Pathophysiology, clinical findings, and management of Fox-Fordyce disease: A systematic review.

BACKGROUND: Fox-Fordyce (FFD), also known as apocrine military, is an uncommon chronic inflammation of the apocrine sweat glands. It is characterized by pruritic, papular eruptions in apocrine-gland-bearing regions. FFD was described a century ago, but the exact pathogenesis of the disease and the management are not well understood. AIMS: This paper provides a wide understanding of the pathophysiology, clinical findings, and management of Fox-Fordyce disease. Its aim is to help the physician to diagnose and manage this entity accordingly. METHODS: A research was done using PubMed database on 12 April 12, 2020, and in order to retrieve all case reports, case series, cohort studies, randomized, and nonrandomized clinical trials were included describing FFD among patients. RESULTS: A total of 43 articles and 68 patients were included in the study. The majority of patients were young females. The disease was bilateral in 90%, affected the axillae and to a lesser extent the pubic and the periareolar areas and rarely the thoracic area, the abdominal area, and the face. FFD followed a relapsing and remitting course, and an evident improvement in disease course was noted after menopause. CONCLUSION: The typical FFD patient is a post-pubertal female and pre-menopause, presenting with pruritic papules in apocrine-gland-bearing regions. FFD can be sporadic or occurs in family, and it can be asymptomatic in 1/(3-4) of patients and can be triggered by laser hair removal and hormonal changes. Further randomized clinical trials assessing different treatment of FFD are now warranted.

Treatment of pruritus with botulinum toxin in a pediatric patient with Fox-Fordyce disease.

Fox-Fordyce disease is a chronic pruritic disorder of apocrine sweat glands that is often associated with significantly decreased quality of life. With no definitive cure, affected patients are often treated with topical corticosteroids as first-line therapy, but evidence for treatment of refractory cases is limited. We present an adolescent with Fox-Fordyce disease successfully treated for symptomatic relief with botulinum toxin type A injections. While previously reported in an adult patient, we detail the efficacy of this therapy in a pediatric patient.

Enfermedad de Fox-Fordyce en edad infantil / Fox-Fordyce Disease in a Pediatric Patient

No disponible

Fox Fordyce disease: a side effect of laser therapy.

Fox-Fordyce disease (FFD) is an uncommon apocrine disorder. We reported a 26-year-old woman with bilateral axillary pruritic papular lesions following 2 sessions of treatment with Diode (800 nm) laser for axillary hair removal. No lesions were developed in the other treated areas. Topical 0.1% tacrolimus gel was used for her treatment.

Rapid remission with calcipotriol betamethasone in refractory Fox-Fordyce disease.

Fox-Fordyce disease (FFD) is a rare pruritic dermatosis whose etiology has not been fully explored. It is mostly seen in women and presents as pruritic follicular papules at the apocrine (gland-bearing) regions, including the axilla, groins, perineum, and areola mammae, as well as the umbilicus. Treatment for FFD is extremely challenging in that there is no curative treatment for it. We report the case of a 26-year-old woman who was refractory to many treatments but who responded to calcipotriol betamethasone with rapid remission of her symptoms.

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.

[Axillary and perimamillary Fox-Fordyce disease (apocrine miliaria) in a 19-year-old woman]. / Axilläre und perimamilläre Fox-Fordyce-Erkrankung (apokrine Miliaria) bei einer 19-jährigen Patientin.

Fox-Fordyce disease (FFD), also known as apocrine miliaria, is a rare and chronic skin disease characterized by itching and skin-colored, light brown or yellowish papules. FFD typically affects postpubertal young women between 13 and 35 years. The etiology is not completely known, but a hormonal component is in discussion. Furthermore, exacerbating factors like laser hair removal and hyperhidrosis have been described. Treatment of FFD is quite challenging, as the reported modalities mostly show limited success.

Foxc1 Ablated Mice Are Anhidrotic and Recapitulate Features of Human Miliaria Sweat Retention Disorder.

Sweat glands are critical for thermoregulation. The single tubular structure of sweat glands has a lower secretory portion and an upper reabsorptive duct leading to the secretory pore in the skin. Genes that determine sweat gland structure and function are largely unidentified. Here we report that a Fox family transcription factor, Foxc1, is obligate for appreciable sweat duct activity in mice. When Foxc1 was specifically ablated in skin, sweat glands appeared mature, but the mice were severely hypohidrotic. Morphologic analysis revealed that sweat ducts were blocked by hyperkeratotic or parakeratotic plugs. Consequently, lumens in ducts and secretory portions were dilated, and blisters and papules formed on the skin surface in the knockout mice. The phenotype was strikingly similar to the human sweat retention disorder miliaria. We further show that Foxc1 deficiency ectopically induces the expression of keratinocyte terminal differentiation markers in the duct luminal cells, which most likely contribute to keratotic plug formation. Among those differentiation markers, we show that Sprr2a transcription is directly repressed by overexpressed Foxc1 in keratinocytes. In summary, Foxc1 regulates sweat duct luminal cell differentiation, and mutant mice mimic miliaria and provide a possible animal model for its study.

Inflammatory and glandular skin disease in pregnancy.

A switch from cell-mediated to humoral immunity (helper T 1 [Th1] to helper T 2 [Th2] shift) during gestation plays a key role in placental immune tolerance. As a result, skin diseases that are Th2 mediated often worsen, whereas skin diseases that are Th1 mediated often improve during gestation. Also, due to fluctuations in glandular activity, skin diseases involving sebaceous and eccrine glands may flare, whereas those involving apocrine glands may improve during pregnancy. Despite these trends, inflammatory and glandular skin diseases do not always follow the predicted pattern, and courses are often diverse. We review the gestational course of inflammatory skin diseases, such as atopic dermatitis (atopic eruption of pregnancy), psoriasis, impetigo herpetiformis, urticaria, erythema annulare centrifugum, pityriasis rosea, sarcoidosis, Sweet syndrome, and erythema nodosum, as well as glandular skin diseases, including acne vulgaris, acne rosacea, perioral dermatitis, hidradenitis suppurativa, Fox-Fordyce disease, hyperhidrosis, and miliaria. For each of these diseases, we discuss the pathogenesis, clinical presentation, and management with special consideration for maternal and fetal safety.

Fox-Fordyce Disease: An under-diagnosed adverse event of laser hair removal?

BACKGROUND: Fox-Fordyce Disease (FFD) is a rare chronic inflammatory skin disease of the apocrine glands, mainly affecting post-pubertal women. It involves apocrine gland-bearing areas including the axilla, areola, anogenital area and umbilicus. FFD induced by laser hair removal is a newly reported entity of unknown pathogenesis. To the best of our knowledge, there are only four reported cases in the literature describing a total of four patients. OBJECTIVES: We are reporting the first case series of laser-induced FFD with a histopathological and clinical evaluation of the reported cases. METHODS: A review of the available literature to date about laser-induced FFD was performed. Clinical and histopathological features were reviewed. RESULTS: In our case series, the clinical and histological appearance of FFD following laser hair removal is similar to that of classic FFD. Exacerbating factors were present in two patients, and were similar to that of classic FFD. The main histopathological features included dilatation of the follicular infundibulum, follicular hyperkeratosis, dyskeratosis in the follicular infundibulum, periductal lymphohistiocytic infiltrate and perifollicular fibrosis. Two of the four reported cases as well as one of our patients had no perifollicular xanthomatous inflammation, a hallmark feature of classic FFD. Mast cells were absent in all reported cases, as well as in our patients. CONCLUSION: We believe that laser-induced FFD is under-diagnosed as a newly reported adverse event of laser hair removal. It is clinically and histopathologically similar to classic FFD.

Successful treatment of areolar Fox-Fordyce disease with surgical excision and 1550-nm fractionated erbium glass laser.

Fox-Fordyce disease (FFD) is a rare chronic disorder characterised by persistent inflammation because of the obstruction of apocrine sweat glands, which is a key factor of pathogenesis. The treatment of FFD is known to be difficult, and the modalities of treatment have not yet been widely studied. We report the successful treatment of a case of bilateral areolar FFD by a combination of surgical excision and 1550-nm fractionated erbium glass laser in an 18-year-old woman. The patient presented with a bilateral areolar eruption of multiple, severely pruritic, 3-4 mm skin- to grey-coloured folliculocentric dome-shaped papules. The initial treatment plan was for bilateral surgical excision of the larger and more highly elevated papules via circumferential dermal excision, which was intended to maintain the areolar contour and minimise distortion. A 1550-nm fractional erbium glass laser was then used to control the remnant lesions. The patient was recurrence-free at 14 months after the final laser treatment, and she was fully satisfied with the treatment results.

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

No disponible