INTERSTITIAL LUNG-DISEASE AND PULMONARY-HYPERTENSION ASSOCIATED WITH EPIDERMOLYSIS-BULLOSA IN AN INFANT

Interstitial lung diseases, with or without pulmonary hypertension and epidermolysis bullosa are rare in infancy. Pathogenetic correlations between these diseases are not known and their coincidence has nor bee n reported, yet. We report on a seven weeks old boy of consanguine par ents with typical skin efflorescences of epidermolysis bullosa, tachyd yspnoea and cyanosis. Echocardiography and cardiac catheterisation rev ealed pulmonary hypertension, which persisted under therapy with oxyge n and nifedipin. Lung biopsy showed interstitial and peribronchiolar i ncreased lymphocytes and lymphfollicels, a mild intraalveolar desquama tion and a media hypertrophy of the arteries. A combined therapy of pr ednisone and nifedipine normalised the pulmonary hypertension and the oxygen saturation. The activity of the epidermolysis bullosa showed no correlation with the interstitial lung disease or with the therapy. A connection between both diseases is discussed.