Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is a rare autosomal dominant genodermatosis with complete penetrance that affects the ATP2C1 gene, located in the long arm of chromosome 3 and responsible for encoding the calcium ATPase enzyme of the Golgi complex. Defective calcium channels in keratinocytes impair the formation of desmosome adhesion proteins and lead to acantholysis. 1-3 The estimated incidence is 1/50000 individuals, 3 and the condition usually appears between the second and fourth decades of life. 2,4 Clinically, it presents with eroded, macerated, vegetating, malodorous plaques in intertriginous areas. 1,3,4 Secondary infections are frequent and relevant in the exacerbation of the condition. 3,4 We describe the case of a healthy 42-year-old woman who had erythematous, macerated, crusted, painful plaques with a foul odor bilaterally in the inframammary regions for 4 years (Figure 1A). She eventually had similar lesions in the groins, but to a lesser extent.

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