Published on 18/03/2015 by admin
Filed under Dermatology
Last modified 18/03/2015
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Rajani Nalluri and Ian Coulson
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, results 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 prior to puberty, after the 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 is usual, although rarely it can be exacerbated by hyperhidrosis. Improvement in pregnancy and during the administration of the oral contraceptive pill has led to speculation regarding an endocrine etiology, but this has been unsubstantiated by blood sex hormone investigations. Very few reports in twins and within families suggest a possible genetic component. It can occur following laser hair removal and has been reported in Turner syndrome.
There are no controlled trials of any agents in Fox–Fordyce disease.
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 has been reported to reduce itch, but its 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 help. 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.
Biopsy
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.
Böer A. Am J Dermatopathol 2004; 26: 482–92.
An exhaustive review of the subtleties of the dermatopathology of Fox–Fordyce disease.
Macarenco RS, Garces SJC. Am J Dermatopathol 2009; 31: 93–7.
Apocrine gland dilation may be used as a low-power magnification clue which should be followed by a search for further histological changes to confirm or rule out the diagnosis.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
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