A 20-year-old woman develops these moist erosions in both axilla.
HAILEY-HAILEY DISEASE
Hailey-Hailey Disease (HHD), AKA benign familial pemphigus, is a rare, autosomal dominant genodermatosis characterized by erosions, vesicles, and bulla predominantly in the flexural areas.
- Thenset is after puberty in the second or third decade of life
- A positive family history is detected in around two‐thirds of cases
- Mutations in the ATP2C1 gene, which encodes a Ca2+ ATPase pump, lead to changes in Ca2+-dependent intracellular signaling, resulting in loss of cell adhesion in the epidermis.
Wet tissue paper erosions or erythematous plaques are found symmetrically in the intertriginous areas where the skin surfaces oppose each other. Most common sites in descending order are groin, axilla, perineum, inframammary region, umbilicus, and retroauricular area. The skin lesions are worsened by friction, heat, injury and sun exposure.
Close up of the axilla.
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