Epidermolysis bullosa pruriginosa: a rare presentation with asymptomatic lesions.

[dystrophic epidermolysis bullosa]

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 COL 7 A 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 .

Diseases
Validation

Diseases presenting "pruritus" symptom

benign recurrent intrahepatic cholestasis

cholangiocarcinoma

cutaneous mastocytosis

dystrophic epidermolysis bullosa

erdheim-chester disease

lamellar ichthyosis

pleomorphic liposarcoma

pyomyositis

zellweger syndrome

This symptom has already been validated