Epidermolysis bullosa pruriginosa--report of three cases.
Epidermolysis bullosa pruriginosa, a genetic mechanobullous disease, is characterized by pruritus, lichenified or nodular prurigo-like lesions, occasional trauma-induced blistering, excoriations, milia, nail dystrophy and albopapuloid lesions, appearing at birth or later. Scarring and prurigo are most prominent on the shins. Treatment is unsatisfactory. We report three such cases: two of them first cousins, are described with history of blisters since childhood, followed by intensely pruritic lesions predominantly on the shins, and dystrophy of toenails, but no albopapuloid lesions or milia. Intact blisters were present in one case, and excoriations were seen in the other two. All of them showed encouraging response to cryotherapy.
Das
Jayanta Kumar
JK
Department of Dermatology, Ramakrishna Mission Seva Pratisthan Hospital, Kolkata, India. jayanta_das@hotmail.com
Sengupta
Sujata
S
Gangopadhyay
Asok Kumar
AK
D000293:Adolescent; D000328:Adult; D017679:Cryotherapy; D004820:Epidermolysis Bullosa / Q000175:diagnosis / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male