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Case 501 - Discussion

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

 

Diagnosis: Grove's disease

 

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