Fox-fordyce's disease L75.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 27.12.2020

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Synonym(s)

acanthosis circumporalis pruriens; apocrine miliaria; Apocrinitis sudoripara pruriens; Fox Fordyce disease; hidradenoma eruptivum; Iridoneurodermite axillary Audry

History
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Fox and Fordyce, 1902

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

Etiopathogenesis
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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.

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

Localization
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Mainly axillary region, nipples, navel region, genital and perianal skin areas. Rarely perineum, navel, inner thighs.

Clinical features
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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.

Histology
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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.

Differential diagnosis
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General therapy
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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.

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

Internal therapy
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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.

Operative therapie
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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.

Progression/forecast
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Spontaneous healing after menopause.

Literature
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  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 perimamillary Fox Fordyce disease (apocrine miliaria) in a 19-year-old female patient Dermatologist 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

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 27.12.2020