Hailey–Hailey Disease (Familial Benign Chronic Pemphigus)

• Figure 2.11 A: Chronic bullous disease of childhood (Courtesy of Dr. Michelle B. Bain) B: Hailey–Hailey disease (Courtesy of Dr. Paul Getz) C: ILVEN (Courtesy of Dr. Paul Getz)

  AD, ATP2C1 gene (encodes Golgi-associated Ca2+ ATPase hSPCA1), results in abnormal intracellular calcium signaling; onset typically second to third decade
• Presents with flaccid vesicles initially on erythematous base over intertriginous areas, ruptures easily, and gives rise to macerated or crusted erosions
• Histology: extensive epidermal acantholysis “dilapidated brick wall”