Brauer-Buschke-Fisher Syndrome - Report of a Case & Review of the Literature
Keywords:
Keratoderma, Brauer-Buschke-Fischer syndrome, Palmoplantar keratodermaAbstract
The palmoplantar keratodermas (PPKs) are a heterogeneous group of disorders characterized by abnormal thickening of the palms and soles. Hereditary punctate palmoplantar keratoderma also known as Brauer-Buschke-Fischer syndrome belongs to a group of rare genodermatosis with a prevalence estimated at 1.17/100000. Punctate PPK, in which multiple tiny "raindrop" keratoses involve the palmoplantar surface. They may involve the whole of the palmoplantar surface or may be more restricted in their distribution. Considerable intra- and interfamily variation is often seen. Hereby we report a case of 70-year-old Indian man, working as a farmer, reported to Dermatology OPD with multiple asymptomatic swellings on the palms and soles. The lesions developed since childhood and slowly increased in size and number thereafter. His sister and son had similar lesions on the palms and soles. Examination revealed numerous yellowish brown, hard, opaque, keratotic papules varying in size from 1 to 4 mm irregularly distributed on his palms, fingers, and soles. Some of the papules were coalesced to form larger hyperkeratotic plaques of varying sizes. Small & large lesion coexisted. The patient had no history of itching, bleeding, exposure to arsenic, seasonal variations or any dermatological/ systemic associations. We diagnosed the disease as Punctate Palmoplantar keratoderma type 1 based on a positive family history, typical lesions at typical locations with typical course.
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