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Fox – Fordyce disease
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Obliteration of the follicular infundibulum with keratin in the apocrine gland–bearing skin is the cause of this rare, paroxysmally intensely itchy condition. Apocrine sweat retention and rupture of the gland duct under periods of apocrine sudomotor stimulation, particularly emotional stress, result in the development of an itchy, spongiotic intraepidermal vesicle. It mainly affects women between the ages of 13 and 35 years but has rarely been reported before puberty, after menopause, and in men. Itchy, dome-shaped, flesh-colored or keratotic papules that develop peripubertally in the apocrine areas of the axillae, pubic, periumbilical, and periareolar skin characterize this condition. Sparsity of axillary hair and hypohidrosis are usual, although rarely the disease can be exacerbated by hyperhidrosis. Improvement observed in pregnancy and during the administration of oral contraceptives has led to speculation regarding an endocrine etiology, although this has been unsubstantiated by blood sex hormone investigations. Very few reports in twins and within families suggest a possible genetic component. Fox–Fordyce disease (FFD) can occur after laser hair removal and has been reported in Turner syndrome.
Management Strategy
There are no controlled trials of any agents in FFD.
Topical and intralesional corticosteroids are frequently tried and may be of limited benefit, but atrophy in the axillary area will limit their potency and duration of use. Topical tretinoin and adapalene have been reported to reduce itch, but alternation with a mild corticosteroid may be needed to reduce retinoid irritancy. Clindamycin lotion may be of help. The oral contraceptive pill ( OCP ) may bring relief to some women. Oral isotretinoin may give temporary relief. Electrocautery and excision of the periareolar skin may offer permanent solutions. A recent report advocates an ingenious method of removal of the apocrine glands using a microliposuction cannula. There is a single report of axillary disease responding to intralesional botulinum toxin injection. Physical modalities such as microwave devices and CO 2 and erbium glass lasers have their advocates.
Fox–Fordyce disease: diagnosis by transverse histologic sections
Stashower ME, Krivda SJ, Turiansky GW. J Am Acad Dermatol 2000; 42: 89–91.
Transverse sectioning demonstrates the follicular plugging and infundibular spongiosis more readily than conventional sections.
Dilation of apocrine glands. A forgotten but helpful histopathological clue to the diagnosis of axillary Fox-Fordyce disease
Macarenco RS, Garces S JC. Am J Dermatopathol 2009; 31: 393–7.
Apocrine gland dilation may be used as a low-power magnification clue, which should be followed by a search for further histologic changes to confirm or rule out the diagnosis.
Clinicopathological study of Fox–Fordyce disease
Kao PH, Hsu CK, Lee JY. J Dermatol 2009; 36: 485–90.
Focal spongiosis in the upper infundibulum with perifollicular fibrosis and lymphohistiocytic infiltrate were consistent features in their case series.
Fox–Fordyce-like disease following laser hair removal appearing on all treated areas
Helou J, Maatouk I, Moutran R, et al. Lasers Med Sci 2013; 28: 1205–7.
The most recent of reports highlighting that laser epilation in several anatomic sites may induce FFD.
Perifollicular xanthomatosis as a key histologic finding in Fox–Fordyce disease
Mataix J, Silvestre JF, Niveiro M, et al. Actas Dermosifiliogr 2008; 99: 145–8.
There are occasional conditions to consider in the differential diagnosis! There is even controversy as to whether perifollicular xanthomatosis is part of the spectrum of this disorder.
A new treatment of Fox–Fordyce disease
Helfman RJ. South Med J 1962; 55: 681–4.
A single report of successful symptom relief of axillary lesions with 10 mg/mL triamcinolone diluted with an equal volume of 1% lidocaine to four sites on nine occasions over 3 months.
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