S6C34bP28bCicatricialEB

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S6C34bP28B-1: This cell-poor, sub-epidermal bulla is cicatricial. The “papillary dermis” is widened and fibrotic; it contains an increased number of vessels in granulation tissue-like patterns. Small keratin-filled cysts in the dermis are milia; they probably are derived from interrupted sweat ducts. The floor of the defect in this area is straight; the dermal papillae are effaced. This alteration of the contour of the surface of the papillary dermis is evidence of scarring (cicatricial epidermolysis bullosa). The clinician with the responsibility for the care of the patient classified the process after work-up as “epidermolysis bullosa acquisita;” I have no information regarding the immunologic findings that would support this interpretation.

S6C34bP28b-2 (EBA): This portion of the lesion also has cicatricial qualities; the interface between the epidermis and the dermis is straight; rete patterns are effaced. A sweat duct centrally shows epithelial hyperplasia. Green arrows identify two small sub-epidermal clefts, one of which appears to be at the tip of a dermal papilla.

S6C34bP28b-3: In this area, the bulla is sub-epidermal. One, detached extremity of a rete ridge is present among two neighboring dermal papillae just to the right of the center of the field. The epidermis shows cytopathic changes with clusters of necrotic keratinocytes. The dermal papillae are preserved; the papillary dermis is irregularly widened in a vertical direction.Scarring is not as prominent in this field as in S6C34bP28b-2. The lesion is cell-poor (cicatricial EBA).

S6C34bP28b-4(EBA): Small clefts are present at the dermal-epidermal interface (green arrows). They mostly are present over dermal papillae. The sweat duct to the right shows epithelial hyperplasia. Vessels are ectatic.

S6C34bP28b-5 (EBA): Strands of connective tissue project along the surface of the otherwise denuded dermal papillae.

S6C34bP28b-6 (EBA): The defect to the right has dermolytic qualities; activated fibroblasts extend to the surface of the defect. Some of the cells may represent activated histiocytes. In the dermal papilla to the left, there is a single, lightly stained colloid body.

S6C34bP28b-7(EBA): The floor of the defect, in an area of scarring, is represented. The change in the character and cellularity of the fibrous matrix along the boundary defined by green arrows might be characterized as an interface (an increment line). The relatively acellular fibrous matrix along the surface of the defect has been deposited in apposition (accretion) to the well-vascularized fibrous matrix beneath the row of green arrows. The surface of the defect is straight. The lesion has a cicatricial quality.

S6C34bP28b-8 (EBA): The margin of the sub-epidermal defect is to the right. At the extremity of the rete ridge, connective tissue is poor in collagen; the changes suggest lysis of connective tissue in this area and in the neighboring dermal papilla. The pattern is cell-poor even at the margin of the defect.

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