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S6C12bP6b-1: In this example of Hailey-Hailey disease, the areas of acantholysis are spotty in distribution; they are more confluent at the left margin of the specimen. The clefts are mostly suprabasilar; they extend to the interface between the superficial and basal units of the epidermis. Perivascular and peri-appendageal infiltrates of lymphoid cells in the dermis are prominent. In some areas, the basic pattern is psoriasiform.

S6C12bP6b-2: The defects are irregular in distribution; focally, they are suprabasilar and, in other areas, are 3 or more cell layers above the basal layer. Dyskeratosis is a prominent feature. In the more superficial portions of the epidermis, some of the cells resemble “corps ronds,” but do not show prominent keratohyaline granules (Hailey-Hailey disease).

S6C12bP6b-3: Acantholysis affects cells of the basal unit of the epidermis. Dyskeratosis is a prominent feature. Some of the large keratinocytes in the more superficial portion of this field resemble “corps ronds,” but are devoid of keratohyaline granules (Hailey-Hailey disease).

S6C12bP6b-4: A portion of the confluent cleft, as seen in S6C12bP6b-1, is represented in this field. The defect is supra-basilar. The basal unit of the epidermis shows extensive acantholysis (in the pattern of a “delapidated brick wall”). There is focal parakeratosis along  the surface. An eosinophil is present in the dermal infiltrates (Hailey-Hailey disease).

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