| • | sub-epidermal (below the lamina densa) IgG; antigen = type VII collagen |
Clinical:
| • | appear to be at least 4 clinical presentations: |
“classic” (dystrophic EB-like)
| • | a mechanobullous disease marked by skin fragility |
| • | intact tense blisters on non-inflamed skin |
| • | distribution - acral and trauma prone surfaces: dorsal hands, knuckles, elbows, knees, sacrum, toes |
| • | blisters heal with scarring and formation of milia |
| • | reminiscent of PCT when mild and dystrophic EB when severe |
Bullous Pemphigoid-like
| • | probably the most common presentation |
| • | widespread inflammatory vesicobullous eruption involving trunk, central body and skin folds as well as extremities |
| • | patients often complain of pruritis and do not demonstrate prominent skin fragility, scarring, or milia formation |
| • | typically smolders into a more non-inflammatory mechanobullous form |
Cicatricial Pemphigoid-like
| • | predominant mucosal involvement |
| • | while both classic and BP-like MAY have mucosal involvement |
Brunsting-Perry-like
| • | head and neck distribution, with little or no mucosal involvement |
Histology:
| • | DIF: linear band at dermal-epidermal junction consisting predominantly of IgG (identical to BP) |
| • | the degree of inflammatory infiltrate within the dermis usually reflects the degree of inflammation of the lesion observed clinically |
see also Basic Science - type VII collagen
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