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Case 493 - Discussion

Uploaded: 2011-05-20, Updated: 2011-05-20

 

Diagnosis: Hailey-Hailey disease

Description: The skin biopsy shows a mild superficial perivascular lymphocytic infiltrate. The epidermis is focally hyperplastic with suprabasal acantholysis and scattered dyskeratotic suprabasal keratinocytes, detached from each other. Overlying the hyperplastic and acantholytic epidermal foci, compact orthohyperkeratoses with focal mild parakeratosis is noted.

 

Hailey-Hailey disease (Benign Familial Pemphigus)

  • AD, ATP2CI gene, 3q21-24
  • Intertriginous vesicles, crusts and erosions on the axilla or neck.
  • HE:

         Extensive suprabasal bullae and acantholysis affecting all epidermal layers (dilapidated brick wall)

         Upper epidermis held loosely together by intercellular bridges

         Subtle dyskeratosis, well-defined nucleus, preserved cytoplasm

         Rare corp ronds and grains

 

Darier’s Disease (Darier-White Disease or Keratosis Follicularis)

  • Autosomal dominant, ATP2A2, 12q23-24.1
  • Trunk, oral, hands/feet, palms/soles
  • HE:

         Suprabasal acantholysis dyskeratosis

         Forming clefts and proliferation of villi

         Dyskeratosis resulting in corp ronds and grains

         Papillomatosis, acanthosis, hyperkeratosis

 

Grover’s Disease (Focal/Transient Acantholytic Dermatosis)

  • Small, discrete, pruritic, on the trunck
  • HE:
    • Smaller and more circumscribed than other acantholytic diseases
    • Acantholysis dyskeratosis, suprabasalar clefting, minimal necrosis
  • DIF: negative
  • DDX: Darier’s disease, Hailey–Hailey disease, pemphigus vulgaris