Quiz

The Diagnosis: Primary Cutaneous Mucinous Carcinoma

Primary cutaneous mucinous carcinoma is a rare tumor of the sweat glands that was first reported in 1952 by Lennox et al.¹ These tumors are slow growing and have a predilection for the head and neck, with the eyelid being the most commonly reported location.² In general, they present as erythematous asymptomatic nodules measuring less than 7 cm in diameter.^2-4 Primary cutaneous mucinous carcinoma tends to have a good prognosis with complete resection, but cases of metastasis and recurrence have been reported.² Although there is no standard of care, treatment typically consists of surgical management, as the tumors are nonresponsive to chemotherapy or radiation.⁴ Kamalpour et al² compared outcomes for Mohs micrographic surgery versus standard excision, the former showing a lower percentage of poor outcomes. Of note, there were fewer cases treated with Mohs surgery in this study; only more recently reported cases have been treated with Mohs surgery.

Histologically, primary cutaneous mucinous carcinoma is composed of cords, tubules, and lobules of epithelial cells floating in large pools of basophilic mucin, separated by thin fibrovascular septa.⁵ It can be difficult to distinguish a primary tumor from a mucinous carcinoma metastasis with histology alone, especially on the breasts and in the gastrointestinal tract. Immunohistochemistry can be helpful in determining the origin of the tumor. A homologue of p53, p63 expressed in basal and myoepithelial cells of the skin can aid in the confirmation of a primary tumor when present.^6,7 Negative staining for cytokeratin 20 and positive staining for cytokeratin 7 also are helpful in distinguishing a primary cutaneous mucinous carcinoma from a gastrointestinal tract metastasis.^4,8

In our patient, no other symptoms were present that raised concern for an internal malignancy. Findings that supported a primary versus metastatic tumor included the clinicopathologic findings (Figure) as well as positive p63, cytokeratin 7, and negative cytokeratin 20 staining. The initial standard excision had tumor cells within 1 mm of the specimen margin; thus, a subsequent wider reexcision was performed. Reexcision was negative for tumor cells. Close follow-up with a primary care physician was recommended, with emphasis on colon and breast cancer screening. A follow-up mammogram was negative for breast cancer.

The Diagnosis: Primary Cutaneous Mucinous Carcinoma

Primary cutaneous mucinous carcinoma is a rare tumor of the sweat glands that was first reported in 1952 by Lennox et al.¹ These tumors are slow growing and have a predilection for the head and neck, with the eyelid being the most commonly reported location.² In general, they present as erythematous asymptomatic nodules measuring less than 7 cm in diameter.^2-4 Primary cutaneous mucinous carcinoma tends to have a good prognosis with complete resection, but cases of metastasis and recurrence have been reported.² Although there is no standard of care, treatment typically consists of surgical management, as the tumors are nonresponsive to chemotherapy or radiation.⁴ Kamalpour et al² compared outcomes for Mohs micrographic surgery versus standard excision, the former showing a lower percentage of poor outcomes. Of note, there were fewer cases treated with Mohs surgery in this study; only more recently reported cases have been treated with Mohs surgery.

Histologically, primary cutaneous mucinous carcinoma is composed of cords, tubules, and lobules of epithelial cells floating in large pools of basophilic mucin, separated by thin fibrovascular septa.⁵ It can be difficult to distinguish a primary tumor from a mucinous carcinoma metastasis with histology alone, especially on the breasts and in the gastrointestinal tract. Immunohistochemistry can be helpful in determining the origin of the tumor. A homologue of p53, p63 expressed in basal and myoepithelial cells of the skin can aid in the confirmation of a primary tumor when present.^6,7 Negative staining for cytokeratin 20 and positive staining for cytokeratin 7 also are helpful in distinguishing a primary cutaneous mucinous carcinoma from a gastrointestinal tract metastasis.^4,8

In our patient, no other symptoms were present that raised concern for an internal malignancy. Findings that supported a primary versus metastatic tumor included the clinicopathologic findings (Figure) as well as positive p63, cytokeratin 7, and negative cytokeratin 20 staining. The initial standard excision had tumor cells within 1 mm of the specimen margin; thus, a subsequent wider reexcision was performed. Reexcision was negative for tumor cells. Close follow-up with a primary care physician was recommended, with emphasis on colon and breast cancer screening. A follow-up mammogram was negative for breast cancer.

The Diagnosis: Primary Cutaneous Mucinous Carcinoma

Primary cutaneous mucinous carcinoma is a rare tumor of the sweat glands that was first reported in 1952 by Lennox et al.¹ These tumors are slow growing and have a predilection for the head and neck, with the eyelid being the most commonly reported location.² In general, they present as erythematous asymptomatic nodules measuring less than 7 cm in diameter.^2-4 Primary cutaneous mucinous carcinoma tends to have a good prognosis with complete resection, but cases of metastasis and recurrence have been reported.² Although there is no standard of care, treatment typically consists of surgical management, as the tumors are nonresponsive to chemotherapy or radiation.⁴ Kamalpour et al² compared outcomes for Mohs micrographic surgery versus standard excision, the former showing a lower percentage of poor outcomes. Of note, there were fewer cases treated with Mohs surgery in this study; only more recently reported cases have been treated with Mohs surgery.

Histologically, primary cutaneous mucinous carcinoma is composed of cords, tubules, and lobules of epithelial cells floating in large pools of basophilic mucin, separated by thin fibrovascular septa.⁵ It can be difficult to distinguish a primary tumor from a mucinous carcinoma metastasis with histology alone, especially on the breasts and in the gastrointestinal tract. Immunohistochemistry can be helpful in determining the origin of the tumor. A homologue of p53, p63 expressed in basal and myoepithelial cells of the skin can aid in the confirmation of a primary tumor when present.^6,7 Negative staining for cytokeratin 20 and positive staining for cytokeratin 7 also are helpful in distinguishing a primary cutaneous mucinous carcinoma from a gastrointestinal tract metastasis.^4,8

In our patient, no other symptoms were present that raised concern for an internal malignancy. Findings that supported a primary versus metastatic tumor included the clinicopathologic findings (Figure) as well as positive p63, cytokeratin 7, and negative cytokeratin 20 staining. The initial standard excision had tumor cells within 1 mm of the specimen margin; thus, a subsequent wider reexcision was performed. Reexcision was negative for tumor cells. Close follow-up with a primary care physician was recommended, with emphasis on colon and breast cancer screening. A follow-up mammogram was negative for breast cancer.

ANSWER
The correct interpretation of this ECG includes normal sinus rhythm, biatrial enlargement, nonspecific ST-T wave abnormality, and an RSR’ or QR pattern in V₁, suggestive of right ventricular conduction delay.

Biatrial enlargement by definition encompasses right atrial enlargement (criteria include P waves in leads II, III, and aVF measuring 2.5 mm or more) and left atrial enlargement (evidenced by P waves in lead I ≥ 110 ms, and a biphasic, or “notched,” P wave with terminal negativity in lead V₁).

Lead V₁ may be interpreted as either an RSR’ or a QR pattern. However, the QRS duration of < 120 ms precludes this from meeting criteria for a right bundle branch block.

Finally, nonspecific ST-T wave changes were present in the precordial leads. These may be consistent with pulmonary disease.

ANSWER
The correct interpretation of this ECG includes normal sinus rhythm, biatrial enlargement, nonspecific ST-T wave abnormality, and an RSR’ or QR pattern in V₁, suggestive of right ventricular conduction delay.

Biatrial enlargement by definition encompasses right atrial enlargement (criteria include P waves in leads II, III, and aVF measuring 2.5 mm or more) and left atrial enlargement (evidenced by P waves in lead I ≥ 110 ms, and a biphasic, or “notched,” P wave with terminal negativity in lead V₁).

Lead V₁ may be interpreted as either an RSR’ or a QR pattern. However, the QRS duration of < 120 ms precludes this from meeting criteria for a right bundle branch block.

Finally, nonspecific ST-T wave changes were present in the precordial leads. These may be consistent with pulmonary disease.

ANSWER
The correct interpretation of this ECG includes normal sinus rhythm, biatrial enlargement, nonspecific ST-T wave abnormality, and an RSR’ or QR pattern in V₁, suggestive of right ventricular conduction delay.

Biatrial enlargement by definition encompasses right atrial enlargement (criteria include P waves in leads II, III, and aVF measuring 2.5 mm or more) and left atrial enlargement (evidenced by P waves in lead I ≥ 110 ms, and a biphasic, or “notched,” P wave with terminal negativity in lead V₁).

Lead V₁ may be interpreted as either an RSR’ or a QR pattern. However, the QRS duration of < 120 ms precludes this from meeting criteria for a right bundle branch block.

Finally, nonspecific ST-T wave changes were present in the precordial leads. These may be consistent with pulmonary disease.

Answer
The radiograph has several findings, one of which is a nondisplaced proximal fibula fracture. In addition, there is a moderate suprapatellar joint effusion. The patient also has fairly advanced tricompartment degenerative arthrosis. (To review, the tricompartment comprises all three anatomic areas of the knee: the patellofemoral, lateral tibiofemoral, and medial tibiofemoral joints.)

Answer
The radiograph has several findings, one of which is a nondisplaced proximal fibula fracture. In addition, there is a moderate suprapatellar joint effusion. The patient also has fairly advanced tricompartment degenerative arthrosis. (To review, the tricompartment comprises all three anatomic areas of the knee: the patellofemoral, lateral tibiofemoral, and medial tibiofemoral joints.)

Answer
The radiograph has several findings, one of which is a nondisplaced proximal fibula fracture. In addition, there is a moderate suprapatellar joint effusion. The patient also has fairly advanced tricompartment degenerative arthrosis. (To review, the tricompartment comprises all three anatomic areas of the knee: the patellofemoral, lateral tibiofemoral, and medial tibiofemoral joints.)

ANSWER
The correct answer is none of the above (choice “d”). These lesions are all intradermal nevi, which have little, if any, risk for malignant transformation. Deeper nevi are considered quite safe, unless significant change has occurred. Despite the unlikelihood, however, it is risky to declare a 0% chance of skin cancer.

DISCUSSION
Slow growth and increased prominence are not the kinds of changes to look for in skin lesions. Rather, look for marked asymmetry (eg, the growth of a new, darker, macular component) or other change in color or consistency.

Hairs on these lesions are quite normal and are actually reassuring in confirming their benign nature. Skin cancers seldom support hair growth.

Most melanomas don’t come from moles. Instead, they are “de novo” lesions, literally coming from nothing, out of clear skin. It is true that the more moles someone has, the greater his or her risk for skin cancer, though not necessarily in one of the moles. When melanomas do develop from nevi (a collection of a certain type of melanocyte), this usually occurs in superficial types, such as compound or junctional nevi. From an objective standpoint, in this patient’s case, family history means nothing.

What does matter is to pay as much attention to the owner as to the lesion. The more fair-skinned and sun-damaged (freckles, blue eyes, red hair) the patient is, the more worrisome a lesion can be.

This patient had none of those traits, and she will likely have one of her lesions surgically excised to ensure she’s satisfied with the resulting scar. Of course, the tissue sample will be sent for pathologic examination, as any specimen should be.

ANSWER
The correct answer is none of the above (choice “d”). These lesions are all intradermal nevi, which have little, if any, risk for malignant transformation. Deeper nevi are considered quite safe, unless significant change has occurred. Despite the unlikelihood, however, it is risky to declare a 0% chance of skin cancer.

DISCUSSION
Slow growth and increased prominence are not the kinds of changes to look for in skin lesions. Rather, look for marked asymmetry (eg, the growth of a new, darker, macular component) or other change in color or consistency.

Hairs on these lesions are quite normal and are actually reassuring in confirming their benign nature. Skin cancers seldom support hair growth.

Most melanomas don’t come from moles. Instead, they are “de novo” lesions, literally coming from nothing, out of clear skin. It is true that the more moles someone has, the greater his or her risk for skin cancer, though not necessarily in one of the moles. When melanomas do develop from nevi (a collection of a certain type of melanocyte), this usually occurs in superficial types, such as compound or junctional nevi. From an objective standpoint, in this patient’s case, family history means nothing.

What does matter is to pay as much attention to the owner as to the lesion. The more fair-skinned and sun-damaged (freckles, blue eyes, red hair) the patient is, the more worrisome a lesion can be.

This patient had none of those traits, and she will likely have one of her lesions surgically excised to ensure she’s satisfied with the resulting scar. Of course, the tissue sample will be sent for pathologic examination, as any specimen should be.

ANSWER
The correct answer is none of the above (choice “d”). These lesions are all intradermal nevi, which have little, if any, risk for malignant transformation. Deeper nevi are considered quite safe, unless significant change has occurred. Despite the unlikelihood, however, it is risky to declare a 0% chance of skin cancer.

DISCUSSION
Slow growth and increased prominence are not the kinds of changes to look for in skin lesions. Rather, look for marked asymmetry (eg, the growth of a new, darker, macular component) or other change in color or consistency.

Hairs on these lesions are quite normal and are actually reassuring in confirming their benign nature. Skin cancers seldom support hair growth.

Most melanomas don’t come from moles. Instead, they are “de novo” lesions, literally coming from nothing, out of clear skin. It is true that the more moles someone has, the greater his or her risk for skin cancer, though not necessarily in one of the moles. When melanomas do develop from nevi (a collection of a certain type of melanocyte), this usually occurs in superficial types, such as compound or junctional nevi. From an objective standpoint, in this patient’s case, family history means nothing.

What does matter is to pay as much attention to the owner as to the lesion. The more fair-skinned and sun-damaged (freckles, blue eyes, red hair) the patient is, the more worrisome a lesion can be.

This patient had none of those traits, and she will likely have one of her lesions surgically excised to ensure she’s satisfied with the resulting scar. Of course, the tissue sample will be sent for pathologic examination, as any specimen should be.

The Diagnosis: Eccrine Angiomatous Hamartoma

Given the progression of symptoms 3 months prior to presentation, an excisional biopsy was performed (Figure 1). Hematoxylin and eosin staining showed prominent eccrine sweat glands and vessels surrounded by superficially located adipose tissue in the mid and deep dermis (Figure 2).

Eccrine angiomatous hamartoma (EAH) is an uncommon benign tumor typically located on the arms and legs or trunk. It is usually solitary, though cases with multiple lesions have been reported.^1,2 Most cases are diagnosed in childhood as either congenital or acquired lesions. However, EAHs can develop in adulthood and have been described in patients up to 70 years of age.³ The median age of diagnosis is 10 years,² indicating that EAH is primarily a pediatric tumor. There is no gender predilection.

Approximately 35% to 66% of patients report pain, pruritus, or hyperhidrosis associated with EAHs, though this incidence may be overrepresented because patients tend to present when the lesions become symptomatic.^2-5 The pain is attributed to nerve fibers infiltrating the tumor. Hypertrichosis also has been described and is thought to be due to hair follicles within the hamartoma.

Histologically, EAHs are characterized by normal-appearing eccrine glands mingled with venules and capillaries. Additional variable pathologic findings include lipomatous, pilar, lymphatic, or mucinous features.² Other vascular anomalies such as hemangiomas or arteriovenous malformations occasionally have been described in association with EAH. The vessels stain for ulex europaeus 1 and factor VIII. Eccrine glands stain for S-100 protein, carcinoembryonic antigen, epithelial membrane antigen, and cytokeratin CAM 5.2. In light of a publication proposing that EAH is a lymphatic proliferation,⁶ a D2-40 stain was performed on the specimen and was negative. 

Eccrine angiomatous hamartoma has been reported to grow mainly during childhood, puberty, or pregnancy, presumably due to hormonal influences.⁷ There are few reports of EAH enlarging in middle-aged adults, and even fewer without pain during the growth phase. It is unclear what triggered the growth in our otherwise healthy postmenopausal patient.

Eccrine angiomatous hamartoma does not have malignant potential and thus treatment is optional and based on relief of symptoms. Simple excision of the EAH usually is curative, but recurrences can occur.⁴ Botulinum toxin also has been used to treat hyperhidrosis in tumors that are too large for resection. Treatment with lasers such as the pulsed dye laser and Nd:YAG laser has not been successful.⁸ A case of spontaneous regression has been reported.¹

Liposuction was considered in our patient given the substantial adipose tissue on biopsy. The patient ultimately declined treatment. This case highlights that EAH can present in adulthood and should be considered in the differential diagnosis of an enlarging but otherwise asymptomatic vascular tumor.

The Diagnosis: Eccrine Angiomatous Hamartoma

Given the progression of symptoms 3 months prior to presentation, an excisional biopsy was performed (Figure 1). Hematoxylin and eosin staining showed prominent eccrine sweat glands and vessels surrounded by superficially located adipose tissue in the mid and deep dermis (Figure 2).

Eccrine angiomatous hamartoma (EAH) is an uncommon benign tumor typically located on the arms and legs or trunk. It is usually solitary, though cases with multiple lesions have been reported.^1,2 Most cases are diagnosed in childhood as either congenital or acquired lesions. However, EAHs can develop in adulthood and have been described in patients up to 70 years of age.³ The median age of diagnosis is 10 years,² indicating that EAH is primarily a pediatric tumor. There is no gender predilection.

Approximately 35% to 66% of patients report pain, pruritus, or hyperhidrosis associated with EAHs, though this incidence may be overrepresented because patients tend to present when the lesions become symptomatic.^2-5 The pain is attributed to nerve fibers infiltrating the tumor. Hypertrichosis also has been described and is thought to be due to hair follicles within the hamartoma.

Histologically, EAHs are characterized by normal-appearing eccrine glands mingled with venules and capillaries. Additional variable pathologic findings include lipomatous, pilar, lymphatic, or mucinous features.² Other vascular anomalies such as hemangiomas or arteriovenous malformations occasionally have been described in association with EAH. The vessels stain for ulex europaeus 1 and factor VIII. Eccrine glands stain for S-100 protein, carcinoembryonic antigen, epithelial membrane antigen, and cytokeratin CAM 5.2. In light of a publication proposing that EAH is a lymphatic proliferation,⁶ a D2-40 stain was performed on the specimen and was negative. 

Eccrine angiomatous hamartoma has been reported to grow mainly during childhood, puberty, or pregnancy, presumably due to hormonal influences.⁷ There are few reports of EAH enlarging in middle-aged adults, and even fewer without pain during the growth phase. It is unclear what triggered the growth in our otherwise healthy postmenopausal patient.

Eccrine angiomatous hamartoma does not have malignant potential and thus treatment is optional and based on relief of symptoms. Simple excision of the EAH usually is curative, but recurrences can occur.⁴ Botulinum toxin also has been used to treat hyperhidrosis in tumors that are too large for resection. Treatment with lasers such as the pulsed dye laser and Nd:YAG laser has not been successful.⁸ A case of spontaneous regression has been reported.¹

Liposuction was considered in our patient given the substantial adipose tissue on biopsy. The patient ultimately declined treatment. This case highlights that EAH can present in adulthood and should be considered in the differential diagnosis of an enlarging but otherwise asymptomatic vascular tumor.

The Diagnosis: Eccrine Angiomatous Hamartoma

Given the progression of symptoms 3 months prior to presentation, an excisional biopsy was performed (Figure 1). Hematoxylin and eosin staining showed prominent eccrine sweat glands and vessels surrounded by superficially located adipose tissue in the mid and deep dermis (Figure 2).

Eccrine angiomatous hamartoma (EAH) is an uncommon benign tumor typically located on the arms and legs or trunk. It is usually solitary, though cases with multiple lesions have been reported.^1,2 Most cases are diagnosed in childhood as either congenital or acquired lesions. However, EAHs can develop in adulthood and have been described in patients up to 70 years of age.³ The median age of diagnosis is 10 years,² indicating that EAH is primarily a pediatric tumor. There is no gender predilection.

Approximately 35% to 66% of patients report pain, pruritus, or hyperhidrosis associated with EAHs, though this incidence may be overrepresented because patients tend to present when the lesions become symptomatic.^2-5 The pain is attributed to nerve fibers infiltrating the tumor. Hypertrichosis also has been described and is thought to be due to hair follicles within the hamartoma.

Histologically, EAHs are characterized by normal-appearing eccrine glands mingled with venules and capillaries. Additional variable pathologic findings include lipomatous, pilar, lymphatic, or mucinous features.² Other vascular anomalies such as hemangiomas or arteriovenous malformations occasionally have been described in association with EAH. The vessels stain for ulex europaeus 1 and factor VIII. Eccrine glands stain for S-100 protein, carcinoembryonic antigen, epithelial membrane antigen, and cytokeratin CAM 5.2. In light of a publication proposing that EAH is a lymphatic proliferation,⁶ a D2-40 stain was performed on the specimen and was negative. 

Eccrine angiomatous hamartoma has been reported to grow mainly during childhood, puberty, or pregnancy, presumably due to hormonal influences.⁷ There are few reports of EAH enlarging in middle-aged adults, and even fewer without pain during the growth phase. It is unclear what triggered the growth in our otherwise healthy postmenopausal patient.

Eccrine angiomatous hamartoma does not have malignant potential and thus treatment is optional and based on relief of symptoms. Simple excision of the EAH usually is curative, but recurrences can occur.⁴ Botulinum toxin also has been used to treat hyperhidrosis in tumors that are too large for resection. Treatment with lasers such as the pulsed dye laser and Nd:YAG laser has not been successful.⁸ A case of spontaneous regression has been reported.¹

Liposuction was considered in our patient given the substantial adipose tissue on biopsy. The patient ultimately declined treatment. This case highlights that EAH can present in adulthood and should be considered in the differential diagnosis of an enlarging but otherwise asymptomatic vascular tumor.

The Diagnosis: Granulomatous Slack Skin Disease

Initial biopsy revealed a lichenoid lymphohistiocytic infiltrate with scattered epidermotropism, papillary dermal sclerosis, and lymphocyte atypia (Figure 1). A repeat biopsy showed a lichenoid granulomatous infiltrate with histiocytes and rare giant cells, superficially located in the dermis, without a deeper dense infiltration. Focal lymphocytic epidermotropism also was present (Figure 2). The infiltrate was CD3⁺CD4⁺ with a minority of cells also staining for CD8. An elastin stain demonstrated diminished elastin fibers in the superficial dermis. A clonal T-cell receptor gene rearrangement was identified by polymerase chain reaction. One group of pink and brown papules was present on the dorsal aspect of the right foot (Figure 3). A biopsy of this area showed similar findings. The patient was treated with a trial of carmustine 20-mg% ointment over the following year with some improvement of the mild pruritus but without notable change in the clinical findings.

Granulomatous slack skin disease (GSSD) is a rare form of mycosis fungoides–type cutaneous T-cell lymphoma. It usually presents as well-demarcated, atrophic, poikilodermatous patches and plaques with a predilection for the inguinal and axillary regions.¹ The affected areas tend to be asymptomatic and enlarge gradually over years to become pendulous with lax skin and wrinkles. In contrast to other forms of cutaneous T-cell lymphoma, extracutaneous spread is rare. The disease shows a slow progression over many years and by itself is not life threatening. However, affected patients have a risk for developing secondary lymphoproliferative neoplasms, which have been documented in approximately 50% of reported cases.² These lymphoproliferative neoplasms may arise concurrently, precede, or follow the development of GSSD lesions. Hodgkin lymphoma, seen in 33% of cases, is the most common association, with others being non-Hodgkin lymphoma, mycosis fungoides, acute myeloid leukemia, and Langerhans cell histiocytosis.^1-3

Histologically, GSSD is characterized by a dense, dermal, granulomatous proliferation of atypical T lymphocytes with scattered multinucleated giant cells.^1,4 There is a loss of elastin fibers in the infiltrated areas, and occasional elastophagocytosis can be seen.^1,2,4 Immunoprofiling of the infiltrate has shown CD3⁺CD4⁺CD45RO⁺ T-helper cells with occasional loss of CD5 and CD7.³ A clonal T-cell receptor rearrangement of the g and b genes frequently is described.^1,4,5

At this time no treatment has been found to be reliably curative. Varying success in treating GSSD has been achieved with topical nitrogen mustard, carmustine, topical and systemic corticosteroids, psoralen plus UVA, radiotherapy, azathioprine, IFN-g, and combinations of these agents.^1-3,6-9 Excision of the diseased skin has been performed for cosmetically or functionally disturbing lesions, but in all but one case the lesions recurred within months.^1,10 A consistently reliable treatment of GSSD has not been established; treatment should be tailored to the individual patient.

The Diagnosis: Granulomatous Slack Skin Disease

Initial biopsy revealed a lichenoid lymphohistiocytic infiltrate with scattered epidermotropism, papillary dermal sclerosis, and lymphocyte atypia (Figure 1). A repeat biopsy showed a lichenoid granulomatous infiltrate with histiocytes and rare giant cells, superficially located in the dermis, without a deeper dense infiltration. Focal lymphocytic epidermotropism also was present (Figure 2). The infiltrate was CD3⁺CD4⁺ with a minority of cells also staining for CD8. An elastin stain demonstrated diminished elastin fibers in the superficial dermis. A clonal T-cell receptor gene rearrangement was identified by polymerase chain reaction. One group of pink and brown papules was present on the dorsal aspect of the right foot (Figure 3). A biopsy of this area showed similar findings. The patient was treated with a trial of carmustine 20-mg% ointment over the following year with some improvement of the mild pruritus but without notable change in the clinical findings.

Granulomatous slack skin disease (GSSD) is a rare form of mycosis fungoides–type cutaneous T-cell lymphoma. It usually presents as well-demarcated, atrophic, poikilodermatous patches and plaques with a predilection for the inguinal and axillary regions.¹ The affected areas tend to be asymptomatic and enlarge gradually over years to become pendulous with lax skin and wrinkles. In contrast to other forms of cutaneous T-cell lymphoma, extracutaneous spread is rare. The disease shows a slow progression over many years and by itself is not life threatening. However, affected patients have a risk for developing secondary lymphoproliferative neoplasms, which have been documented in approximately 50% of reported cases.² These lymphoproliferative neoplasms may arise concurrently, precede, or follow the development of GSSD lesions. Hodgkin lymphoma, seen in 33% of cases, is the most common association, with others being non-Hodgkin lymphoma, mycosis fungoides, acute myeloid leukemia, and Langerhans cell histiocytosis.^1-3

Histologically, GSSD is characterized by a dense, dermal, granulomatous proliferation of atypical T lymphocytes with scattered multinucleated giant cells.^1,4 There is a loss of elastin fibers in the infiltrated areas, and occasional elastophagocytosis can be seen.^1,2,4 Immunoprofiling of the infiltrate has shown CD3⁺CD4⁺CD45RO⁺ T-helper cells with occasional loss of CD5 and CD7.³ A clonal T-cell receptor rearrangement of the g and b genes frequently is described.^1,4,5

At this time no treatment has been found to be reliably curative. Varying success in treating GSSD has been achieved with topical nitrogen mustard, carmustine, topical and systemic corticosteroids, psoralen plus UVA, radiotherapy, azathioprine, IFN-g, and combinations of these agents.^1-3,6-9 Excision of the diseased skin has been performed for cosmetically or functionally disturbing lesions, but in all but one case the lesions recurred within months.^1,10 A consistently reliable treatment of GSSD has not been established; treatment should be tailored to the individual patient.

The Diagnosis: Granulomatous Slack Skin Disease

Initial biopsy revealed a lichenoid lymphohistiocytic infiltrate with scattered epidermotropism, papillary dermal sclerosis, and lymphocyte atypia (Figure 1). A repeat biopsy showed a lichenoid granulomatous infiltrate with histiocytes and rare giant cells, superficially located in the dermis, without a deeper dense infiltration. Focal lymphocytic epidermotropism also was present (Figure 2). The infiltrate was CD3⁺CD4⁺ with a minority of cells also staining for CD8. An elastin stain demonstrated diminished elastin fibers in the superficial dermis. A clonal T-cell receptor gene rearrangement was identified by polymerase chain reaction. One group of pink and brown papules was present on the dorsal aspect of the right foot (Figure 3). A biopsy of this area showed similar findings. The patient was treated with a trial of carmustine 20-mg% ointment over the following year with some improvement of the mild pruritus but without notable change in the clinical findings.

Granulomatous slack skin disease (GSSD) is a rare form of mycosis fungoides–type cutaneous T-cell lymphoma. It usually presents as well-demarcated, atrophic, poikilodermatous patches and plaques with a predilection for the inguinal and axillary regions.¹ The affected areas tend to be asymptomatic and enlarge gradually over years to become pendulous with lax skin and wrinkles. In contrast to other forms of cutaneous T-cell lymphoma, extracutaneous spread is rare. The disease shows a slow progression over many years and by itself is not life threatening. However, affected patients have a risk for developing secondary lymphoproliferative neoplasms, which have been documented in approximately 50% of reported cases.² These lymphoproliferative neoplasms may arise concurrently, precede, or follow the development of GSSD lesions. Hodgkin lymphoma, seen in 33% of cases, is the most common association, with others being non-Hodgkin lymphoma, mycosis fungoides, acute myeloid leukemia, and Langerhans cell histiocytosis.^1-3

Histologically, GSSD is characterized by a dense, dermal, granulomatous proliferation of atypical T lymphocytes with scattered multinucleated giant cells.^1,4 There is a loss of elastin fibers in the infiltrated areas, and occasional elastophagocytosis can be seen.^1,2,4 Immunoprofiling of the infiltrate has shown CD3⁺CD4⁺CD45RO⁺ T-helper cells with occasional loss of CD5 and CD7.³ A clonal T-cell receptor rearrangement of the g and b genes frequently is described.^1,4,5

At this time no treatment has been found to be reliably curative. Varying success in treating GSSD has been achieved with topical nitrogen mustard, carmustine, topical and systemic corticosteroids, psoralen plus UVA, radiotherapy, azathioprine, IFN-g, and combinations of these agents.^1-3,6-9 Excision of the diseased skin has been performed for cosmetically or functionally disturbing lesions, but in all but one case the lesions recurred within months.^1,10 A consistently reliable treatment of GSSD has not been established; treatment should be tailored to the individual patient.

The Diagnosis: Fibroepithelioma of Pinkus

Fibroepithelioma of Pinkus (FeP) was first described in 1953¹ and was thought to be premalignant as evidenced by the proposed name premalignant fibroepithelial tumor of the skin. This neoplasm now is largely believed to represent a rare form of basal cell carcinoma (BCC). Typical presentation is a smooth, flesh-colored or pink plaque or nodule.² Fibroepithelioma of Pinkus has a predilection for the lumbosacral back, though the groin also has been reported as a common site of incidence.^1,3 Similar to other BCCs, it is seen in older individuals, typically those older than 50 years.^3,4

Clinical diagnosis of FeP can be difficult. The differential diagnosis of FeP can include acrochordon, amelanotic melanoma, compound nevus, hemangioma, neurofibroma, nevus sebaceous, pyogenic granuloma, and seborrheic keratosis.⁵ Dermoscopic evaluation can aid in the diagnosis. A vascular network composed of fine arborizing vessels with or without dotted vessels and white streaks are characteristic findings of FeP. Patients with pigment also demonstrate structureless gray-brown areas and gray-blue dots.⁶

Biopsy with subsequent histopathologic evaluation confirms the diagnosis of FeP. The characteristic microscopic findings of thin eosinophilic epithelial strands with eccrine ducts anastomosing in an abundant fibromyxoid stroma with collections of basophilic cells located at the ends of the epithelial strands were demonstrated in our patient’s histopathologic specimen (Figure). The histologic appearance is similar to syringofibroadenoma of Mascaro. Recognition of basaloid nests, which often demonstrate retraction, and mitotic activity can differentiate FeP from syringofibroadenoma of Mascaro.⁷

Treatment of FeP is largely the same as other BCCs including destruction by electrodesiccation and curettage or complete removal by surgical excision. Several studies have demonstrated effective treatment of nonaggressive BCCs with curettage alone and subjectively reported improved cosmesis compared to electrodesiccation and curettage.^8-10 Although methyl aminolevulinate photodynamic therapy has demonstrated some therapeutic efficacy for superficial and nodular BCCs,¹¹ a case report utilizing the same modality for FeP did not provide adequate response.¹² However, adequate data are not available to assess potential use of this less invasive therapy.

The Diagnosis: Fibroepithelioma of Pinkus

Fibroepithelioma of Pinkus (FeP) was first described in 1953¹ and was thought to be premalignant as evidenced by the proposed name premalignant fibroepithelial tumor of the skin. This neoplasm now is largely believed to represent a rare form of basal cell carcinoma (BCC). Typical presentation is a smooth, flesh-colored or pink plaque or nodule.² Fibroepithelioma of Pinkus has a predilection for the lumbosacral back, though the groin also has been reported as a common site of incidence.^1,3 Similar to other BCCs, it is seen in older individuals, typically those older than 50 years.^3,4

Clinical diagnosis of FeP can be difficult. The differential diagnosis of FeP can include acrochordon, amelanotic melanoma, compound nevus, hemangioma, neurofibroma, nevus sebaceous, pyogenic granuloma, and seborrheic keratosis.⁵ Dermoscopic evaluation can aid in the diagnosis. A vascular network composed of fine arborizing vessels with or without dotted vessels and white streaks are characteristic findings of FeP. Patients with pigment also demonstrate structureless gray-brown areas and gray-blue dots.⁶

Biopsy with subsequent histopathologic evaluation confirms the diagnosis of FeP. The characteristic microscopic findings of thin eosinophilic epithelial strands with eccrine ducts anastomosing in an abundant fibromyxoid stroma with collections of basophilic cells located at the ends of the epithelial strands were demonstrated in our patient’s histopathologic specimen (Figure). The histologic appearance is similar to syringofibroadenoma of Mascaro. Recognition of basaloid nests, which often demonstrate retraction, and mitotic activity can differentiate FeP from syringofibroadenoma of Mascaro.⁷

Treatment of FeP is largely the same as other BCCs including destruction by electrodesiccation and curettage or complete removal by surgical excision. Several studies have demonstrated effective treatment of nonaggressive BCCs with curettage alone and subjectively reported improved cosmesis compared to electrodesiccation and curettage.^8-10 Although methyl aminolevulinate photodynamic therapy has demonstrated some therapeutic efficacy for superficial and nodular BCCs,¹¹ a case report utilizing the same modality for FeP did not provide adequate response.¹² However, adequate data are not available to assess potential use of this less invasive therapy.

The Diagnosis: Fibroepithelioma of Pinkus

Fibroepithelioma of Pinkus (FeP) was first described in 1953¹ and was thought to be premalignant as evidenced by the proposed name premalignant fibroepithelial tumor of the skin. This neoplasm now is largely believed to represent a rare form of basal cell carcinoma (BCC). Typical presentation is a smooth, flesh-colored or pink plaque or nodule.² Fibroepithelioma of Pinkus has a predilection for the lumbosacral back, though the groin also has been reported as a common site of incidence.^1,3 Similar to other BCCs, it is seen in older individuals, typically those older than 50 years.^3,4

Clinical diagnosis of FeP can be difficult. The differential diagnosis of FeP can include acrochordon, amelanotic melanoma, compound nevus, hemangioma, neurofibroma, nevus sebaceous, pyogenic granuloma, and seborrheic keratosis.⁵ Dermoscopic evaluation can aid in the diagnosis. A vascular network composed of fine arborizing vessels with or without dotted vessels and white streaks are characteristic findings of FeP. Patients with pigment also demonstrate structureless gray-brown areas and gray-blue dots.⁶

Biopsy with subsequent histopathologic evaluation confirms the diagnosis of FeP. The characteristic microscopic findings of thin eosinophilic epithelial strands with eccrine ducts anastomosing in an abundant fibromyxoid stroma with collections of basophilic cells located at the ends of the epithelial strands were demonstrated in our patient’s histopathologic specimen (Figure). The histologic appearance is similar to syringofibroadenoma of Mascaro. Recognition of basaloid nests, which often demonstrate retraction, and mitotic activity can differentiate FeP from syringofibroadenoma of Mascaro.⁷

Treatment of FeP is largely the same as other BCCs including destruction by electrodesiccation and curettage or complete removal by surgical excision. Several studies have demonstrated effective treatment of nonaggressive BCCs with curettage alone and subjectively reported improved cosmesis compared to electrodesiccation and curettage.^8-10 Although methyl aminolevulinate photodynamic therapy has demonstrated some therapeutic efficacy for superficial and nodular BCCs,¹¹ a case report utilizing the same modality for FeP did not provide adequate response.¹² However, adequate data are not available to assess potential use of this less invasive therapy.

Answer
The radiograph shows a large cavitary lesion within the left mid-lung with evidence of an air fluid level. This finding is strongly suggestive of a postoperative abscess or empyema. Secondarily, there is some pleural thickening within the left lateral apex region. This can be suggestive of scarring or possibly a neoplasm.

The patient was admitted to the ICU for a sepsis workup, and Interventional Radiology was consulted to evaluate for CT-guided drain placement.

Answer
The radiograph shows a large cavitary lesion within the left mid-lung with evidence of an air fluid level. This finding is strongly suggestive of a postoperative abscess or empyema. Secondarily, there is some pleural thickening within the left lateral apex region. This can be suggestive of scarring or possibly a neoplasm.

The patient was admitted to the ICU for a sepsis workup, and Interventional Radiology was consulted to evaluate for CT-guided drain placement.

Answer
The radiograph shows a large cavitary lesion within the left mid-lung with evidence of an air fluid level. This finding is strongly suggestive of a postoperative abscess or empyema. Secondarily, there is some pleural thickening within the left lateral apex region. This can be suggestive of scarring or possibly a neoplasm.

The patient was admitted to the ICU for a sepsis workup, and Interventional Radiology was consulted to evaluate for CT-guided drain placement.

ANSWER
Findings on this ECG include sinus rhythm with frequent, consecutive premature ventricular complexes (PVCs) consistent with nonsustained ventricular tachycardia (NSVT). There is also evidence of a probable left atrial enlargement.

The key to interpreting this ECG is to first locate normal-appearing complexes. These are illustrated by the third, fourth, 10th, and 11th complexes on the rhythm strip (lead I) at the bottom of the ECG. Notice that there is a normal-appearing PQRST complex for each of these beats.

The rate of 82 beats/min is calculated from a sum average of all beats on the 12-lead ECG; however, the R-R interval between the third and fourth and the 10th and 11th beats is roughly 60 beats/min, signifying a normal sinus rhythm. All other beats are PVCs arising from the left ventricle (as evidenced by a right bundle branch pattern in lead V₁).

Careful inspection will reveal retrograde P waves located in the terminal upstroke of the S wave. NSVT is defined as three or more consecutive PVCs at a rate greater than 100 beats/min with a duration of less than 30 seconds. The pauses seen between a PVC and a normally conducting P wave are caused by retrograde conduction from the ventricle to the atrium, with subsequent block within the atrium.

Finally, left atrial enlargement is evidenced by a biphasic P wave in the normally conducting beat seen in lead V₁.

ANSWER
Findings on this ECG include sinus rhythm with frequent, consecutive premature ventricular complexes (PVCs) consistent with nonsustained ventricular tachycardia (NSVT). There is also evidence of a probable left atrial enlargement.

The key to interpreting this ECG is to first locate normal-appearing complexes. These are illustrated by the third, fourth, 10th, and 11th complexes on the rhythm strip (lead I) at the bottom of the ECG. Notice that there is a normal-appearing PQRST complex for each of these beats.

The rate of 82 beats/min is calculated from a sum average of all beats on the 12-lead ECG; however, the R-R interval between the third and fourth and the 10th and 11th beats is roughly 60 beats/min, signifying a normal sinus rhythm. All other beats are PVCs arising from the left ventricle (as evidenced by a right bundle branch pattern in lead V₁).

Careful inspection will reveal retrograde P waves located in the terminal upstroke of the S wave. NSVT is defined as three or more consecutive PVCs at a rate greater than 100 beats/min with a duration of less than 30 seconds. The pauses seen between a PVC and a normally conducting P wave are caused by retrograde conduction from the ventricle to the atrium, with subsequent block within the atrium.

Finally, left atrial enlargement is evidenced by a biphasic P wave in the normally conducting beat seen in lead V₁.

ANSWER
Findings on this ECG include sinus rhythm with frequent, consecutive premature ventricular complexes (PVCs) consistent with nonsustained ventricular tachycardia (NSVT). There is also evidence of a probable left atrial enlargement.

The key to interpreting this ECG is to first locate normal-appearing complexes. These are illustrated by the third, fourth, 10th, and 11th complexes on the rhythm strip (lead I) at the bottom of the ECG. Notice that there is a normal-appearing PQRST complex for each of these beats.

The rate of 82 beats/min is calculated from a sum average of all beats on the 12-lead ECG; however, the R-R interval between the third and fourth and the 10th and 11th beats is roughly 60 beats/min, signifying a normal sinus rhythm. All other beats are PVCs arising from the left ventricle (as evidenced by a right bundle branch pattern in lead V₁).

Careful inspection will reveal retrograde P waves located in the terminal upstroke of the S wave. NSVT is defined as three or more consecutive PVCs at a rate greater than 100 beats/min with a duration of less than 30 seconds. The pauses seen between a PVC and a normally conducting P wave are caused by retrograde conduction from the ventricle to the atrium, with subsequent block within the atrium.

Finally, left atrial enlargement is evidenced by a biphasic P wave in the normally conducting beat seen in lead V₁.

ANSWER
The correct answer is nevus sebaceous (choice “b”), a rather common hamartomatous congenital tumor. The diagnosis was confirmed by pathologic examination of a tiny sample from the most papular portion of the lesion.

Given the lesion’s congenital nature and complete lack of response to treatment, wart (choice “a”) was quite unlikely. And although trichofolliculoma (choice “c”) and epidermal nevus (choice “d”) were possible differential items, the former usually appears much later in life and the latter is usually more dry and rough to the touch.

DISCUSSION
Nevus sebaceous (NS) of Jadassohn was first described in 1895 by a Swedish dermatologist who had seen a series of young patients with hairless plaques in the scalp or on surrounding neck or facial skin. Pathologic exam confirmed them to be organoid nevi representing an overgrowth of sebaceous glands.

Over time, it became clear that NS affects all genders and races equally. For most patients, the lesions are of cosmetic concern due to the lack of hair. But it has also been established that NS can develop in areas, including the face, ears, and neck, on which they may be cosmetically significant and difficult to remove.

Concern arose when reports began to surface that NS could undergo malignant transformation, especially in larger scalp lesions that are subject to years of excess UV exposure. This was the driving force behind the common practice of removing NS at puberty. We now know that although basal or squamous cell carcinoma, or even melanoma, can develop in longstanding NS, the frequency is probably far less than previously thought.

Most cases of NS in the scalp are easy to diagnose by their pink color, plaquish morphology, and mammillated hairless surface (coupled with congenital manifestation). But a few, such as this patient’s ear lesion, require biopsy for confirmation. As this patient ages, he may feel the need to have the rest of it surgically removed.

ANSWER
The correct answer is nevus sebaceous (choice “b”), a rather common hamartomatous congenital tumor. The diagnosis was confirmed by pathologic examination of a tiny sample from the most papular portion of the lesion.

Given the lesion’s congenital nature and complete lack of response to treatment, wart (choice “a”) was quite unlikely. And although trichofolliculoma (choice “c”) and epidermal nevus (choice “d”) were possible differential items, the former usually appears much later in life and the latter is usually more dry and rough to the touch.

DISCUSSION
Nevus sebaceous (NS) of Jadassohn was first described in 1895 by a Swedish dermatologist who had seen a series of young patients with hairless plaques in the scalp or on surrounding neck or facial skin. Pathologic exam confirmed them to be organoid nevi representing an overgrowth of sebaceous glands.

Over time, it became clear that NS affects all genders and races equally. For most patients, the lesions are of cosmetic concern due to the lack of hair. But it has also been established that NS can develop in areas, including the face, ears, and neck, on which they may be cosmetically significant and difficult to remove.

Concern arose when reports began to surface that NS could undergo malignant transformation, especially in larger scalp lesions that are subject to years of excess UV exposure. This was the driving force behind the common practice of removing NS at puberty. We now know that although basal or squamous cell carcinoma, or even melanoma, can develop in longstanding NS, the frequency is probably far less than previously thought.

Most cases of NS in the scalp are easy to diagnose by their pink color, plaquish morphology, and mammillated hairless surface (coupled with congenital manifestation). But a few, such as this patient’s ear lesion, require biopsy for confirmation. As this patient ages, he may feel the need to have the rest of it surgically removed.

ANSWER
The correct answer is nevus sebaceous (choice “b”), a rather common hamartomatous congenital tumor. The diagnosis was confirmed by pathologic examination of a tiny sample from the most papular portion of the lesion.

Given the lesion’s congenital nature and complete lack of response to treatment, wart (choice “a”) was quite unlikely. And although trichofolliculoma (choice “c”) and epidermal nevus (choice “d”) were possible differential items, the former usually appears much later in life and the latter is usually more dry and rough to the touch.

DISCUSSION
Nevus sebaceous (NS) of Jadassohn was first described in 1895 by a Swedish dermatologist who had seen a series of young patients with hairless plaques in the scalp or on surrounding neck or facial skin. Pathologic exam confirmed them to be organoid nevi representing an overgrowth of sebaceous glands.

Over time, it became clear that NS affects all genders and races equally. For most patients, the lesions are of cosmetic concern due to the lack of hair. But it has also been established that NS can develop in areas, including the face, ears, and neck, on which they may be cosmetically significant and difficult to remove.

Concern arose when reports began to surface that NS could undergo malignant transformation, especially in larger scalp lesions that are subject to years of excess UV exposure. This was the driving force behind the common practice of removing NS at puberty. We now know that although basal or squamous cell carcinoma, or even melanoma, can develop in longstanding NS, the frequency is probably far less than previously thought.

Most cases of NS in the scalp are easy to diagnose by their pink color, plaquish morphology, and mammillated hairless surface (coupled with congenital manifestation). But a few, such as this patient’s ear lesion, require biopsy for confirmation. As this patient ages, he may feel the need to have the rest of it surgically removed.