RT Book, Section A1 Burge, Susan A1 Hovnanian, Alain A2 Goldsmith, Lowell A. A2 Katz, Stephen I. A2 Gilchrest, Barbara A. A2 Paller, Amy S. A2 Leffell, David J. A2 Wolff, Klaus SR Print(0) ID 56036310 T1 Chapter 51. Acantholytic Disorders of the Skin T2 Fitzpatrick's Dermatology in General Medicine, 8e YR 2012 FD 2012 PB The McGraw-Hill Companies PP New York, NY SN 978-0-07-166904-7 LK accessmedicine.mhmedical.com/content.aspx?aid=56036310 RD 2024/03/28 AB |PrintAcantholytic Diseases at a GlanceThe acantholytic diseases are a heterogeneous group of diseases with overlapping clinical and histological features.Darier (or Darier–White) disease (DD) and Hailey–Hailey disease (HHD) are autosomal dominant disorders that are caused by defective calcium pumps—a sarco/endoplasmic reticulum pump in DD and a Golgi apparatus pump in HHD.Typical DD presents with greasy keratotic papules in a seborrheic distribution while HHD is characterized by painful oozing erosions in flexures and at sites of trauma.Signs in the nails (DD, HHD), flat-topped warty papules on dorsa of hands and feet (DD) and palmar pits (DD, HHD) or palmar keratotic papules (DD) help to confirm the diagnosis.Hypertrophic malodorous flexural disease is particularly disabling in DD and HHD.Grover disease (GD) is a sporadic papular condition of uncertain etiology that presents most often in sun-damaged skin. Intractable pruritus is common.Histopathological examination of involved skin in DD, HH, and GD reveals breakdown of intercellular contacts between suprabasal keratinocytes (acantholysis) with variable dyskeratosis.Acrokeratosis verruciformis of Hopf (AKV) is autosomal dominant and characterized by signs that mimic acral DD: flat-topped warty papules on dorsa of hands and feet and nail dystrophy. The histology in AKV is not acantholytic, but some (if not most) cases may be limited variants of DD.Treatment options for these diseases include topical corticosteroids (DD, HHD, GD) and topical or oral retinoids (DD, GD, AKV).