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The apocrine sweat glands are located in the armpits, areola, pubis and external genital organs. Their excretory duct empties into the follicular infundibulum above that of the sebaceous gland. The intra-dermal portion of the apocrine duct becomes blocked, preventing release of sweat and sometimes causing a discrete local inflammatory reaction which is the source of the papular rash described by G. H. Fox and J. A. Fordyce in 1902. The pathogenesis of this apocrine miliaria has not been fully elucidated and the mechanisms leading to follicular obstruction are not yet understood.
Fox-Fordyce disease presents as a scattering of small, extremely itchy, slightly translucent or normal flesh-coloured follicular papules clustered preferentially in the areas where apocrine glands are found. Itching and the chronic nature of the condition often cause changes in the surrounding skin, which may become darker and lichenified. It mainly affects younger women (90 percent of cases) and flares up, sometimes by stress or menstrual cycles, suggesting the condition is determined by the intervention of endocrine factors. The differential diagnoses are lichen planus and, above all, acanthosis nigricans.
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