BART-SYNDROME - DISEASE ENTITY OR VARIANT OF EPIDERMOLYSIS-BULLOSA

Bart syndrome was described first by Bart in 1966; it represents the c ombination of congenital epidermolysis bullosa, congenital localized a bsence of skin affecting the extremities and shedding or dystrophy of nails. This syndrome may be of clinical relevance because of its more favourable prognosis in comparison with other forms of epidermolysis b ullosa. We report two patients with Bart syndrome and focus on the que stion, if this syndrome represents a distinct entity or a variant of e pidermolysis bullosa.