Fox-Fordyce disease: report of two cases with perifollicular xanthomatosis on histological image.

Fox-Fordyce disease is a relatively infrequent pathology of the apocrine glands that affects almost exclusively young women. The disease is characterized by the presence of pruritic follicular papules mainly in the armpits that respond poorly to treatment and severely affect the patient's quality of life. We report two cases with clinical diagnosis and histopathological confirmation, presenting perifollicular xanthomatosis on histological examination, recently described as a distinctive, consistent, and specific feature of this disease.

Fox-Fordyce disease: report of two cases with perifollicular xanthomatosis on histological image

Abstract: Fox-Fordyce disease is a relatively infrequent pathology of the apocrine glands that affects almost exclusively young women. The disease is characterized by the presence of pruritic follicular papules mainly in the armpits that respond poorly to treatment and severely affect the patient's quality of life. We report two cases with clinical diagnosis and histopathological confirmation, presenting perifollicular xanthomatosis on histological examination, recently described as a distinctive, consistent, and specific feature of this disease.

Glándulas de Fordyce en cuerpo del pene: un reto diagnóstico / Fordyce glands in the shaft of the penis: a diagnostic challenge

No disponible

Fox-Fordyce disease exacerbated by hyperhidrosis.

Fox-Fordyce disease is an uncommon disorder primarily affecting postpubertal females. It is characterized by intensely pruritic, papular eruptions in apocrine-gland bearing regions. Rarity and scant literature have resulted in a lack of definitive treatment options or pathognomonic diagnostic indicators. We report a 16-year-old boy with typical findings of Fox-Fordyce disease including numerous, grouped, rounded (1-3 mm), skin-colored papules confined to the axillary and periareolar areas, bilaterally. These lesions started before puberty, and were exacerbated by hyperhidrosis.

[Perifollicular xanthomatosis as a key histological finding in Fox-Fordyce disease]. / Xantomatosis perifolicular: hallazgo histológico clave en la enfermedad de Fox-Fordyce.

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.

Xantomatosis perifolicular: hallazgo histológico clave en la enfermedad de Fox-Fordyce / Perifollicular xanthomatosis as a key histological finding in Fox-Fordyce disease

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 (AU)

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 (AU)

Angiokeratomas in Fabry's disease and Fordyce's disease: successful treatment with copper vapour laser.

Two patients with multiple angiokeratomas on genitalia and thighs, one with Fabry's disease and one with Fordyce's disease, were treated with copper vapour laser light of 578 nm wavelength. The result was desirable, with destruction and disappearance of the lesions and minimal scarring and posttreatment hyper- or hypopigmentation.

Ectopic epibulbar Fordyce nodules in a buccal mucous membrane graft.

A 70-year-old man underwent oral mucous membrane grafts to the right eye for presumed trachoma at the ages 26 and 32 years. A superior limbic mass within the site of the graft was found in 1983; the mass was composed of mature sebaceous glands compatible with the lesions called Fordyce nodules when located in the buccal mucosa. Recession of the levator aponeurosis and partial excision of the mass restored the upper eyelid's normal position, and visual acuity in this eye improved from 20/300 to 20/40.

Vulvar pruritus--a symptom, not a disease.

Because the underlying causes are so varied, vulvar pruritus must be understood as a symptom and not as a disease entity in itself. Successful treatment depends on proper identification of the underlying disorder.