Perifollicular Xanthomatosis as a Key Histological Finding in Fox-Fordyce Disease

Mataix, J.; Silvestre, J.F.; Niveiro, M.; Lucas, A.; Pérez-Crespo, M.

doi:10.1016/S1578-2190(08)70215-4

Article information

Abstract

Fox–Fordyce disease is a rare skin condition characterized by the presence of multiple pruritic follicular papules in areas rich in apocrine glands, such as the axillae, mammary areolae, or genital regions. There is a high degree of variability in the histological findings seen in Fox–Fordyce disease. In addition to those described as typical of this entity, such as dilation of the infundibulum and hyperkeratosis and spongiosis of the infundibular epithelium, many other histological changes can be observed. We report the case of a 21-year-old woman with Fox–Fordyce disease and highlight the importance of perifollicular xanthomatosis as a key histological finding in the diagnosis of the disease.

Key words:

Fox–Fordyce disease

apocrine miliaria

perifollicular xanthomatosis

Resumen

La enfermedad de Fox-Fordyce es una rara dermatosis caracterizada por la presencia de múltiples pápulas foliculares pruriginosas en áreas corporales con riqueza de glándulas apocrinas como axilas, areolas mamarias o región genital. Los hallazgos histopatológicos que definen la enfermedad de Fox-Fordyce son muy variados. Además de los hallazgos descritos como típicos de esta entidad, como la dilatación del infundíbulo y la hiperqueratosis y espongiosis del epitelio infundibular, se pueden observar otros muchos hallazgos histológicos. Presentamos el caso de una mujer de 21 años de edad afectada por esta enfermedad y recalcamos la importancia de la xantomatosis perinfundibular como hallazgo histológico clave en el diagnóstico de esta entidad.

Palabras clave:

enfermedad de Fox-Fordyce

miliaria apocrina

xantomatosis perifolicular

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