ABSTRACT
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.
Subject(s)
Fox-Fordyce Disease , Hair Removal , Apocrine Glands , Axilla , Epidermis , Female , Fox-Fordyce Disease/diagnosis , Fox-Fordyce Disease/therapy , HumansABSTRACT
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.
Subject(s)
Fox-Fordyce Disease , Hair Removal , Adult , Axilla , Betamethasone/therapeutic use , Calcitriol/analogs & derivatives , Female , Fox-Fordyce Disease/diagnosis , Fox-Fordyce Disease/drug therapy , HumansABSTRACT
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.
Subject(s)
Fox-Fordyce Disease , Hyperhidrosis , Axilla , Female , Fox-Fordyce Disease/diagnosis , Fox-Fordyce Disease/therapy , Hair Removal , Humans , Hyperhidrosis/diagnosis , Hyperhidrosis/therapy , Skin , Young AdultSubject(s)
Fox-Fordyce Disease/diagnosis , Fox-Fordyce Disease/pathology , Axilla , Female , Fox-Fordyce Disease/therapy , Humans , Young AdultABSTRACT
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.
Subject(s)
Fox-Fordyce Disease/diagnosis , Fox-Fordyce Disease/surgery , Laser Therapy/instrumentation , Nipples/surgery , Adolescent , Erbium , Female , Glass , Humans , Treatment OutcomeABSTRACT
No disponible
Subject(s)
Humans , Male , Young Adult , Fox-Fordyce Disease/complications , Fox-Fordyce Disease/diagnosis , Penile Diseases/diagnosis , Cryotherapy , Laser Therapy/methods , Fox-Fordyce Disease/physiopathology , Fox-Fordyce Disease/therapy , Penis/microbiology , Penis/pathology , Medical History Taking/methods , Medical History Taking/standards , Retrospective StudiesABSTRACT
No disponible
Subject(s)
Humans , Female , Young Adult , Axilla/injuries , Fox-Fordyce Disease/diagnosis , Laser Therapy/adverse effects , Hair Removal/adverse effects , Tretinoin/therapeutic useSubject(s)
Angiofibroma/pathology , Facial Neoplasms/pathology , Protein Kinase Inhibitors/therapeutic use , Tuberous Sclerosis/diagnosis , Acneiform Eruptions/diagnosis , Acneiform Eruptions/etiology , Acrodermatitis/diagnosis , Acrodermatitis/etiology , Asteraceae/adverse effects , Dermatitis, Allergic Contact/immunology , Fabry Disease/diagnosis , Facial Dermatoses/pathology , Fox-Fordyce Disease/diagnosis , Fox-Fordyce Disease/drug therapy , HIV Infections/complications , Humans , Interferons/therapeutic use , Skin Diseases, Vesiculobullous/etiology , TOR Serine-Threonine Kinases/antagonists & inhibitors , Terminology as Topic , Tuberous Sclerosis/drug therapy , Tuberous Sclerosis/genetics , Tuberous Sclerosis/pathology , Urticaria/etiology , Waxes/adverse effects , Xeroderma Pigmentosum/genetics , Xeroderma Pigmentosum/therapy , Zinc/deficiencySubject(s)
Humans , Male , Female , Child , Adolescent , Young Adult , Adult , Fabry Disease/complications , Fabry Disease/diagnosis , Fox-Fordyce Disease/complications , Fox-Fordyce Disease/diagnosis , Fox-Fordyce Disease/therapy , Fabry Disease/physiopathology , Fabry Disease/therapy , Gaucher Disease/complicationsSubject(s)
Fox-Fordyce Disease/diagnosis , Immunoglobulin G/metabolism , Lactoglobulins/immunology , Xanthomatosis/diagnosis , Adult , Axilla , Carmine/chemistry , Coloring Agents/chemistry , Female , Foam Cells/metabolism , Foam Cells/pathology , Fox-Fordyce Disease/complications , Fox-Fordyce Disease/metabolism , Humans , Mucins/analysis , Mucins/metabolism , Staining and Labeling , Xanthomatosis/etiology , Xanthomatosis/metabolismSubject(s)
Diseases in Twins/diagnosis , Fox-Fordyce Disease/diagnosis , Twins, Monozygotic , Axilla , Biopsy, Needle , Chronic Disease , Diseases in Twins/drug therapy , Diseases in Twins/pathology , Female , Follow-Up Studies , Fox-Fordyce Disease/drug therapy , Fox-Fordyce Disease/pathology , Humans , Immunohistochemistry , Monitoring, Physiologic/methods , Rare Diseases , Young AdultABSTRACT
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.
Subject(s)
Axilla , Fox-Fordyce Disease/diagnosis , Hyperhidrosis/etiology , Adolescent , Aluminum Hydroxide/therapeutic use , Female , Fox-Fordyce Disease/complications , Fox-Fordyce Disease/drug therapy , Fox-Fordyce Disease/pathology , Humans , MaleABSTRACT
Fox-Fordyce disease (FFD) is a rare skin disease manifesting as multiple pruritic follicular papules involving the skin-bearing apocrine glands. Reports of FFD in Asian people are scant. In this retrospective study, we describe the clinicopathological findings of five cases of FFD affecting Taiwanese subjects. Clinically, all patients presented with numerous uniform, 2-3-mm, skin-colored to light brown, dome-shaped papules with smooth surface, which were distributed in the apocrine gland-containing areas. Pruritus varied from mild to severe. The histopathology is characterized by focal spongiosis in the upper infundibulum with perifollicular fibrosis and lymphohistiocytic infiltrate. FFD needs to be differentiated from lichen amyloidosis, Darier's disease, syringoma, lichen simplex chronicus and spongiotic dermatitis clinically or pathologically. The findings of focal spongiosis in upper infundibulum associated with a perifollicular lymphohistiocytic infiltrate can facilitate the diagnosis of FFD.
Subject(s)
Fox-Fordyce Disease/pathology , Adolescent , Axilla/pathology , Diagnosis, Differential , Female , Fox-Fordyce Disease/diagnosis , Histiocytes/pathology , Humans , Keratosis/pathology , Male , Young AdultSubject(s)
Fox-Fordyce Disease/genetics , Adolescent , Adult , Fathers , Female , Fox-Fordyce Disease/diagnosis , Fox-Fordyce Disease/therapy , Humans , Male , Nuclear Family , Pedigree , Risk FactorsABSTRACT
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)