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Therapeutic Approach

Acantholytic disorders of the skin are a heterogeneous group of disorders characterized by the loss of intercellular contacts between suprabasal keratinocytes, or acantholysis. The three major acantholytic disorders of the skin are Darier-White disease (DD), Hailey-Hailey disease (HHD), and Grover disease (GD). DD and HHD are autosomal dominant disorders, whereas GD is a sporadic disease of unknown etiology that is mostly seen in patients with sun-damaged skin. While clinical trial data are needed to determine disease-specific therapies, therapeutic strategies are aimed at reducing inflammation, relieving symptoms, and prevention of secondary infections. Treatment for localized inflammation should be with topical monotherapy or combination therapy with corticosteroids, antibiotics, calcineurin inhibitors, and retinoids. Treatment for generalized disease include systemic agents such as oral acitretin and isotretinoin. See Table 36-1.

Table 36-1Acantholytic Disorders of the Skin Treatment Table

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