| • | aka Benign Familial Pemphigus |
pathogenesis:
| • | both Hailey Hailey and Darier’s involve a molecular defect in a Ca dependent ATPase (but Darier’s is chromosome 12q vs. H-H is 3q) |
clinical:
| • | presents in the 3rd to 4th decade |
| • | flaccid vesico-pustules, crusted erosions, or expanding circinate plaques |
| • | areas exposed to friction: sides of neck, axillae, groin, and perineum |
| • | lesions extend peripherally, healing in the center |
| • | flexural disease may be hypertrophic and malodorous with soft, flat , moist vegetations and painful fissures (rhagades) |
| • | mucosal involvement rare |
| • | nails: longitudinal white bands |
ddx: (misdiagnosis frequent)
| • | impetigo/ impetiginized eczema |
| • | candida intertrigo (mnemonic: symmetric, scrotum, and satellites) |
| • | vegetating flexural diseases: |
| • | Darier’s (distinctive nails and hyperkeratotic papules); was once considered to be the vesicular variant of Darier’s (but Darier’s is chromosome 12q vs. H-H is 3q) |
| • | P. vegetans (oral lesions distinguishes) |
histology: “dilapidated brick wall”; DIF (-)
histology ddx: (vs. Darier’s)
| • | epidermal hyperplasia and suprabasal separation occur in both |
| • | but acantholysis tends to be more widespread in fully developed lesions of Hailey-Hailey |
| • | while dyskeratosis with corps ronds and grains is more prominent in Darier’s |
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