Epiderrmolysis bullosa pruginosa

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Presentation transcript:

Epiderrmolysis bullosa pruginosa

Epidermolysis bullosa pruriginosa Dystrophic epidermolysis bullosa (DEB) pruriginosa (DEB-Pr) is a rare variant of DEB due to COL7A1 mutations. Both AR and AD inheritance and sporadic inheritance patterns have been recognized. EB pruriginosa presents either at birth or during infancy or childhood but in some cases appears later.

Milia, nail dystrophy and intact blisters are rarely seen. Clinically There are severe itching and violaceous papular and nodular lesions, often in a linear arrangement , mainly on extremities. Milia, nail dystrophy and intact blisters are rarely seen. The cause of severe itching is unknown. DD Lichen simplex chronicus. Hypertrophic LP. Cutaneous amyloidosis. Dermatitis artefacta.

Potent topical steriods. IL triamcinolone. Topical tacrolimus. Treatment is aimed at controlling itching and halting the progression of cutaneous lesions and includes : Potent topical steriods. IL triamcinolone. Topical tacrolimus. Antihistamines. Systemic steriods. Cyclosporin. Thalidomide.

A 25-year-old male patient presented with itchy skin lesions of 5 years duration, with gradual onset and progressive course. The patient gave family history with similar condition.

E/M LL BC LD 11