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Year : 2016  |  Volume : 7  |  Issue : 1  |  Page : 66  

Adult-onset familial palmoplantar keratoderma: An uncommon presentation


1 Department of DVL, Muzaffarnagar Medical College and Hospital, Muzaffarnagar, Uttar Pradesh, India
2 Department of Pathology, Muzaffarnagar Medical College and Hospital, Muzaffarnagar, Uttar Pradesh, India

Date of Web Publication18-Jan-2016

Correspondence Address:
Dr. Tarang Goyal
Muzaffarnagar Medical College and Hospital, Muzaffarnagar, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.174316

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How to cite this article:
Goyal T, Varshney A. Adult-onset familial palmoplantar keratoderma: An uncommon presentation. Indian Dermatol Online J 2016;7:66

How to cite this URL:
Goyal T, Varshney A. Adult-onset familial palmoplantar keratoderma: An uncommon presentation. Indian Dermatol Online J [serial online] 2016 [cited 2020 Aug 7];7:66. Available from: http://www.idoj.in/text.asp?2016/7/1/66/174316

A father and son duo presented with palmoplantar hyperkeratotic papules of late teenage onset. On evaluation they were both diagnosed to have Buschke–Fisher–Brauer syndrome (Syn: Punctate palmoplantar keratoderma type IA, keratosis palmoplantaris papulosa). The condition is autosomal dominant with an incidence 1:1,00,000 with an equal incidences among the sexes. Lesions begin as small keratotic translucent papules that may turn verrucous and opaque with sizes between 2 and 8 mm [Figure 1]. Both our patients gave a history of aggravation of symptoms on performing manual works. Histopathologically, a focal, compact column of massive hyperkeratosis with a thickened granular layer and no inflammation in the dermis was seen. Often misdiagnosed as verruca vulgaris, spiny keratoderma, Darier, Cowden disease, and arsenical keratoses; it partially responds to chemical or mechanical keratolytics, systemic retinoids, and systemic 5-fluorouracil.
Figure 1: Discrete and grouped, transluscent as well as opaque, hyperkeratotic papules over the palmoplantar surfaces with prominence on pressure-bearing sites

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