Fox-Fordyce disease (FFD), also known as apocrine miliaria or chronic . López- Ávila A, Ortiz S. Enfermedad de Fox-Fordyce con presentación clínica atípica. Request PDF on ResearchGate | Enfermedad de Fox-Fordyce | —Fox-Fordyce disease is an infrequent inflammatory disorder characterized by the appearance . Request PDF on ResearchGate | On Nov 1, , I. Ballester and others published Enfermedad de Fox-Fordyce con presentación clínica atípica.
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Fox-Fordyce disease is a relatively infrequent pathology of the apocrine glands that affects almost exclusively young women. 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 FFDalso known as apocrine miliaria or chronic pruritic papular eruption of the pubis and armpits, affects areas where apocrine glands are found predominantly, such as the pubic, axillary, and anogenital regions.
We present two cases with both clinical and histopathological diagnosis of perifollicular xanthomatosis. A year-old female patient, resident of Mexico City, previously healthy, reported a one-month history of multiple mildly pruritic papules in both armpits.
Patient reported hyperhidrosis and no prior treatment. Physical examination showed localized, bilateral, asymmetric alterations affecting both axillae, more prominently on the left, characterized by yellowish-brown punctate papules 1 mm in diameter with a smooth uniform surface and follicular distribution Figure 1.
Yellowish-brown punctate papules with follicular distribution coalescing in plaques and affecting both armpits. Intrafollicular and apocrine gland mucin deposits were observed with Alcian blue staining.
Immunohistochemistry showed perifollicular cells strongly positive for CD Carcinoembryonic antigen and epithelial membrane antigen were negative in the xanthomatous cells Figure 3. A – CD68 intensely positive in peripheral xanthomatous histiocytes. B – Negative membrane epithelial antigen C – Negative carcinoembryonic antigen in histiocytes. FFD was confirmed and topical clindamycin was initiated, with improvement of the symptoms.
Patient had received prior treatment with antihistamines and emollients with no improvement.
Patient reported that other females in her family had presented this skin condition in the armpits, although less severe. Patient had a year history of comedogenic acne on the face and trunk as well as menstrual alterations with amenorrhea of up to 2 months. Therefore, pelvic ultrasound was ordered, showing polycystic ovaries.
Physical examination showed a disseminated dermatological condition affecting the axillae and genitalia, in hairy areas, characterized by skin-colored punctate papules 1 to 2 mm in diameter with follicular distribution and some crusts Figure 4. Punctate papules with follicular distribution in the armpits and hemorrhagic crusts due to scratching.
Histopathology showed dilated follicular infundibula with hyperkeratosis, as well as xanthomatous histiocytes with perifollicular distribution and discrete inflammatory lymphocytic infiltrate.
Fox-Fordyce Disease: An Endocrinological Study | JAMA Dermatology | JAMA Network
Intrafollicular mucin deposits were also observed Figure 5. FFD was diagnosed, and combined oral contraceptives cyproterone with ethinylestradiol and topical hydrocortisone were prescribed, with partial improvement of lesions and pruritus. This disease entity presents characteristic clinical data, affecting areas of the body where apocrine glands are found, such as armpits, pubis, and the anogenital region.
However, less frequent locations on the thorax, areolas, abdomen, and legs have been described. Both patients were childbearing-age women.
Clinically, FFD is characterized by the presence of multiple skin-colored enfermedar papules, slightly yellowish or brown, dome-shaped, with a smooth surface, which may be accompanied by mild to moderate pruritus or even be asymptomatic.
Enfermedad de Fox-Fordyce | Actas Dermo-Sifiliográficas (English Edition)
Exacerbating symptoms include heat, moisture, physical activity, friction with clothing, and excessive sweating. Definitive diagnosis is made by histopathological examination in which nonspecific findings such as intrafollicular corneal plug, hyperkeratosis, spongiosis, retention vesicles, glandular dilation with mucin deposits, and perifollicular lymphohistiocytic inflammatory infiltrate can be observed.
Treatment response tends to be limited or partial; the lesions and symptoms may recur or persist. First-line treatments include topical and oral retinoids, benzoyl peroxide, topical calcineurin inhibitors, clindamycin, intralesional or topical steroids, and oral contraceptives, the latter reported with complete resolution of lesions.
In the second case, the engermedad was treated with combined oral contraceptives and topical hydrocortisone, achieving partial improvement of lesions and pruritus.
frdyce Alternative therapies as second-line treatment or in severe cases, such as botulinum toxin, phototherapy, electrocoagulation, copper vapor and CO 2 laser, liposuction, curettage, and microwave have been described with favorable results. We have reported two cases here, both with perifollicular xanthomatosis on histology, supporting the position that this is the most specific and distinctive dr of this relatively rare disease.
Approval of the final version of the manuscript; Conception and planning if the study; Elaboration and writing of the manuscript; Obtaining, analyzing and interpreting the data; Effective participation in research enfermexad Critical review of the literature; Critical review of the manuscript. National Center for Biotechnology InformationU. Journal List An Bras Dermatol v. Enfermedac information Article notes Copyright and License information Disclaimer.
Received Aug 18; Accepted Nov 3. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivative License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the original work is properly cited and the work is not changed in any way.
Abstract Fox-Fordyce disease is a relatively infrequent pathology of the apocrine glands that affects almost exclusively young women. Apocrine glands, Fox-Fordyce disease, Miliaria. Open in a separate window. Patterns histopathologic of Fox-Fordyce disease.
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Apocrine sweat retention in man I. Experimental production of asymptomatic form. Fox-Fordyce disease of the vulva. Indian J Sex Transm Dis. Clinicopathological Study of Fox-Fordyce disease. Fox Fordyce exacerbated by hyperhidrosis.
A report of 2 cases responding to topical clindamycin. Indian J Dermatol Venereol Leprol. Mc Graw Hill; J Am Acad Dermatol. Perifollicular xanthomatosis as the hallmark of axillary Fox-Fordyce disease: An evaluation of histopathologic features of 7 cases.