S6C23P17-Cicatricial Pemphigoid

S6C23P17-1(cicatricial pemphoigoid): At the margin of this vesicle, a defect (to the right) between the papillary dermis and the partially detached epidermis has been inlaid with loose fibrous tissue. Blue arrows, in the region of the defect, identify dendritic fibroblasts among the connective tissue fibers of the newly formed fibrous tissue (organization and accretive growth). There are also loose infiltrates of lymphocytes and histiocytes. A basal layer is partially preserved but, to the right and above, there are defects among basal keratinocytes.

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S6C23P17-2: With this Verhoeff-van Gieson stain, connective tissue is red. Blue arrows are distributed in an attempt to define the basement membrane zone. The defect is undergoing organization: fibroblasts are loosely spaced in the defect, as evidence of early organization.

S6C23P17-3: There is a subepidermal vesicle. Fibrin is condensed in the defect; it stains yellow with this Verhoeff-van Gieson stain. Dermal papillae are partially digested and, in areas, the capillary of a papilla is outlined by a thin sheath of fibrous tissue. The papillary dermis is widened and edematous. It is free of elastic fibers (the process is in part elastolytic). Collagen bundles near the interface between the reticular dermis and the papillary dermis are delicate and lightly stained (they are partially digested). There is a paucity of elastic fibers in the upper portion of the reticular dermis (cicatricial pemphigoid ).

S6C23P17-4: Red arrows identify the “dermal-epidermal” interface in this lesion of cicatricial pemphigoid. Some of the dermal papillae (red arrows) are thin and partially digested (reticular fibers are reduced in the vicinity of the capillaries at the tips of the altered dermal papillae on this reticulum stain). The blue arrows outline a small area of delicate reticular fibers, apparently in the lumen of the subepidermal defect. The reticular fibers in this area are a marker for early organization of the condensed fibrin in the lumen of the defect.

S6C23P17-5: At higher magnification, the lysis of reticular fibers is focal. Some of the dermal papillae are thin; their contours are distorted. Blue arrows point to the limits of the domain of reticular fibers of the original papillary dermis. Clearly, there is irregular lysis of connective tissue fibers. The area outlined by red arrows is a marker for the formation of new reticular fibers, as a product resulting from the organization of fibrin in the subepidermal defect; failing acceptance of this explanation, the lesion would qualify as a dermolytic process.

 

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