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ANSWER
The correct answer is discoid lupus (choice “d”); see discussion for further details.
Sarcoidosis (choice “a”) is a worthy item in this differential, since it can be chronic, often affects the face (especially in African-Americans), and frequently defies ready diagnosis. But the biopsy was totally inconsistent with this diagnosis; in a case of sarcoidosis, it instead would have shown noncaseating granulomas, which are characteristic of the condition.
Lichen planus (choice “b”) is likewise worth consideration, since it can present in a similar fashion (although the chronicity of this patient’s lesions would have been atypical). Moreover, lichen planus is almost invariably symptomatic (itch). Biopsy would have shown obliteration of the dermoepidermal junction by an intense lymphocytic infiltrate—findings totally at odds with what was seen.
Polymorphous light eruption (PMLE; choice “c”) is the name given to a variety of photosensitivities that, true to the term polymorphous (or polymorphic), can present in numerous ways—although the lesions on any given patient tend to be monomorphic. These can take the form of vesicles, papules, and even erythema multiforme–like targetoid lesions, most commonly (as expected) on sun-exposed skin. Curiously, though, PMLE seldom affects the face or hands. It can manifest early in a patient’s life, but it would have been “seasonal,” disappearing in winter, and would have revealed a totally different picture on biopsy.
DISCUSSION
This patient suffered needlessly for more than half his life for lack of one simple thing: a correct diagnosis. Truth be known, the patient and his family probably bear some responsibility—but at some point, one of his many providers should have either obtained a punch biopsy or sent him to someone who would do so.
Instead, as is often the case, the emphasis was on treatment: trying one thing after another. The lack of success with these endeavors speaks loudly for the need for a definitive diagnosis. This could only be established one way: with a biopsy.
All the items mentioned in the above differential were legitimately considered. So was the possibility of infection, especially atypical types such as mycobacterial, deep fungal, or those involving other unusual organisms (eg, Nocardia, Actinomycetes). As in this case, tissue can be collected and submitted for culture, but the usual formalin preservative will kill any organism, necessitating prompt processing in saline.
Discoid lupus erythematosus (DLE) can be purely cutaneous (as seen here) or can be a manifestation of more serious systemic lupus. In any case, it is an autoimmune process, made worse by the sun, and can be chronic (though this case is exceptional in that regard).
This patient’s chance of developing systemic lupus erythematosus is slight, at most, since his antinuclear antibody test was negative. But his lifetime risk for another autoimmune disease is high.
TREATMENT
DLE is usually treated successfully with a combination of sun avoidance and a course of oral hydroxychloroquine (200 mg QD to bid, depending on the patient’s body habitus and the severity of the disease). Given the advanced state of this patient’s condition, he received the more frequent dosage, which should yield positive results. However, he will likely be on this regimen for some time.
ANSWER
The correct answer is discoid lupus (choice “d”); see discussion for further details.
Sarcoidosis (choice “a”) is a worthy item in this differential, since it can be chronic, often affects the face (especially in African-Americans), and frequently defies ready diagnosis. But the biopsy was totally inconsistent with this diagnosis; in a case of sarcoidosis, it instead would have shown noncaseating granulomas, which are characteristic of the condition.
Lichen planus (choice “b”) is likewise worth consideration, since it can present in a similar fashion (although the chronicity of this patient’s lesions would have been atypical). Moreover, lichen planus is almost invariably symptomatic (itch). Biopsy would have shown obliteration of the dermoepidermal junction by an intense lymphocytic infiltrate—findings totally at odds with what was seen.
Polymorphous light eruption (PMLE; choice “c”) is the name given to a variety of photosensitivities that, true to the term polymorphous (or polymorphic), can present in numerous ways—although the lesions on any given patient tend to be monomorphic. These can take the form of vesicles, papules, and even erythema multiforme–like targetoid lesions, most commonly (as expected) on sun-exposed skin. Curiously, though, PMLE seldom affects the face or hands. It can manifest early in a patient’s life, but it would have been “seasonal,” disappearing in winter, and would have revealed a totally different picture on biopsy.
DISCUSSION
This patient suffered needlessly for more than half his life for lack of one simple thing: a correct diagnosis. Truth be known, the patient and his family probably bear some responsibility—but at some point, one of his many providers should have either obtained a punch biopsy or sent him to someone who would do so.
Instead, as is often the case, the emphasis was on treatment: trying one thing after another. The lack of success with these endeavors speaks loudly for the need for a definitive diagnosis. This could only be established one way: with a biopsy.
All the items mentioned in the above differential were legitimately considered. So was the possibility of infection, especially atypical types such as mycobacterial, deep fungal, or those involving other unusual organisms (eg, Nocardia, Actinomycetes). As in this case, tissue can be collected and submitted for culture, but the usual formalin preservative will kill any organism, necessitating prompt processing in saline.
Discoid lupus erythematosus (DLE) can be purely cutaneous (as seen here) or can be a manifestation of more serious systemic lupus. In any case, it is an autoimmune process, made worse by the sun, and can be chronic (though this case is exceptional in that regard).
This patient’s chance of developing systemic lupus erythematosus is slight, at most, since his antinuclear antibody test was negative. But his lifetime risk for another autoimmune disease is high.
TREATMENT
DLE is usually treated successfully with a combination of sun avoidance and a course of oral hydroxychloroquine (200 mg QD to bid, depending on the patient’s body habitus and the severity of the disease). Given the advanced state of this patient’s condition, he received the more frequent dosage, which should yield positive results. However, he will likely be on this regimen for some time.
ANSWER
The correct answer is discoid lupus (choice “d”); see discussion for further details.
Sarcoidosis (choice “a”) is a worthy item in this differential, since it can be chronic, often affects the face (especially in African-Americans), and frequently defies ready diagnosis. But the biopsy was totally inconsistent with this diagnosis; in a case of sarcoidosis, it instead would have shown noncaseating granulomas, which are characteristic of the condition.
Lichen planus (choice “b”) is likewise worth consideration, since it can present in a similar fashion (although the chronicity of this patient’s lesions would have been atypical). Moreover, lichen planus is almost invariably symptomatic (itch). Biopsy would have shown obliteration of the dermoepidermal junction by an intense lymphocytic infiltrate—findings totally at odds with what was seen.
Polymorphous light eruption (PMLE; choice “c”) is the name given to a variety of photosensitivities that, true to the term polymorphous (or polymorphic), can present in numerous ways—although the lesions on any given patient tend to be monomorphic. These can take the form of vesicles, papules, and even erythema multiforme–like targetoid lesions, most commonly (as expected) on sun-exposed skin. Curiously, though, PMLE seldom affects the face or hands. It can manifest early in a patient’s life, but it would have been “seasonal,” disappearing in winter, and would have revealed a totally different picture on biopsy.
DISCUSSION
This patient suffered needlessly for more than half his life for lack of one simple thing: a correct diagnosis. Truth be known, the patient and his family probably bear some responsibility—but at some point, one of his many providers should have either obtained a punch biopsy or sent him to someone who would do so.
Instead, as is often the case, the emphasis was on treatment: trying one thing after another. The lack of success with these endeavors speaks loudly for the need for a definitive diagnosis. This could only be established one way: with a biopsy.
All the items mentioned in the above differential were legitimately considered. So was the possibility of infection, especially atypical types such as mycobacterial, deep fungal, or those involving other unusual organisms (eg, Nocardia, Actinomycetes). As in this case, tissue can be collected and submitted for culture, but the usual formalin preservative will kill any organism, necessitating prompt processing in saline.
Discoid lupus erythematosus (DLE) can be purely cutaneous (as seen here) or can be a manifestation of more serious systemic lupus. In any case, it is an autoimmune process, made worse by the sun, and can be chronic (though this case is exceptional in that regard).
This patient’s chance of developing systemic lupus erythematosus is slight, at most, since his antinuclear antibody test was negative. But his lifetime risk for another autoimmune disease is high.
TREATMENT
DLE is usually treated successfully with a combination of sun avoidance and a course of oral hydroxychloroquine (200 mg QD to bid, depending on the patient’s body habitus and the severity of the disease). Given the advanced state of this patient’s condition, he received the more frequent dosage, which should yield positive results. However, he will likely be on this regimen for some time.
Since the fourth grade, this 22-year-old African-American man has had facial lesions that, although constantly present, worsen in the summer. The problem is so severe that it has greatly affected his quality of life: In school and in his neighborhood, he has been subjected to rumors that his condition might be contagious. Despite numerous treatment attempts—including topical and oral anti-acne medications, topical and oral antifungal medications, and oral antibiotics—the condition has persisted. Once, at his mother’s urging, the patient even sought the assistance of a faith healer at a religious revival. Initially seen by a primary care provider at a free clinic, he was then referred to a dermatology clinician at the same facility. History taking reveals that the lesions are asymptomatic and appear in additional locations (eg, arms, ears, and neck). The patient denies any family history of similar problems, as well as persistent fever, cough, or shortness of breath. Lab studies obtained by his primary care provider, including a complete blood count and chem screen, were within normal limits. Most of the lesions on the patient’s face are round areas of slightly erythematous erosion covered by eschar. They range in size from 3 mm to more than 1 cm. Focal postinflammatory hyperpigmentation is noted (a common finding in those with type V/VI skin). Focal hyperpigmentation is also seen on both ears, in some cases with scaling and faint erosion on the surface. A KOH prep is performed with scale collected from perilesional skin; results are negative for fungal elements. There are no palpable nodes in the adjacent nodal locations. Two punch biopsies are done, with samples taken from the active margins of the lesions. One is submitted for routine H&E (hematoxylin and eosin) handling and the other in saline for bacterial, acid-fast bacilli (AFB), and fungal cultures. The biopsy shows hyperkeratosis, follicular plugging, and epidermal atrophy. Marked vacuolar degeneration of the dermoepidermal junction is also noted, along with mucin deposition. Stains for bacteria, AFB, and fungi yield negative results.
