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S6C12aP6a-1: Hailey-Hailey disease is basically a familial disorder affecting the basal unit of the epidermis. It is manifested in rather restricted anatomic sites of the skin. In the basal unit of the epidermis, there is loss of conformational junctions among keratinocytes. In this example, the effects are not uniformly supra-basilar but, in most affected rete ridges, several layers of cells are relatively intact above the basal layer. The suprabasilar patterns are most prominent along the surface of the dermal papillae (inter-rete ridge zones). The superficial unit is intact and hyperplastic; it is associated with a compact, wide, orthokeratotic keratin layer. Melanin deposits are present in the keratin layer. Inflammatory infiltrates in the dermis are mild. The lesion has nevoid qualities.

S6C12aP6a-2: At higher magnification, the acantholytic cells at the interface between the basal and the superficial units are rounded in outline and show cytoplasmic acidophilia; some show a perinuclear halo. Nuclei are enlarged; they show a prominent, central nucleolus. At the extremity of a dermal papilla, the acantholytic cleft is supra-basilar. The granular layer is prominent (Hailey-Hailey disease).

S6C12aP6a-3: In the area of acantholysis, a few intercellular bridges are preserved. The changes may offer support for an interpretation that the dyskeratotic changes precede the acantholytic phenomena.

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