Epidermolysis bullosa pruriginosa: A rare presentation with asymptomatic lesions.
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Date
2013-03
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Abstract
Epidermolysis bullosa pruriginosa (EBP) is a subtype of dominant dystrophic epidermolysis bullosa (DDEB) and is clinically characterized by pruritic lichenified plaques or prurigo-like lesions with violaceous linear scarring. Pruritus has always been described as one of the most striking features in EBP. Mutations in COL7A gene, especially in the glycine residue, have been shown to cause this form of DDEB. In this report, we describe a north Indian familial clustering of three cases of EBP, spread across two generations, presenting with hypertrophic lichenoid cutaneous lesions, which were completely asymptomatic. Clinical and histopathological analysis favored the diagnosis of EBP in all three cases. They are being reported for their unusual asymptomatic presentation.
Description
Keywords
COL7A1, epidermolysis bullosa pruriginosa, nail dystrophy
Citation
Ghosh Sangita, Chaudhuri Soumik, Jain Vijay Kumar. Epidermolysis bullosa pruriginosa: A rare presentation with asymptomatic lesions. Indian Journal of Dermatology, Venereology and Leprology. 2013 Mar-Apr; 79(2): 235-237.