Fox–Fordyce disease

Published on 18/03/2015 by admin

Filed under Dermatology

Last modified 22/04/2025

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Fox–Fordyce disease

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

image

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.

Specific investigations

First-line therapies

image Topical and intralesional corticosteroids D
image Topical clindamycin E
image Oral contraceptive pill D
image Topical retinoids D
image UVB D
image Topical pimecrolimus D

Second-line therapies

image Oral isotretinoin E
image Electrocautery D
image Excision D
image Apocrine gland removal using microliposuction E

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Fox–Fordyce disease

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

image

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.

Specific investigations

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