There were also discrete papules on the anterior and medial aspect of the knee. The plaque measured approximately 2.8 cm by 3.8 cm. There were no tender nodules on the shins, nor was lymphadenopathy present. A routine chest x-ray was normal.

To support our clinical diagnosis, we took a 4-mm punch biopsy from the center of the plaque. The histologic examination revealed changes in the dermis termed noncaseating “naked” granulomas.

What is your diagnosis?
How would you manage this condition?

Diagnosis: Scar sarcoidosis

Sarcoidosis is a systemic granulomatous disease that may affect any organ system, and therefore may present with various clinical manifestations.¹ Sarcoidosis can be an incidental finding on chest x-ray or be discovered in patients that present with respiratory or constitutional symptoms.²

Cutaneous sarcoidosis occurs in up to one third of patients with systemic sarcoidosis.² The classic skin lesions are “erythema nodosum”—an acute, nodular, erythematous eruption that usually is limited to the shins, and “lupus pernio”—red-to-purple or violaceous indurated nodules affecting the nose, cheeks, ears, and lips. There are other uncommon skin presentations of sarcoidosis ranging from scattered papules and annular lesions to erythrodermic skin manifestations.³

In scar sarcoidosis, there is spontaneous development of livid or reddish-brown plaques on scars that were previously atrophic for the most part. Scar sarcoidosis may be caused by:^4-6

• venipuncture
• tuberculin skin tests
• herpes zoster
• tattoos
• cosmetic fillers such as hyaluronic acid injection.

Infection and other factors may be at work

Although the precise cause of sarcoidosis remains unknown, various infectious, noninfectious, environmental, and genetic factors may be at work. Researchers have theorized that immune dysregulation may be involved. Contact with a persistent antigen that is poorly cleared by the immune system may lead to T lymphocytes and mononuclear phagocytes accumulating in the granulomas of sarcoidosis.⁷ Researchers have proposed that inoculation of foreign matter from minor trauma may be one type of pathogenic mechanism in cutaneous sarcoid.⁸

Granuloma annulare is part of the differential

A wide range of diseases comprise the differential diagnosis of sarcoidosis. These diseases include:⁹

• Granuloma annulare. It is also a granulomatous skin disease, but it appears as single or multiple rings.
• Rheumatoid nodules. These usually appear in the context of a diagnosis of rheumatoid arthritis.
• Granulomatous mycosis fungoides. This type of cutaneous lymphoma has many clinical forms, including granuloma formation.
• Syringoma. On inspection, you’ll see small, firm adnexal benign tumors that usually appear around the upper cheeks and lower eyelids
• Xanthelasma. These are benign, yellow macules, papules, or plaques that tend to appear on the eyelids. Patients with xanthelasma often have a lipid disorder.
• Lichen planus. This is a very pruritic skin involvement with pink to violaceous papules and plaques. It may present in different locations, but the most common areas are the wrists and ankles.
• Granulomatous rosacea. This is a variant of rosacea characterized by uniform papules on the face.

Clinical findings, biopsy clinch the diagnosis

The diagnosis of sarcoidosis is made by a combination of clinical and histologic findings.¹

• Clinical findings. Cutaneous involvement is either “specific” or “nonspecific.”
• With specific cutaneous involvement, which our patient had, you’ll see typical noncaseating granulomas, with no evidence of infection or a foreign body. It may be disfiguring, but it’s almost always nontender and it is rarely ulcerative.
• With nonspecific cutaneous involvement, you’ll see erythema nodosum lesions, especially on the legs. The serum angiotensin-converting enzyme (ACE) level is elevated in many of these patients.

Histologic findings. Skin biopsy demonstrating noncaseating granulomas provides definitive evidence of skin involvement. Typical sarcoid lesions are characterized by circumscribed granulomas of epithelioid cells with little or no necrosis. (The term “naked” granuloma refers to the absence, or small number, of surrounding lymphocytes.)

Other granulomatous diseases, such as berylliosis and tuberculosis, must be excluded since they often present the same way as scar sarcoidosis.⁷

Steroids control symptoms, slow disease progression

Topical, intralesional, and systemic corticosteroids are used to treat scar sarcoidosis, as are systemic medications such as chloroquine¹⁰ and allopurinol.¹¹ Corticosteroids (local and systemic) are effective in controlling all sarcoid symptoms; they also slow disease progression.¹

For localized skin involvement, intralesional corticosteroids are typically more effective than topical steroids. Systemic corticosteroids are reserved for widespread, progressive lesions or those that impair function.^1,12,13 A starting dose of 1 mg/kg of prednisone is appropriate.

In general, the prognosis of cutaneous sarcoidosis is good.² The course is variable, ranging from self-limited acute episodes to a chronic debilitating disease that may result in death.² Spontaneous remissions occur in nearly two thirds of patients, but 10% to 30% have a more chronic or progressive course.^1,2,13

Our patient responds to treatment

Our patient declined intralesional corticosteroid injections, so we started her on potent topical corticosteroid tapes (Cordran). She had significant improvement 6 weeks later.

Correspondence
Amor Khachemoune, MD, CWS, 450 Clarkson Avenue Box 46, Brooklyn, NY 11203; amorkh@pol.net

A progressively growing lesion on the left knee prompted a 35-year-old woman to visit our clinic. She reported that about 3 months earlier, she had developed a small ulceration on the knee following a fall. With local wound care, the ulceration healed with a scar. The scar, however, continued to grow and she developed distinct papules outside the original scar.

FIGURE
Scar develops into a dusky plaque

The scar subsequently became raised with violaceous discoloration. The patient reported having no history of excessive scarring or keloid forming after skin surgery or trauma. There was neither a personal nor family history of inflammatory or infectious granulomatous diseases.

On physical examination, there was an erythematous to dusky plaque with well-defined irregular borders. There were also discrete papules on the anterior and medial aspect of the knee. The plaque measured approximately 2.8 cm by 3.8 cm. There were no tender nodules on the shins, nor was lymphadenopathy present. A routine chest x-ray was normal.

To support our clinical diagnosis, we took a 4-mm punch biopsy from the center of the plaque. The histologic examination revealed changes in the dermis termed noncaseating “naked” granulomas.

What is your diagnosis?
How would you manage this condition?

Diagnosis: Scar sarcoidosis

Sarcoidosis is a systemic granulomatous disease that may affect any organ system, and therefore may present with various clinical manifestations.¹ Sarcoidosis can be an incidental finding on chest x-ray or be discovered in patients that present with respiratory or constitutional symptoms.²

Cutaneous sarcoidosis occurs in up to one third of patients with systemic sarcoidosis.² The classic skin lesions are “erythema nodosum”—an acute, nodular, erythematous eruption that usually is limited to the shins, and “lupus pernio”—red-to-purple or violaceous indurated nodules affecting the nose, cheeks, ears, and lips. There are other uncommon skin presentations of sarcoidosis ranging from scattered papules and annular lesions to erythrodermic skin manifestations.³

In scar sarcoidosis, there is spontaneous development of livid or reddish-brown plaques on scars that were previously atrophic for the most part. Scar sarcoidosis may be caused by:^4-6

• venipuncture
• tuberculin skin tests
• herpes zoster
• tattoos
• cosmetic fillers such as hyaluronic acid injection.

Infection and other factors may be at work

Although the precise cause of sarcoidosis remains unknown, various infectious, noninfectious, environmental, and genetic factors may be at work. Researchers have theorized that immune dysregulation may be involved. Contact with a persistent antigen that is poorly cleared by the immune system may lead to T lymphocytes and mononuclear phagocytes accumulating in the granulomas of sarcoidosis.⁷ Researchers have proposed that inoculation of foreign matter from minor trauma may be one type of pathogenic mechanism in cutaneous sarcoid.⁸

Granuloma annulare is part of the differential

A wide range of diseases comprise the differential diagnosis of sarcoidosis. These diseases include:⁹

• Granuloma annulare. It is also a granulomatous skin disease, but it appears as single or multiple rings.
• Rheumatoid nodules. These usually appear in the context of a diagnosis of rheumatoid arthritis.
• Granulomatous mycosis fungoides. This type of cutaneous lymphoma has many clinical forms, including granuloma formation.
• Syringoma. On inspection, you’ll see small, firm adnexal benign tumors that usually appear around the upper cheeks and lower eyelids
• Xanthelasma. These are benign, yellow macules, papules, or plaques that tend to appear on the eyelids. Patients with xanthelasma often have a lipid disorder.
• Lichen planus. This is a very pruritic skin involvement with pink to violaceous papules and plaques. It may present in different locations, but the most common areas are the wrists and ankles.
• Granulomatous rosacea. This is a variant of rosacea characterized by uniform papules on the face.

Clinical findings, biopsy clinch the diagnosis

The diagnosis of sarcoidosis is made by a combination of clinical and histologic findings.¹

• Clinical findings. Cutaneous involvement is either “specific” or “nonspecific.”
• With specific cutaneous involvement, which our patient had, you’ll see typical noncaseating granulomas, with no evidence of infection or a foreign body. It may be disfiguring, but it’s almost always nontender and it is rarely ulcerative.
• With nonspecific cutaneous involvement, you’ll see erythema nodosum lesions, especially on the legs. The serum angiotensin-converting enzyme (ACE) level is elevated in many of these patients.

Histologic findings. Skin biopsy demonstrating noncaseating granulomas provides definitive evidence of skin involvement. Typical sarcoid lesions are characterized by circumscribed granulomas of epithelioid cells with little or no necrosis. (The term “naked” granuloma refers to the absence, or small number, of surrounding lymphocytes.)

Other granulomatous diseases, such as berylliosis and tuberculosis, must be excluded since they often present the same way as scar sarcoidosis.⁷

Steroids control symptoms, slow disease progression

Topical, intralesional, and systemic corticosteroids are used to treat scar sarcoidosis, as are systemic medications such as chloroquine¹⁰ and allopurinol.¹¹ Corticosteroids (local and systemic) are effective in controlling all sarcoid symptoms; they also slow disease progression.¹

For localized skin involvement, intralesional corticosteroids are typically more effective than topical steroids. Systemic corticosteroids are reserved for widespread, progressive lesions or those that impair function.^1,12,13 A starting dose of 1 mg/kg of prednisone is appropriate.

In general, the prognosis of cutaneous sarcoidosis is good.² The course is variable, ranging from self-limited acute episodes to a chronic debilitating disease that may result in death.² Spontaneous remissions occur in nearly two thirds of patients, but 10% to 30% have a more chronic or progressive course.^1,2,13

Our patient responds to treatment

Our patient declined intralesional corticosteroid injections, so we started her on potent topical corticosteroid tapes (Cordran). She had significant improvement 6 weeks later.

Correspondence
Amor Khachemoune, MD, CWS, 450 Clarkson Avenue Box 46, Brooklyn, NY 11203; amorkh@pol.net

A 5-year-old girl came into the office complaining of severe burning and tingling sensation of her right forehead. She’d had fever, chills, myalgia, and a relentless headache for 3 days. The morning of her appointment, a few “bumps” and water-filled blisters began to appear on the right side of the forehead; the lesions then started to multiply and grow (FIGURE). The patient’s mother expressed concern over the rapid development of the lesions, which were accompanied by marked edema of the forehead and right eyelid. The mother indicated that no one else in the family was affected at the time of presentation.

The child appeared ill and pale at the time of presentation, but there was no history of immediate antecedent illness or any drug intake prior to the current eruption. Her growth and development were in the lower normal range. The child had a history of recurrent bacterial infections; she was not vaccinated for varicella. There was, however, a personal (and family) history of varicella when the child was about 3 years old.

FIGURE
Vesicles and bullae on forehead

On physical examination, there were multiple vesicles and bullae that varied in size from 2 mm to 1 cm in diameter on the right side of the forehead; there were areas of dried yellowish serous exudates limited to the right eyebrow. The forehead and periorbital areas were edematous with underlying erythematous skin. The entire eruption appeared to be restricted to the right upper part of the face, extending from the eyelid and medial canthus up to the frontal scalp. On close examination, several vesicles and crusts were present on the right side of the nasal tip. Serology for HIV was negative.

What is your diagnosis?
How would you treat?

Diagnosis: Herpes zoster ophthalmicus

This case of herpes zoster ophthalmicus (HZO) was unusual—not because of the way the patient presented, but because of her age. This condition is rarely seen in children, though it is not uncommon in adults—specifically, patients who are 60 years of age and older.^1-4

Herpes zoster ophthalmicus, which refers to the involvement of the ophthalmic branch of the trigeminal nerve or the fifth cranial nerve, usually manifests with a typical vesicular or bullous eruption of the forehead, often in unilateral fashion. The lesions of herpes zoster (shingles) are similar to those of varicella (chickenpox) but in herpes zoster, painful unilateral vesicular eruptions are usually limited to one dermatome. (If hematogenous dissemination occurs, more than 20 vesicles will form in skin areas away from the affected dermatome.¹)

Patients typically present with fever, headache, and abnormal sensations that precede the development of cutaneous lesions by a few days. The eruptions may be pustular and hemorrhagic initially, and within 10 days evolve into crusts.^5,6

Differential diagnosis: Pain sets HZO apart

In its classical presentation, HZO does not pose a diagnostic challenge for practitioners well-versed in skin disease management. The clinical differential diagnosis of herpes zoster will include other causes of blisters or vesicular eruptions of autoimmune etiologies, viral infections, or hypersensitivity reactions.

The most common blistering diseases that may be mistaken for zoster include herpes simplex, contact dermatitis, erythema multiforme, and cellulitis. The prodromal stage and the characteristics of pain usually set herpes zoster apart from the other diagnoses.

Beware of complications

Eye complications. HZO may result in paralytic ptosis, conjunctivitis, keratitis, cataracts, glaucoma, retinitis, and optic neuritis and atrophy.^2,5,7

Herpetic lesions on the tip of the nose are believed to herald ocular involvement and may precede typical dermatomal eruption of HZO.² Such cutaneous involvement along the distribution of the nasociliary nerve is called Hutchinson’s sign. It should prompt a complete ophthalmologic evaluation, as it did with our patient.

Neurologic complications. The most common complication of herpes zoster infection in general is postherpetic neuralgia (PHN), which results in severe pain that persists for months—or years—after the skin lesions have completely healed. Patients over 70 have a 70% chance of developing PHN.^4,7

Neurological complications such as encephalitis, myelitis, and Guillain-Barré syndrome have also been reported.

Other complications. Other complications include secondary bacterial infections, pneumonitis, and polyradiculitis.

Management: Antivirals and pain meds

Patients with HZO have a 50% chance of having eye complications (iritis and keratitis) without antiviral treatment.^2,5,6 Therefore, treatment is recommended for all HZO patients.

Antivirals ASAP

Start antiviral drugs within 72 hours of clinical presentation, and when new lesions are still appearing on the skin, to achieve optimal effect. Acyclovir, famciclovir, and valacyclovir are the antivirals of choice.⁶ The usual dosages for adults are: acyclovir (800 mg orally 5 times per day for 7–10 days), valacyclovir (1000 mg orally 3 times daily for 7 days) and famciclovir (500 mg orally 3 times daily for 7 days). The suggested dose of acyclovir for children is 10 to 20 mg/kg/dose qid for 5 days; not to exceed 800 mg per day.^2,5,6

Preventing postherpetic neuralgia

The role of systemic corticosteroids in the prevention of PHN, decreasing duration and severity of the acute symptoms in the initial days of herpes zoster infection, remains controversial.⁸

Immunosuppressed individuals may be treated with acyclovir, interferon-alpha, and vidarabine. In this population the live, attenuated vaccine is safer and is preferred to the varicella zoster immunoglobulins (VZIG).^1,6,8,9

PHN can be reduced by treating the patient within the first 24 hours of symptom onset. Pain usually resolves within 3 months in 50% of patients and within 1 year in 75% of patients.^1,6,7

Pain therapies for PHN

Therapeutic approaches to the management of PHN include topical anesthetic creams (lidocaine plus prilocaine), capsaicin, and oral medications such as tricyclic antidepressants (amitriptyline and desipramine), carbamazepine, and gabapentin, as well as nerve blocks.^6,7

Gloves and handwashing are key for caregivers

Individuals who have not had a confirmed varicella infection should avoid contact with those who have shingles unless a varicella zoster virus antibody is satisfactory and shows immunity.^6,7

Gloves should be worn when touching the lesions or infectious drainage, and hands should be washed after glove removal.^5-9

Patients with disseminated disease require hospitalization with airborne precautions, which include a negative air pressure room and ensuring that caregivers wear N95 respirators.

What put our patient at risk?

In the case of our young patient, low immunity may have played a role in her contracting HZO. She had a history of recurrent bacterial infections and suffered from generally poor health.

She was referred to an ophthalmologist, who did not find any dendritic corneal ulcer or other complications on weekly follow up. The patient was successfully managed with oral acyclovir 10 mg/kg/dose for 5 days, and she responded well to treatment. We also prescribed acyclovir eye ointment, and local wet compresses with good outcome.

Acknowledgments

The authors thank Dr Shahbaz Janjua for his assistance in the preparation of this manuscript.

Correspondence
Amor Khachemoune, MD, CWS, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, 530 First Avenue, Suite 7R, New York, NY 10016; amorkh@pol.net.

A 5-year-old girl came into the office complaining of severe burning and tingling sensation of her right forehead. She’d had fever, chills, myalgia, and a relentless headache for 3 days. The morning of her appointment, a few “bumps” and water-filled blisters began to appear on the right side of the forehead; the lesions then started to multiply and grow (FIGURE). The patient’s mother expressed concern over the rapid development of the lesions, which were accompanied by marked edema of the forehead and right eyelid. The mother indicated that no one else in the family was affected at the time of presentation.

The child appeared ill and pale at the time of presentation, but there was no history of immediate antecedent illness or any drug intake prior to the current eruption. Her growth and development were in the lower normal range. The child had a history of recurrent bacterial infections; she was not vaccinated for varicella. There was, however, a personal (and family) history of varicella when the child was about 3 years old.

FIGURE
Vesicles and bullae on forehead

On physical examination, there were multiple vesicles and bullae that varied in size from 2 mm to 1 cm in diameter on the right side of the forehead; there were areas of dried yellowish serous exudates limited to the right eyebrow. The forehead and periorbital areas were edematous with underlying erythematous skin. The entire eruption appeared to be restricted to the right upper part of the face, extending from the eyelid and medial canthus up to the frontal scalp. On close examination, several vesicles and crusts were present on the right side of the nasal tip. Serology for HIV was negative.

What is your diagnosis?
How would you treat?

Diagnosis: Herpes zoster ophthalmicus

This case of herpes zoster ophthalmicus (HZO) was unusual—not because of the way the patient presented, but because of her age. This condition is rarely seen in children, though it is not uncommon in adults—specifically, patients who are 60 years of age and older.^1-4

Herpes zoster ophthalmicus, which refers to the involvement of the ophthalmic branch of the trigeminal nerve or the fifth cranial nerve, usually manifests with a typical vesicular or bullous eruption of the forehead, often in unilateral fashion. The lesions of herpes zoster (shingles) are similar to those of varicella (chickenpox) but in herpes zoster, painful unilateral vesicular eruptions are usually limited to one dermatome. (If hematogenous dissemination occurs, more than 20 vesicles will form in skin areas away from the affected dermatome.¹)

Patients typically present with fever, headache, and abnormal sensations that precede the development of cutaneous lesions by a few days. The eruptions may be pustular and hemorrhagic initially, and within 10 days evolve into crusts.^5,6

Differential diagnosis: Pain sets HZO apart

In its classical presentation, HZO does not pose a diagnostic challenge for practitioners well-versed in skin disease management. The clinical differential diagnosis of herpes zoster will include other causes of blisters or vesicular eruptions of autoimmune etiologies, viral infections, or hypersensitivity reactions.

The most common blistering diseases that may be mistaken for zoster include herpes simplex, contact dermatitis, erythema multiforme, and cellulitis. The prodromal stage and the characteristics of pain usually set herpes zoster apart from the other diagnoses.

Beware of complications

Eye complications. HZO may result in paralytic ptosis, conjunctivitis, keratitis, cataracts, glaucoma, retinitis, and optic neuritis and atrophy.^2,5,7

Herpetic lesions on the tip of the nose are believed to herald ocular involvement and may precede typical dermatomal eruption of HZO.² Such cutaneous involvement along the distribution of the nasociliary nerve is called Hutchinson’s sign. It should prompt a complete ophthalmologic evaluation, as it did with our patient.

Neurologic complications. The most common complication of herpes zoster infection in general is postherpetic neuralgia (PHN), which results in severe pain that persists for months—or years—after the skin lesions have completely healed. Patients over 70 have a 70% chance of developing PHN.^4,7

Neurological complications such as encephalitis, myelitis, and Guillain-Barré syndrome have also been reported.

Other complications. Other complications include secondary bacterial infections, pneumonitis, and polyradiculitis.

Management: Antivirals and pain meds

Patients with HZO have a 50% chance of having eye complications (iritis and keratitis) without antiviral treatment.^2,5,6 Therefore, treatment is recommended for all HZO patients.

Antivirals ASAP

Start antiviral drugs within 72 hours of clinical presentation, and when new lesions are still appearing on the skin, to achieve optimal effect. Acyclovir, famciclovir, and valacyclovir are the antivirals of choice.⁶ The usual dosages for adults are: acyclovir (800 mg orally 5 times per day for 7–10 days), valacyclovir (1000 mg orally 3 times daily for 7 days) and famciclovir (500 mg orally 3 times daily for 7 days). The suggested dose of acyclovir for children is 10 to 20 mg/kg/dose qid for 5 days; not to exceed 800 mg per day.^2,5,6

Preventing postherpetic neuralgia

The role of systemic corticosteroids in the prevention of PHN, decreasing duration and severity of the acute symptoms in the initial days of herpes zoster infection, remains controversial.⁸

Immunosuppressed individuals may be treated with acyclovir, interferon-alpha, and vidarabine. In this population the live, attenuated vaccine is safer and is preferred to the varicella zoster immunoglobulins (VZIG).^1,6,8,9

PHN can be reduced by treating the patient within the first 24 hours of symptom onset. Pain usually resolves within 3 months in 50% of patients and within 1 year in 75% of patients.^1,6,7

Pain therapies for PHN

Therapeutic approaches to the management of PHN include topical anesthetic creams (lidocaine plus prilocaine), capsaicin, and oral medications such as tricyclic antidepressants (amitriptyline and desipramine), carbamazepine, and gabapentin, as well as nerve blocks.^6,7

Gloves and handwashing are key for caregivers

Individuals who have not had a confirmed varicella infection should avoid contact with those who have shingles unless a varicella zoster virus antibody is satisfactory and shows immunity.^6,7

Gloves should be worn when touching the lesions or infectious drainage, and hands should be washed after glove removal.^5-9

Patients with disseminated disease require hospitalization with airborne precautions, which include a negative air pressure room and ensuring that caregivers wear N95 respirators.

What put our patient at risk?

In the case of our young patient, low immunity may have played a role in her contracting HZO. She had a history of recurrent bacterial infections and suffered from generally poor health.

She was referred to an ophthalmologist, who did not find any dendritic corneal ulcer or other complications on weekly follow up. The patient was successfully managed with oral acyclovir 10 mg/kg/dose for 5 days, and she responded well to treatment. We also prescribed acyclovir eye ointment, and local wet compresses with good outcome.

Acknowledgments

The authors thank Dr Shahbaz Janjua for his assistance in the preparation of this manuscript.

Correspondence
Amor Khachemoune, MD, CWS, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, 530 First Avenue, Suite 7R, New York, NY 10016; amorkh@pol.net.

A 5-year-old girl came into the office complaining of severe burning and tingling sensation of her right forehead. She’d had fever, chills, myalgia, and a relentless headache for 3 days. The morning of her appointment, a few “bumps” and water-filled blisters began to appear on the right side of the forehead; the lesions then started to multiply and grow (FIGURE). The patient’s mother expressed concern over the rapid development of the lesions, which were accompanied by marked edema of the forehead and right eyelid. The mother indicated that no one else in the family was affected at the time of presentation.

The child appeared ill and pale at the time of presentation, but there was no history of immediate antecedent illness or any drug intake prior to the current eruption. Her growth and development were in the lower normal range. The child had a history of recurrent bacterial infections; she was not vaccinated for varicella. There was, however, a personal (and family) history of varicella when the child was about 3 years old.

FIGURE
Vesicles and bullae on forehead

On physical examination, there were multiple vesicles and bullae that varied in size from 2 mm to 1 cm in diameter on the right side of the forehead; there were areas of dried yellowish serous exudates limited to the right eyebrow. The forehead and periorbital areas were edematous with underlying erythematous skin. The entire eruption appeared to be restricted to the right upper part of the face, extending from the eyelid and medial canthus up to the frontal scalp. On close examination, several vesicles and crusts were present on the right side of the nasal tip. Serology for HIV was negative.

What is your diagnosis?
How would you treat?

Diagnosis: Herpes zoster ophthalmicus

This case of herpes zoster ophthalmicus (HZO) was unusual—not because of the way the patient presented, but because of her age. This condition is rarely seen in children, though it is not uncommon in adults—specifically, patients who are 60 years of age and older.^1-4

Herpes zoster ophthalmicus, which refers to the involvement of the ophthalmic branch of the trigeminal nerve or the fifth cranial nerve, usually manifests with a typical vesicular or bullous eruption of the forehead, often in unilateral fashion. The lesions of herpes zoster (shingles) are similar to those of varicella (chickenpox) but in herpes zoster, painful unilateral vesicular eruptions are usually limited to one dermatome. (If hematogenous dissemination occurs, more than 20 vesicles will form in skin areas away from the affected dermatome.¹)

Patients typically present with fever, headache, and abnormal sensations that precede the development of cutaneous lesions by a few days. The eruptions may be pustular and hemorrhagic initially, and within 10 days evolve into crusts.^5,6

Differential diagnosis: Pain sets HZO apart

In its classical presentation, HZO does not pose a diagnostic challenge for practitioners well-versed in skin disease management. The clinical differential diagnosis of herpes zoster will include other causes of blisters or vesicular eruptions of autoimmune etiologies, viral infections, or hypersensitivity reactions.

The most common blistering diseases that may be mistaken for zoster include herpes simplex, contact dermatitis, erythema multiforme, and cellulitis. The prodromal stage and the characteristics of pain usually set herpes zoster apart from the other diagnoses.

Beware of complications

Eye complications. HZO may result in paralytic ptosis, conjunctivitis, keratitis, cataracts, glaucoma, retinitis, and optic neuritis and atrophy.^2,5,7

Herpetic lesions on the tip of the nose are believed to herald ocular involvement and may precede typical dermatomal eruption of HZO.² Such cutaneous involvement along the distribution of the nasociliary nerve is called Hutchinson’s sign. It should prompt a complete ophthalmologic evaluation, as it did with our patient.

Neurologic complications. The most common complication of herpes zoster infection in general is postherpetic neuralgia (PHN), which results in severe pain that persists for months—or years—after the skin lesions have completely healed. Patients over 70 have a 70% chance of developing PHN.^4,7

Neurological complications such as encephalitis, myelitis, and Guillain-Barré syndrome have also been reported.

Other complications. Other complications include secondary bacterial infections, pneumonitis, and polyradiculitis.

Management: Antivirals and pain meds

Patients with HZO have a 50% chance of having eye complications (iritis and keratitis) without antiviral treatment.^2,5,6 Therefore, treatment is recommended for all HZO patients.

Antivirals ASAP

Start antiviral drugs within 72 hours of clinical presentation, and when new lesions are still appearing on the skin, to achieve optimal effect. Acyclovir, famciclovir, and valacyclovir are the antivirals of choice.⁶ The usual dosages for adults are: acyclovir (800 mg orally 5 times per day for 7–10 days), valacyclovir (1000 mg orally 3 times daily for 7 days) and famciclovir (500 mg orally 3 times daily for 7 days). The suggested dose of acyclovir for children is 10 to 20 mg/kg/dose qid for 5 days; not to exceed 800 mg per day.^2,5,6

Preventing postherpetic neuralgia

The role of systemic corticosteroids in the prevention of PHN, decreasing duration and severity of the acute symptoms in the initial days of herpes zoster infection, remains controversial.⁸

Immunosuppressed individuals may be treated with acyclovir, interferon-alpha, and vidarabine. In this population the live, attenuated vaccine is safer and is preferred to the varicella zoster immunoglobulins (VZIG).^1,6,8,9

PHN can be reduced by treating the patient within the first 24 hours of symptom onset. Pain usually resolves within 3 months in 50% of patients and within 1 year in 75% of patients.^1,6,7

Pain therapies for PHN

Therapeutic approaches to the management of PHN include topical anesthetic creams (lidocaine plus prilocaine), capsaicin, and oral medications such as tricyclic antidepressants (amitriptyline and desipramine), carbamazepine, and gabapentin, as well as nerve blocks.^6,7

Gloves and handwashing are key for caregivers

Individuals who have not had a confirmed varicella infection should avoid contact with those who have shingles unless a varicella zoster virus antibody is satisfactory and shows immunity.^6,7

Gloves should be worn when touching the lesions or infectious drainage, and hands should be washed after glove removal.^5-9

Patients with disseminated disease require hospitalization with airborne precautions, which include a negative air pressure room and ensuring that caregivers wear N95 respirators.

What put our patient at risk?

In the case of our young patient, low immunity may have played a role in her contracting HZO. She had a history of recurrent bacterial infections and suffered from generally poor health.

She was referred to an ophthalmologist, who did not find any dendritic corneal ulcer or other complications on weekly follow up. The patient was successfully managed with oral acyclovir 10 mg/kg/dose for 5 days, and she responded well to treatment. We also prescribed acyclovir eye ointment, and local wet compresses with good outcome.

Acknowledgments

The authors thank Dr Shahbaz Janjua for his assistance in the preparation of this manuscript.

Correspondence
Amor Khachemoune, MD, CWS, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, 530 First Avenue, Suite 7R, New York, NY 10016; amorkh@pol.net.

A 50-year-old woman came to the office for medical advice regarding bilateral erythematous lesions on the inner aspects of both of her legs. She had been aware of the lesions for more than 5 months. She had no other medical complaints, and was not taking any prescribed, over-the-counter, or herbal medications, or using any moisturizers or other medicated topical preparations. She mentioned that she was using a hot water bottle on her legs to keep her warm.

On physical examination, we noted bilateral erythema in a mesh-like distribution on her frontal, inner lower thighs as well as the upper and medial aspects of her calves. There were prominent telangiectatic vessels and “spider veins” surrounding the erythema, but her skin was otherwise normal. The review of her systems showed no problems. Personal and family histories were not contributory.

FIGURE 1
Lesions on the legs

Skin biopsy revealed subepidermal separation accompanied by thinning of the epidermis with loss of rete ridges and the presence of dermal edema. There was an increase in the number of elastic fibers with aberrant disruption. Perivascular infiltration of inflammatory cells was also noted.

FIGURE 2
Close-up

What is your diagnosis?
How would you manage this condition?

Diagnosis: Erythema ab igne

Erythema ab igne is characterized by localized erythema, reticulosis, and hypo-/hyperpigmentation, with telangiectsia and skin atrophy appearing in severe cases. This condition is caused by continuous exposure to sources of heat for prolonged periods of time.¹ Patients often do not connect their heat exposure to the rash, so you must recognize the local pattern of erythema ab igne and use your detective skills to identify the source.

History: Old disease, modern causes

Erythema ab igne has been noted for many years, and the sources of heat have changed over time. It used to be seen in women who stayed for long periods in front of open fires or furnaces to cook.^2,3 Most of the lesions appeared on the medial side of the thigh and the lower leg.

In modern times it is seen on different parts of the body depending on the heat source that initiated the pathology, the angle of the heat radiation, the morphology of the skin, and the layers of clothing.¹ Some of the modern causes of erythema ab igne are repeated application of hot water bottles or heat pads to treat chronic pain, exposure to car heaters and furniture with internal heaters, use of a laptop computer for long periods,⁴ and cook stoves, for restaurant workers who stand for long periods near the heat.³ Ultrasound physiotherapy was also reported as a cause.⁵ Recently, a case caused by frequent and prolonged hot bathing was reported.⁶

Clinical features: Mottled pattern clinically, subtle changes

The pattern of lesions form as a result of multiple exposures to the source of heat. Skin lesions may not appear immediately after the exposure—it might take a month to show up.⁷ Changes start as a reddish-brown pigmentation distributed as a mottled rash, followed by skin atrophy. Telangiectasias with diffuse hyperpigmentation and subepidermal bullae may also develop.⁸ Some patients complain of mild pruritus or burning sensation, but most patients are asymptomatic.⁹ Erythema ab igne should be differentiated from other diseases with skin changes that mimic its presentation (TABLE).

If clinically warranted, perform a biopsy to exclude the possibility of malignant formation. Histopathology results show epidermal atrophy, subepidermal separation, and haziness of the dermoepidermal junction. Dilatation of capillaries and connective tissue disintegration, elastosis, hemosiderin deposition, melanocytosis, and abundance of inflammatory cells are all seen in the dermis.¹⁰ Some of these lesions might progress to actinic keratosis, which could be a precursor for squamous cell carcinoma of the skin.¹¹ In some rare cases, Merkel cell carcinoma has developed in areas of erythema ab igne.¹²

Treatment: Eliminate heat source

The first goal of treatment is to identify the source of heat radiation to avoid further exposure. For mild lesions, no intervention is needed after the heat source is removed; the probability of full resolution is good. In this case, the patient was advised to stop using the hot water bottle on her skin. Over 4 months her lesions started to clear with no further intervention.

Topical meds help with cosmesis

Topical retinoids, vitamin A derivatives, hydroquinone, and 5-fluorouracil can be prescribed to treat abnormal skin pigmentation.¹³ Laser therapy has been used to even out the skin color.

TABLE
Differential diagnosis for erythema ab igne

CORRESPONDENCE
Amor Khachemoune, MD, CWS, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, 530 First Avenue, Suite 7R, New York, NY 10016. E-mail: amorkh@pol.net

A 50-year-old woman came to the office for medical advice regarding bilateral erythematous lesions on the inner aspects of both of her legs. She had been aware of the lesions for more than 5 months. She had no other medical complaints, and was not taking any prescribed, over-the-counter, or herbal medications, or using any moisturizers or other medicated topical preparations. She mentioned that she was using a hot water bottle on her legs to keep her warm.

On physical examination, we noted bilateral erythema in a mesh-like distribution on her frontal, inner lower thighs as well as the upper and medial aspects of her calves. There were prominent telangiectatic vessels and “spider veins” surrounding the erythema, but her skin was otherwise normal. The review of her systems showed no problems. Personal and family histories were not contributory.

FIGURE 1
Lesions on the legs

Skin biopsy revealed subepidermal separation accompanied by thinning of the epidermis with loss of rete ridges and the presence of dermal edema. There was an increase in the number of elastic fibers with aberrant disruption. Perivascular infiltration of inflammatory cells was also noted.

FIGURE 2
Close-up

What is your diagnosis?
How would you manage this condition?

Diagnosis: Erythema ab igne

Erythema ab igne is characterized by localized erythema, reticulosis, and hypo-/hyperpigmentation, with telangiectsia and skin atrophy appearing in severe cases. This condition is caused by continuous exposure to sources of heat for prolonged periods of time.¹ Patients often do not connect their heat exposure to the rash, so you must recognize the local pattern of erythema ab igne and use your detective skills to identify the source.

History: Old disease, modern causes

Erythema ab igne has been noted for many years, and the sources of heat have changed over time. It used to be seen in women who stayed for long periods in front of open fires or furnaces to cook.^2,3 Most of the lesions appeared on the medial side of the thigh and the lower leg.

In modern times it is seen on different parts of the body depending on the heat source that initiated the pathology, the angle of the heat radiation, the morphology of the skin, and the layers of clothing.¹ Some of the modern causes of erythema ab igne are repeated application of hot water bottles or heat pads to treat chronic pain, exposure to car heaters and furniture with internal heaters, use of a laptop computer for long periods,⁴ and cook stoves, for restaurant workers who stand for long periods near the heat.³ Ultrasound physiotherapy was also reported as a cause.⁵ Recently, a case caused by frequent and prolonged hot bathing was reported.⁶

Clinical features: Mottled pattern clinically, subtle changes

The pattern of lesions form as a result of multiple exposures to the source of heat. Skin lesions may not appear immediately after the exposure—it might take a month to show up.⁷ Changes start as a reddish-brown pigmentation distributed as a mottled rash, followed by skin atrophy. Telangiectasias with diffuse hyperpigmentation and subepidermal bullae may also develop.⁸ Some patients complain of mild pruritus or burning sensation, but most patients are asymptomatic.⁹ Erythema ab igne should be differentiated from other diseases with skin changes that mimic its presentation (TABLE).

If clinically warranted, perform a biopsy to exclude the possibility of malignant formation. Histopathology results show epidermal atrophy, subepidermal separation, and haziness of the dermoepidermal junction. Dilatation of capillaries and connective tissue disintegration, elastosis, hemosiderin deposition, melanocytosis, and abundance of inflammatory cells are all seen in the dermis.¹⁰ Some of these lesions might progress to actinic keratosis, which could be a precursor for squamous cell carcinoma of the skin.¹¹ In some rare cases, Merkel cell carcinoma has developed in areas of erythema ab igne.¹²

Treatment: Eliminate heat source

The first goal of treatment is to identify the source of heat radiation to avoid further exposure. For mild lesions, no intervention is needed after the heat source is removed; the probability of full resolution is good. In this case, the patient was advised to stop using the hot water bottle on her skin. Over 4 months her lesions started to clear with no further intervention.

Topical meds help with cosmesis

Topical retinoids, vitamin A derivatives, hydroquinone, and 5-fluorouracil can be prescribed to treat abnormal skin pigmentation.¹³ Laser therapy has been used to even out the skin color.

TABLE
Differential diagnosis for erythema ab igne

CORRESPONDENCE
Amor Khachemoune, MD, CWS, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, 530 First Avenue, Suite 7R, New York, NY 10016. E-mail: amorkh@pol.net

A 50-year-old woman came to the office for medical advice regarding bilateral erythematous lesions on the inner aspects of both of her legs. She had been aware of the lesions for more than 5 months. She had no other medical complaints, and was not taking any prescribed, over-the-counter, or herbal medications, or using any moisturizers or other medicated topical preparations. She mentioned that she was using a hot water bottle on her legs to keep her warm.

On physical examination, we noted bilateral erythema in a mesh-like distribution on her frontal, inner lower thighs as well as the upper and medial aspects of her calves. There were prominent telangiectatic vessels and “spider veins” surrounding the erythema, but her skin was otherwise normal. The review of her systems showed no problems. Personal and family histories were not contributory.

FIGURE 1
Lesions on the legs

Skin biopsy revealed subepidermal separation accompanied by thinning of the epidermis with loss of rete ridges and the presence of dermal edema. There was an increase in the number of elastic fibers with aberrant disruption. Perivascular infiltration of inflammatory cells was also noted.

FIGURE 2
Close-up

What is your diagnosis?
How would you manage this condition?

Diagnosis: Erythema ab igne

Erythema ab igne is characterized by localized erythema, reticulosis, and hypo-/hyperpigmentation, with telangiectsia and skin atrophy appearing in severe cases. This condition is caused by continuous exposure to sources of heat for prolonged periods of time.¹ Patients often do not connect their heat exposure to the rash, so you must recognize the local pattern of erythema ab igne and use your detective skills to identify the source.

History: Old disease, modern causes

Erythema ab igne has been noted for many years, and the sources of heat have changed over time. It used to be seen in women who stayed for long periods in front of open fires or furnaces to cook.^2,3 Most of the lesions appeared on the medial side of the thigh and the lower leg.

In modern times it is seen on different parts of the body depending on the heat source that initiated the pathology, the angle of the heat radiation, the morphology of the skin, and the layers of clothing.¹ Some of the modern causes of erythema ab igne are repeated application of hot water bottles or heat pads to treat chronic pain, exposure to car heaters and furniture with internal heaters, use of a laptop computer for long periods,⁴ and cook stoves, for restaurant workers who stand for long periods near the heat.³ Ultrasound physiotherapy was also reported as a cause.⁵ Recently, a case caused by frequent and prolonged hot bathing was reported.⁶

Clinical features: Mottled pattern clinically, subtle changes

The pattern of lesions form as a result of multiple exposures to the source of heat. Skin lesions may not appear immediately after the exposure—it might take a month to show up.⁷ Changes start as a reddish-brown pigmentation distributed as a mottled rash, followed by skin atrophy. Telangiectasias with diffuse hyperpigmentation and subepidermal bullae may also develop.⁸ Some patients complain of mild pruritus or burning sensation, but most patients are asymptomatic.⁹ Erythema ab igne should be differentiated from other diseases with skin changes that mimic its presentation (TABLE).

If clinically warranted, perform a biopsy to exclude the possibility of malignant formation. Histopathology results show epidermal atrophy, subepidermal separation, and haziness of the dermoepidermal junction. Dilatation of capillaries and connective tissue disintegration, elastosis, hemosiderin deposition, melanocytosis, and abundance of inflammatory cells are all seen in the dermis.¹⁰ Some of these lesions might progress to actinic keratosis, which could be a precursor for squamous cell carcinoma of the skin.¹¹ In some rare cases, Merkel cell carcinoma has developed in areas of erythema ab igne.¹²

Treatment: Eliminate heat source

The first goal of treatment is to identify the source of heat radiation to avoid further exposure. For mild lesions, no intervention is needed after the heat source is removed; the probability of full resolution is good. In this case, the patient was advised to stop using the hot water bottle on her skin. Over 4 months her lesions started to clear with no further intervention.

Topical meds help with cosmesis

Topical retinoids, vitamin A derivatives, hydroquinone, and 5-fluorouracil can be prescribed to treat abnormal skin pigmentation.¹³ Laser therapy has been used to even out the skin color.

TABLE
Differential diagnosis for erythema ab igne

CORRESPONDENCE
Amor Khachemoune, MD, CWS, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, 530 First Avenue, Suite 7R, New York, NY 10016. E-mail: amorkh@pol.net