Koebner's epidermolysis bullosa simplex.

Introduction

Epidermolysis bullosa comprises of a group of genetically determined skin fragility disorders char-acterized by blistering of the skin and mucosa fol-lowing mild mechanical trauma. Epidermolysis bullosa simplex is the most frequent form of epider-molysis bullosa. Inheritance is usually autosomal dominant, and only rarely autosomal recessive forms are seen. A rare form of epidermolysis bullosa sim-plex where the bullae are serous, tense and heal without scarring, and sparing the hair, teeth, and nails, which are normal, is Koebner′s epidermolysis bullosa simplex.

Case Report

A 26- year- old male, who had been suffering from vesico- bullous lesions since 4 days of his birth presented with tense bullous lesions over the hands, feet and acromian spine. Lesions ranged from 0.5 cm - 5 cms in size. Some of these lesions subsided spontaneously, while the rest got secondarily in-fected to become painful. The tense lesions became flaccid and ruptured in due course of time. These left areas of depigmentation but scarring was ab-sent. -

These lesions which were more severe in child-hood and now had gradually become less severe and less numerous, showed exacerbations during the months between summer and rainy seasons and improved thereafter to recur in the next year.

The nails were normal though the teeth were discolored but not dystrophic. No other complaints were present and the patient was in good health otherwise. Family history was negative. Routine in-vestigations were carried out and were found to be normal. Histology of the lesions was suggestive of epidermolysis bullosa.

Discussion

Epidermolysis bullosa simplex has various sub-types of which Koebner′s epidermolysis bullosa sim-plex is quite rare. Clinical features of this subtype and that of the patient mentioned above show simi-larities to a great extent, except that the mode of transmission mentioned in literature which is auto-somal dominant, could not be substantiated in this patient.