SPONTANEOUS DISAPPEARANCE OF INTRAEPIDERMAL TYPE-VII COLLAGEN IN A PATIENT WITH DYSTROPHIC EPIDERMOLYSIS-BULLOSA

Recently, a peculiar self-healing neonatal blistering disease has been reported, which is characterized by perinuclear stellate inclusions within basilar keratinocytes, representing abnormal retention of type VII collagen. We report a Japanese patient with this condition, in whom we studied the expression of a variety of basement membrane zone (BMZ)-related antigens. Skin biopsy specimens at 3 days of age showed abundant accumulation of both the NC-1 domain and the collagenous part of type VII collagen within the basal and suprabasal keratinocytes, in addition to patchy and weak staining along the BMZ, In contrast, at 4 years of age, when the disease activity was markedly attenuated, a second biopsy showed complete linear staining of type VII collagen along the BMZ, with no detectable intracytoplasmic deposits, Expression of other BMZ-related antigens, including laminin 5, alpha 6 and beta 4 integrins, bullous pemphigoid antigens 1 and 2, and type TV collagen, was normal in both the biopsy specimens, Our observations further confirm that the perinuclear stellate bodies seen in this peculiar condition are composed of both collagenous and non-collagenous domains of type VII collagen retained within the epidermis, and that these bodies disappear when the disease activity remits.