A 27-day-old neonate, product of 3rd degree consanguineous marriage, youngest of three siblings, presented with complaints of absence of skin of medial aspect of both legs and nail dystrophy, and fluid-filled lesions on trauma-prone sites of body since 2nd day of life. Examination revealed absence of skin involving anteromedial aspect of shin and dorsum of both feet (Fig. 1). Nails were dystrophic. Multiple erosions involving buttocks, elbows and occipital scalp were noted. Mucous membranes were normal. Histopathological examination of cutaneous blister revealed subepidermal blister and absence of inflammatory infiltrate on histopathology and immune complex on direct immunofluorescence. A diagnosis of Bart syndrome with underlying Junctional or Dystrophic epidermolysis bullosa was made. Erosions were covered with paraffin gauze dressing, thin layer of mupirocin and non-adherent dressing. Parents were counseled about the favourable outcome of the disease.

Fig.1 Absence of skin over anteromedial aspect of shin and dorsum of both feet in Bart syndrome.

Bart syndrome is rare disorder characterized by triad of congenital localized absence of skin (CLAS), mucocutaneous blistering and nail abnormalities. CLAS is classically present as S-shaped area from knee to anterolateral surface of leg, ankle and foot, and is generally bilateral. Cause of CLAS has been proposed to be rubbing of limbs together in utero. It may represent any of the three subtypes of epidermolysis bullosa: simplex, junctional or dystrophic. Diagnosis is confirmed by histopathological studies, antigen mapping and genetic studies. Prognosis is favourable in absence of other anomalies as localised absence of skin heals with minimal scarring and blistering improves with time.