Clinical spectrum of epidermolysis bullosa acquisita / 대한피부과학회지

ABSTRACT

The clinical spectruni of epidermolysis bullosa acquisita(EBA) is much broader than originally thought. Although the full extent of the clinical presenation is still being defined, it is now known that EBA include the followings a non-inflammatory mechanobullous condition equating wit,h classical EBA; an inflammatory vesiculosullous eruption mimicking bullous pernphigoid; and a mucosal-centered disease with sarring similar to cicatricial pemphigoid. Among the nine cases of EBA, aged between 34 to 70 year-old, seen in recent years, three patients had mechanobullous lesions with skin fragilities and scarrings; three patients had inflammatory bullous eruptions, and three other patient had combined features of mechanobullous/inflammatory bullous lesions. Mucous membrane lesions were recognized in sex cases, and the rnos! frequent site of involvement was the oral mucosae. According to observations of these patients episodes of inflammatory bullous eruptions appeared to be present in seven cases and have been considered as early sympoms of the disease. It has been noted, however, that in two cases lesions develop d as an non-inflammatory mechanobullous from thonset. Based on the ability of EBA to mimick bullous pemphigoid or cicatricial pernphogoid and the fact that such cases have perhaps been missed, we feel EBA is more common than past literature has suggested.