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)

Two overweight male patients developed acanthosis nigrican of the axillae. Seven to nine months later, the patients developed hidradenitis suppurativa deep within the areas of acanthosis nigricans. On examination of biopsy specimens, the hyperkeratosis of acanthosis nigricans was found to extend into the follicles. In previous reports, Fox-Fordyce disease and pityriasis rubra pilaris have preceded the onset of hidradenitis suppurativa.
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PMID:Hidradenitis suppurativa following acanthosis nigricans. Report of two cases. 95 34

Dermatoses of the nipple and areola are rare. The commonest dermatosis is Paget's disease, which presents in the form of a well demarcated erythematous area, sometimes erosive, oozing or hyperkeratotic. Histological examination reveals an intraepidermal proliferation of large clear cells, either isolated or grouped in clumps, predominantly in the suprabasal layers. Immunohistochemistry shows that these cells express low molecular weight cytokeratins and the epithelial membrane antigen, fairly frequently carcinoembryonic antigen. In 96% of cases, Paget's disease is associated with underlying breast carcinoma, either in situ or invasive. Erosive adenomatosis presents in the form of an erosion of the nipple, which is sometimes increased in size. Histologically, it consists of a benign tumour which may ulcerate the epidermis, composed of tubes and papillae lined by a double layer of epithelial and myoepithelial cells. The syringomatous tumour is exceptional. In places, it forms rudimentary sweat ducts and is considered to have an intermediate malignancy; its resection must be complete. Other tumours may also be observed in this site: leiomyoma, leiomyosarcoma, benign cutaneous lymphocytoma, basal cell carcinoma, naevoid areolar hyperkeratosis. They are exceptional except areolar neurofibromas in case of neurofibromatosis. Infectious dermatoses (viral warts, molluscum contagiosum, scabies) are accompanied by lesions in other sites. They same applied to the majority of inflammatory dermatoses such as eczema or Fox-Fordyce disease. Supernumerary nipples are situated on a line extending from the anterior part of the axillary crease to the medial part of the inguinal crease.
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PMID:[Dermatoses of the nipple and the areola]. 779 32

This report presents two prepubertal girls with Fox-Fordyce disease. The pruritic papules extensively affected the areas where apocrine glands are distributed (axillae, periareolar and intermammary zones, pubes, infraumbilical midline), and also extended to the neck and face near the external angle of the eyes in one child. Analyses of several biopsy specimens showed that the main lesion was a spongiotic vesicle containing inflammatory cells and keratinocytes affecting the hair infundibula and acrosyringia, together, with hyperkeratosis of both adnexa. The cause of the disease remains elusive, but the microscopic findings may explain the good results obtained with keratolytic agents.
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PMID:Fox-Fordyce disease in two prepubertal girls: histopathologic demonstration of eccrine sweat gland involvement. 884 48

The pathogenesis of Fox-Fordyce disease has been reported to be hyperkeratosis and obstruction of the upper hair follicle, where the duct of the apocrine sweat gland opens. We report a case of Fox-Fordyce disease with full clinical manifestation. It appeared to be caused by the obstruction of intraepidermal apoeccrine sweat ducts by apoeccrine secretory cells detached and released from the secretory epithelium. A 24-year-old woman visited our clinic with intensely pruritic papules on axillae, mammary areolae, and pubic areas. Histopathologic examination revealed an obstruction of the sweat duct in the epidermis, which opened directly to the skin surface. The closing substance of the duct was an aggregate of epithelial cells, probably derived from the secretory portion. In the dermis, the secretory cells of apocrinelike sweat glands had been detaching from the secretory epithelia. These findings suggest that Fox-Fordyce disease can occur by the mechanism in which apoeccrine secretory cells obstruct sweat ducts.
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PMID:Apoeccrine sweat duct obstruction as a cause for Fox-Fordyce disease. 1263 30

Fox-Fordyce disease is an uncommon condition that is biopsied even less commonly. Since the publication of Shelley and Levy, in 1956, the disease is regarded widely to be "apocrine miliaria", an analogue of eccrine miliaria. Histopathologically, an "intraepidermal sweat retention vesicle" is considered the only feature diagnostic of the condition. In this article, the entire literature devoted to the subject and reviewed features clinically and histopathologically as they were presented in those publications. Furthermore, observations in sections of tissue cut from 8 biopsy specimens taken from 4 patients with Fox-Fordyce disease are presented including correlation clinicopathologic. Changes histopathologically in Fox-Fordyce disease vary greatly. Apart from findings described previously as being typical (e.g., dilation, hyperkeratosis, and spongiosis of infundibula), there were vacuolar alteration at the dermo-epithelial junction of infundibula, dyskeratotic cells scattered in infundibula, and parakeratosis in the manner of cornoid lamellation within an orthokeratotic plug filling a dilated infundibulum. Infundibula and apocrine ducts were surrounded by numerous foamy macrophages. Despite that variation of features histopathologically, the appearance clinically, was similar in all patients, namely small infundibulocentric papules, sometimes with a keratotic plug in the center of it, and sometimes with a yellow cast. In conclusion, current concept of Fox-Fordyce disease is too restricted. All the different patterns histopathologically observed by us are manifestations of one single pathologic process named for Fox and Fordyce.
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PMID:Patterns histopathologic of Fox-Fordyce disease. 1561 31

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 (FFD) is characterized by a pruritic eruption of skin-coloured or yellowish papules in areas rich in apocrine glands. The histology comprises dilatation of follicular infundibula with hyperkeratosis, acanthosis, and spongiosis of the infundibular epithelium with perifollicular infiltration of lymphocytes and foamy histiocytes. We treated a 12-year-old girl with FFD with topical pimecrolimus for 12 weeks, this resulted in a complete clearance of lesions. After the therapy, the patient was followed for an additional 19 months without signs of relapse. The effects of pimecrolimus in FFD might imply that an inflammatory process inducing secondary reactive hyperkeratosis could be involved in the pathogenesis of FFD.
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PMID:Clinical effects of topical pimecrolimus in a patient with Fox-Fordyce disease. 2257 82