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 w ithin basilar keratinocytes, representing abnormal retention of type V II 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 abun dant accumulation of both the NC-1 domain and the collagenous part of type VII collagen within the basal and suprabasal keratinocytes, in ad dition to patchy and weak staining along the BMZ, In contrast, at 4 ye ars of age, when the disease activity was markedly attenuated, a secon d biopsy showed complete linear staining of type VII collagen along th e BMZ, with no detectable intracytoplasmic deposits, Expression of oth er BMZ-related antigens, including laminin 5, alpha 6 and beta 4 integ rins, bullous pemphigoid antigens 1 and 2, and type TV collagen, was n ormal in both the biopsy specimens, Our observations further confirm t hat the perinuclear stellate bodies seen in this peculiar condition ar e composed of both collagenous and non-collagenous domains of type VII collagen retained within the epidermis, and that these bodies disappe ar when the disease activity remits.