EPIDERMOLYSIS-BULLOSA SIMPLEX HERPETIFORMIS OF DOWLING-MEARA WITH MOTTLED PIGMENTATION - THE RELATIONSHIP BETWEEN EBS HERPETIFORMIS AND EBSWITH MOTTLED PIGMENTATION

A 6-year-old girl with epidermolysis bullosa simplex (EBS) is describe d, Clinical features include generalized herpetiform blistering of the skin, healing without scars, intraoral blistering, nail dystrophy, pa lmoplantar keratoderma, and improvement with age, An unusual feature w as the presence of a striking mottled pigmentation involving the arms, trunk, and legs, Histology, immunofluorescence, and electron microsco py of a fresh lesion showed an intraepidermal split within the basal k eratinocytes, focal hyperpigmentation of the basal cells without an in flammatory infiltrate, and tonofilament clumping, Our patient illustra tes the clinical features of Dowling-Meara EBS with the pigmentary cha nges of EBS with mottled pigmentation, The relationship between EBS of Dowling-Meara and EBS with mottled pigmentation is discussed.