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Fox-Fordyce disease (FFD)

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Fox-Fordyce disease (FFD), also known as apocrine miliaria, is a chronic papular eruption that is localized to areas of skin containing apocrine sweat glands

Pruritic skin-colored or slightly erythematous papules typically occur in the axillae and the pubic areas (1):

  • affects the axillae, the pubes, labia, perineum, mammary areolae, and, less frequently, the umbilicus and the presternal area
  • more than 90% of patients are female, between the ages of 13 and 35 years
  • pruritus is very severe, and becomes paroxysmally intense with emotional crises, sexual activity, excitement, and exercise
  • histopathology demonstrates hyperkeratosis and plugging of hair follicles, and dilatation of the apocrine sweat glands and ducts
    • histologic features of FFD are a keratotic plug in the follicular orifice, and spongiosis of the infundibular epithelium at the level of entry of the apocrine duct

Although the eruption is distinctive in distribution, the clinical differential diagnosis includes infectious folliculitis, contact dermatitis, scabies, arthropod bites, syringomas, lichen planus, lichen nitidus, and chronic dermatitis. Histologic confirmation is thus important to exclude other diagnostic possibilities.

Management and prognosis (2):

  • there are no reported cases of spontaneous resolution, and there is no known cure for Fox-Fordyce disease
  • many treatment strategies have had poor success and include oral contraceptives; oral tretinoin; topical retinoids, antibiotics, and steroids; intralesional triamcinolone; ultraviolet light; and dermabrasion
  • irritation from topical retinoids generally limits long-term therapy
  • successful treatment with clindamycin in alcoholic propylene glycol solution has been reported
  • surgical excision has been suggested for the treatment of recalcitrant cases

Click here for an example image of this condition

Reference:


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