Fox-Fordyce disease
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Etiology
- idiopathic
- hormonal component suspected (see epidemiology)
Epidemiology
- rare
- primarily affects women
- resolution during pregnancy, with oral contraceptive use, & after menopause
Pathology
- inflammatory condition of skin regions containing apocrine glands
- pruritic, dome-shaped, monomorphic, skin-colored, folliculocentric papules
- most commonly affects the axilla but can also affect the areola, umbilicus, & anogenital regions
- consensus is that infundibular plugging causes apocrine ducts to rupture, eliciting inflammation
- includes apocrine sweat gland occlusion[1]
- histologic features include hyperkeratosis, acanthosis, infundibular dilation & spongiosis, perifollicular fibrosis & lymphohistiocytic inflammation.
- perifollicular foamy histiocytes (containing lipid, mucin, or both) may be the most distinctive histopathologic feature
- Fox-Fordyce Disease of the breast (case report)[2]
- low-power microscopy: (image)[2]
- dilated follicular opening, focal hyperkeratosis, perifollicular infiltrate
- higher-power microscopy: (image)[2]
- lymphocytic inflammation with prominent foamy histiocytes
- increased dermal mucin surrounding the hair follicle & adjacent eccrine duct
- low-power microscopy: (image)[2]
Clinical manifestations
- patients often exhibit a waxing & waning course
- Fox-Fordyce Disease of the breast (case report)[2]
- 6-month history of intermittent pruritus of the breast.
- skin-colored, monomorphic to slightly hyperpigmented follicle-based papules just outside the areola (image)[2]
Management
- triamcinolone 0.1% ointment twice daily as needed for symptom relief[2]
- topical or oral retinoids, benzoyl peroxide wash, topical clindamycin, topical or intralesional corticosteroids, topical calcineurin inhibitors, oral contraceptives, pulsed dye laser, fractional CO2 laser reportedly effective[2]
More general terms
References
- ↑ 1.0 1.1 Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 398
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Davis MI et al Fox-Fordyce Disease of the Breast. Mayo Clinic Proceedings. 2025. Jan 100(1):109-110 PMID: https://pubmed.ncbi.nlm.nih.gov/39755421 https://www.mayoclinicproceedings.org/article/S0025-6196(24)00550-0/fulltext
- ↑ Salloum A, Bouferraa Y, Bazzi N et al Pathophysiology, clinical findings, and management of Fox-Fordyce disease: A systematic review. J Cosmet Dermatol. 2022 Feb;21(2):482-500. PMID: https://pubmed.ncbi.nlm.nih.gov/33817950 Review.