Ear Nose Throat J. 2016 Sep;95(9):370.
FIRST PARAGRAPH: Hailey-Hailey disease is a genodermatosis characterized by incomplete penetrance of an autosomal dominant disorder with defects in the ATP2C1 gene. Patients present with well-demarcated, erythematous plaques that often show crusting. The plaques may be vegetative, commonly associated with a distinct malodor. There may be associated burning or itching. While intertriginous areas are most commonly affected, the neck is also frequently involved. There is a natural remission and exacerbation cycle, with heat, moisture, and trauma the most common exacerbating factors. The lesions may be managed by corticosteroids or antibiotics, along with retinoids for severe disease.
PubMed ID: 27657314
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