EPIDERMOLYSIS BULLOSA PRURIGINOSA

By Gary M. White, MD


Epidermolysis Bullosa Pruriginosa (EBP) is a rare subtype of dystrophic epidermolysis bullosa (DEB) characterized by intense pruritus, nodular or lichenoid lesions, and violaceous linear scarring, most prominently on the extensor extremities, especially the shins.

Clinical

Initially, erosions and bulla may be seen. Later, lichenified papules and linear plaques from scratching develop. The lesions may be very pruritic. Bulla and lichenoid violaceous papules may be occur together on the anterior aspects of the lower legs. DIF is negative. There may be scattered lesions elsewhere. The nails may be dystrophic.

Treatment

The typical therapy as provided for EB patients is needed. See epidermolysis bullosa. The pruritus may be treated with topical and intralesional steroids. Skin grafts have occasionally been needed.

References

A 12-year-old girl with low IQ with intense pruritic lesions on the leg for the past 10 years.
Pretibial dystrophic epidermolysis bullosa pruriginosa: A rare case report in a child with low intelligent quotient. Indian J Paediatr Dermatol 2016;17:32-4

Pretibial epidermolysis bullosa Dermatology Online Journal 14(10)

Lichen amyloidosis was coexistent in his case. Indian J Dermatol Venereol Leprol 2015;81:40-2

Indian Dermatol Online J. 2014 Jan-Mar; 5(1): 44–47

RegionalDerm

Homepage | FAQs | Contact Dr. White


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.