Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0016632 (Fox)
 1,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 40-year-old woman presented with a 2-year history of intermittently pruritic pale yellow follicular papules localized to both axillae associated with decreased axillary hair growth and sweating. Skin biopsies revealed an expanded perifollicular adventitial sheath packed with xanthoma cells. There was scant lymphocytic inflammation around the follicles. Vacuolated keratinocytes were present within the infundibular region of the follicles. Serum lipid levels and serum protein electrophoresis were both normal. The features in our case overlap those described recently as a xanthomatous variant of Fox-Fordyce disease. However, in our patient the pruritus was not intense, the lesions were confined to the axillae, and the histopathological features of Fox-Fordyce disease were not confirmed. We prefer to classify our case as an axillary perifollicular xanthomatosis. It is possible that axillary perifollicular xanthomatosis is the follicular counterpart of the epidermal-based verruciform xanthomas, as both are normolipaemic and are limited to the adventitial tissue close to keratinocytes that may be the source of lipid. The finding of vacuolated keratinocytes in the infundibular region in our case may support this mechanism.
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PMID:Axillary perifollicular xanthomatosis resembling Fox-Fordyce disease. 1552 40

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.
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PMID:[Perifollicular xanthomatosis as a key histological finding in Fox-Fordyce disease]. 1834 37

Fox-Fordyce disease is a condition with protean histopathological alterations whose pathogenesis remains a mystery. Although recent studies have addressed histological changes specific of this disease, including perifollicular xanthomatosis, no attention has been given to apocrine acini dilation as an adjunct histopathological finding to the diagnosis. Moreover, although previous efforts were done to demonstrate that perifollicular foamy histiocytes harbor apocrine secretion content, this concept has not been proved to date. In this study, we report 2 cases harboring prominent dilation of apocrine coils with mucinous content. Such mucinous content showed mucin profile identical to the dermal mucin deposits in both cases. Of note, perifollicular foamy histiocytes demonstrated cytoplasmic mucin, supporting the suggestion that these cells phagocytose apocrine secretion. Although not specific, apocrine coil dilation is another histopathological feature of Fox-Fordyce disease and it may be used as a low-power magnification clue for the correct diagnosis. We also propose that the so-called perifollicular xanthomatosis may be composed of muciphages or mixed cell (muciphages/xanthomatous) population, an issue that should be further investigated in future studies.
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PMID:Dilation of apocrine glands. A forgotten but helpful histopathological clue to the diagnosis of axillary Fox-Fordyce disease. 1946 Dec 48

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.
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PMID:Fox-Fordyce disease: report of two cases with perifollicular xanthomatosis on histological image. 3006 65

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.
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PMID:Fox-Fordyce disease: report of two cases with perifollicular xanthomatosis on histological image. 3328 49