Ultrastructural differentiation of epidermolysis bullosa subtypes and porphyria cutanea tarda.
Identifying subtypes of epidermolysis bullosa clinically, particularly at the time of disease onset, can be extremely difficult. In this investigation samples of intact skin from cases of epidermolysis bullosa and porphyria cutanea tarda were examined ultrastructurally and compared with normal tissue. The histological composition of the blister roof and floor surfaces was also evaluated. Three of the ten epidermolysis bullosa subtypes examined revealed specific features. Distinctive, circumscribed, clumped tonofilament bodies were present in basal keratinocytes from epidermolysis bullosa herpetiformis Dowling-Meara. Blister formation in epidermolysis bullosa simplex generalisata gravis occurred immediately above the dermo-epidermal junction aspect of stratum basale cells and thick (30 nm diameter), cross-striated anchoring fibrils were absent in epidermolysis bullosa dystrophica generalisata gravis. Features relating to the lamina densa of the dermo-epidermal junction, dermal capillaries and blister composition were distinctive, but not confined to a particular epidermolysis bullosa subtype or porphyria cutanea tarda.
Jaunzems
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Department of Histopathology, Women's and Children's Hospital, North Adelaide, South Australia.
Woods
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AE
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D004820:Epidermolysis Bullosa / Q000145:classification / Q000473:pathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008875:Middle Aged; D017119:Porphyria Cutanea Tarda / Q000473:pathology*; D012867:Skin / Q000648:ultrastructure*