Fox-fordyce's diseaseL75.2

Fox and Fordyce, 1902

Rare, inflammatory disease of the apocrine sweat glands, clinically characterized by skin-coloured or slightly reddish, intensely itchy papules attached to the sweat gland ducts. The itching is intensified during physical exertion associated with sweating.

Closure of the apocrine ducts by hyperkeratotic plug, congestion of secretion, rupture of the duct, secretion leakage into the periadnexal connective tissue, inflammatory reaction.

Family history suggests a genetic component.

Almost exclusively (90%) female patients, postpubertal, premenstrual exacerbations. Improvement during pregnancy, under ovulation inhibitors or postmenopausal.

Mainly armpit region, nipple region, navel region, genital and perianal skin areas. Rarely perineum, navel, inner thighs.

Grouped standing, pinhead-sized, flat or hemispherical, rough, skin-coloured to reddish, 0.2-0.3 cm large, protuberant nodules. Excruciating, localized itching. Localised sweating. Sparse underarm hair.

Closure of the excretory duct of the apocrine gland by a keratotic plug in the uppermost part of the hair follicle. Rupture of the sweat gland, formation of a spongiotic vesicle in the follicle wall (most likely visible in serial incisions). Inflammatory infiltrate in the surrounding dermis.

Syringoma

Lichen simplex chronicus

Atopic dermatitis

Hidradenitis suppurativa

Darier's disease

Treatment results are often unsatisfactory, a standard therapy does not exist. Listed treatment approaches are to be understood as therapy trials with very different success rates in individual cases. The invasiveness of the treatment should be proportionate to the clinical findings.

• Peeling treatment with 0.05%-0.1% vitamin A acid cream or ointment (e.g. Cordes VAS, R256 ) usually has a positive effect on itching and hair growth (less on the papules themselves) and should be the first choice therapy.
• Alternatively, partial successes with glucocorticoids as cream such as 0.5% hydrocortisone cream(Hydro-Wolff, R120 ) or 0.05-0.1% betamethasone cream(e.g. Betagalen, R029 ), methylprednisolone aceponate (Advantan cream), prednicarbate (Dermatop cream), tacrolimus or intralesional injections with triamcinolone acetonide (e.g. Volon A) are described.
• Alternatively, clindamycin in propylene glycol solution (e.g. Sobelin solution), UV light or electrocoagulation may be successful.
• Alternative: 1 publication proves success with local botulinum toxin applications.
• For the prevention of superinfections, local antibiotics such as tetracycline (e.g. Imex ointment) or erythromycin (e.g. acne mycin ointment).

Remember! Instead of Eucerin cum aqua Eucerin anhyd., Eucerin O/W- or W/O can be used as a basis for magisterial prescriptions.

In severe cases and in the absence of success with local therapy, contraceptives with antiandrogenic effects such as cyproterone acetate (e.g. Diane 35) or chlormadinone acetate (e.g. Gestamestrol N) can be tried.

Alternative: Retinoids like isotretinoin (e.g. isotretinoin-ratiopharm; acne normin) initial 0.5 mg/kg bw/day p.o. Reduction of the dose to the lowest possible maintenance dose according to the clinic. Long-term therapy is usually necessary because of recurrences on discontinuation.

In case of resistance to therapy or in severe cases, surgical measures may be necessary: excision of the affected areas with subsequent treatment with swivel valves or skin transplants leads to definitive healing.

FFD is relapsing and remitting; a significant improvement in the course of the disease can be expected after the menopause (Salloum A et al. 2022). Spontaneous healing possible after the menopause.

1. Bernad I et al. (2014) FoxFordyce disease as a secondary effect of laser hair removal. J Cosmet Laser Ther 16:141-143
2. Chae KM et al. (2002) Axillary Fox-Fordyce disease treated with liposuction-assisted curettage. Arch Dermatol 138: 452-454
3. Effendy I et al. (1994) Fox-Fordyce disease in a male patient-response to oral retinoid treatment. Clin Exp Dermatol 19: 67-69
4. Feldmann R et al. (1992) Fox-Fordyce Disease: Successful Treatment with topical Clindamycin in alcoholic Propylene glycol solution. Dermatology 184: 310-313
5. Fox GH, Fordyce JA (1902) Two cases of a rare papular disease affecting the axillary region. Journal of Cutaneous and Genitourinary Diseases (Chicago) 20: 1-5
6. Ghislain PD et al (2002) Itchy papules of the axillae. Arch Dermatol 138: 259-264
7. González-Ramos J et al. (2016)Successful treatmeant of refractory pruritic Fox-Fordyce disease with botulinum toxin type A. Br J Dermatol 174: 458-459
8. Hanner S et al. (2018) Axillary and perimammillary Fox-Fordyce disease (apocrine miliaria) in a 19-year-old female patient. Dermatology 69: 313-315
9. Kamada A et al. (2003) Apoeccrine sweat duct obstruction as a cause for Fox-Fordyce disease. J Am Acad Dermatol 48: 453-455
10. Mayser P et al (1993) Fox-Fordyce disease (apocrine miliaria). Dermatologist 44: 309-311
11. Kaya Erdoğan H et al (2015) Clinical Effects of Topical Tacrolimus on Fox-Fordyce Disease. Case Rep Dermatol Med 2015:205418

12. Salloum A et al. (2022) Pathophysiology, clinical findings, and management of Fox-Fordyce disease: A systematic review. J Cosmet Dermatol 21:482-500.