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Chapter 75: Fox-Fordyce Disease

Stephen M. Squires; Garrett N. McCoy; Anthony V. Benedetto

FOX-FORDYCE DISEASE

Therapeutic Approach

Fox-Fordyce disease (FFD) is a rare inflammatory eruption of the apocrine glands that usually presents in females (9:1) between 13 and 35 years of age. It presents as small monomorphic skin-colored to yellow peri-follicular papules. Most commonly reported in the axillae (AX), periareolar (PA), anogenital (AG), or periumbilical (PU) skin. No definitive treatment for FFD exists, and treatment recommendations are based on case reports or case series. Therapy is usually aimed at treating pruritus. Consideration of unique patient factors should guide management, as most modalities have variable outcomes and questionable long-term efficacy. See Table 75-1.

Table 75-1Fox-Fordyce Treatment Table
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Table 75-1 Fox-Fordyce Treatment Table
MEDICATION NAME INDICATIONa MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
No Therapy
No treatment Asymptomatic N/A None None None IV1-2
Topical Therapy
Topical steroids AX, AG Anti-inflammatory Low-potency cream/oint QD Potential skin atrophy/striae Limit long-term use IV3-4
Calcipotriol betamethasone oint AX, PA, AG Anti-inflammatory 50-500 μg/g QD Potential skin atrophy/striae Limit long-term use IV5
Tretinoin with low-potency topical steroid AX, AG Keratolytic, anti-inflammatory 0.025-0.05% cr, HC1% cr or desonide 0.05% cr QD-TIW None None IV6-8
Tretinoin AX Reduce keratin plugging 0.025%-0.1% cream QD-TIW Skin irritation None IV9-11
Adapalene AX, AG Reduce keratin plugging 0.1% gel QD Skin irritation None IV12-13
Clindamycin AX Anti-bacterial, anti-inflammatory 1% solution QD None None IV14
Clindamycin in propylene glycol AX Propylene glycol is keratolytic 1% solution QD None None IV10,15
Benzoyl peroxide AX, AG, PA Anti-bacterial BP 5% QD for 2 mo None None IV16
Pimecrolimus AX, AG, PU, PA Calcineurin inhibitor, anti-inflammatory 1% BID 8 wk None, skin irritation None IV17-18
Tacrolimus AX Calcineurin inhibitor, anti-inflammatory 0.1% ointment BID 12 wk None None IV19
Neomycin – hydrocortisone lotion AX, AG, PA Anti-inflammatory Neomycin—hydrocortisone 1% lotion QD Not listed None IV20
Systemic Therapy
Oral contraceptives AX Hormonal regulation Norethynodrel with mestranol Not listed None IV7,13,21
Antihistamines AX, AG Antihistamine Loratadine 10 mg/d × 1 mo Sedating None IV4,16,22
Isotretinoin AX Normalize follicular hyperkeratosis, anti-inflammatory 30 mg BID 8 wk, then 15 mg QD 8 wk Xerosis Pregnancy, isotretinoin monitoring IV16,23
Procedural Therapy
Intralesional triamcinolone acetonide AX Anti-inflammatory 10 mg/mL equal volume 1% lidocaine Potential skin atrophy/striae Limit long-term use IV3
Ultraviolet light Not specified Exfoliation reduces keratin plugging, restores duct patency Weekly for 4-6 wk Erythema None IV24
Electrocoagulation AX Destruction of apocrine gland local anesthesia, 3-4 mm depth None None IV25
Excision AX Surgical removal local anesthesia, narrow margins None None IV26
Botulinum toxin A AX Inhibition of sub P, glutamate; chemodenervation of cholinergic glands 100 units injected into each axilla None None IV27
Dermal detachment of areola, excision of apocrine ...

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