MUCINOSIS FOLICULAR PDF

The face, neck, and scalp are the most frequently affected sites, although lesions may occur on any site of the body. Histologically, the disorder is characterized by mucin deposition in the follicular epithelium. The condition is frequently divided into primary and secondary forms, with the latter form frequently associated with mycosis fungoides. In this case report, we describe a child with follicular mucinosis of the back and trunk and discuss the clinical variants, histopathological pattern, and treatment options. Follicular mucinosis is a rare disorder. The primary form occurs in children and young adults.

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Primary follicular mucinosis is a rare dermatosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. Clinically, it is characterized by the presence of papules or well-circumscribed and infiltrated plaques. In this paper, we report the case of a female patient, seven years old, evolving for three months with an asymptomatic, erythematous and infiltrated plaque located in the chin region.

The research of thermal, pain and tactile sensitivity was inconclusive. Histological findings confirmed the diagnosis of follicular mucinosis. There was regression of the lesion with the use of medium potency topical corticosteroids for 20 days. The pathogenesis of follicular mucinosis remains unknown, being in some cases associated with lymphoproliferative disorders. In endemic areas of leprosy, isolated and infiltrated follicular mucinosis lesions should be further differentiated from leprosy.

Follicular mucinosis FM is a rare dermatosis whose etiology is not fully understood, characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. It was described for the first time by Pinkus et al.

Follicular keratosis or areas of alopecia may also be observed. Other less common presentations have been already described, with acneiform, eczematous, cystic or nodular lesions. Female patient, seven years old, evolving for three months with asymptomatic cutaneous lesion located in the chin region. At the dermatological examination, an erythematous-brownish plaque, oval-shaped, well-circumscribed and infiltrated, measuring approximately 2.

The evaluation of thermal, pain and tactile sensitivity was inconclusive. In light of the clinical picture, hypotheses of childhood tuberculoid leprosy and follicular mucinosis were raised. Bacilloscopy was negative. At the histological examination, reticular degeneration in the infundibulum of hair follicles was observed, forming large clear spaces, with lymphohistiocytic infiltrate around vessels and appendages and discrete collagen fibrosis.

Alcian blue staining confirmed accumulation of mucin in hair follicles. Atypical lymphocytes or epidermotropism were not observed Figure 2. Patient was treated with medium-potency topical corticoid during 20 days, evolving with complete remission of lesion Figure 3. She remains under outpatient clinic follow-up, twelve months after the initial treatment, with no relapses.

Erythematous-brownish oval-shaped plaque, well-circumscribed and infiltrated, measuring approximately 2. In hematoxilin-eosin staining, reticular degeneration in the infundibulum of hair follicles was observed, forming large clear spaces, with lymphohistiocytic infiltrate around vessels and appendages and discrete collagen fibrosis. Follicular mucinosis is a disorder histopathologically defined by the accumulation of mucin in hair follicles and sebaceous glands, which undergo epithelial reticular degeneration.

It is believed that follicular keratinocytes are responsible for mucin production, as a response to the stimulus of cytokines released by perifollicular T lymphocytes.

Its pathogenesis, however, remains unknown, even though the role of circulating immune complexes and cell immunity have already been considered. Primary follicular mucinosis of short evolution affects children and young adults, presenting few lesions, limited to the head and neck regions, which may remit spontaneously with no relapses, similarly to the case presented. Primary FM of prolonged course occurs in older patients, manifesting itself with multiple lesions of chronic course, prone to relapses.

In both cases there is usually no association with systemic diseases, although some of the latest studies have demonstrated the presence of histological characteristics of mycosis fungoides in patients with primary FM. Studies published in the literature show that, in endemic areas, the differential diagnosis between Hansen's disease and follicular mucinosis is not always easy and must be considered.

In the case presented, the histological examination was essential for the diagnosis, since the inflammatory infiltrate composed of macrophages, perifollicular and perivascular lymphocytes, with no atypias or epidermotropism, rejected the diagnosis of childhood tuberculoid leprosy, confirming the diagnosis of FM.

Besides Hansen's disease, primary FM must be differentiated from persistent reaction to insect bite, lymphocytoma and lymphocytic infiltrate, among others. Several therapeutic modalities have been described for primary FM, with the use of topical or intralesional corticosteroids, dapsone, antimalarial agents, oral retinoids, interferon alpha, minocycline and phototherapy. Financial Support: None. Follicular mucinosis: an important differential diagnosis of leprosy in an endemic area. An Bras Dermatol.

National Center for Biotechnology Information , U. Journal List An Bras Dermatol v. Find articles by Danielle Cristine Westphal. Find articles by Silmara Navarro Pennini. Find articles by Petra Pereira de Souza. Author information Article notes Copyright and License information Disclaimer. E-mail: moc. Received Feb 14; Accepted Mar This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

This article has been cited by other articles in PMC. Abstract Primary follicular mucinosis is a rare dermatosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands.

Keywords: Diagnosis, differential; Leprosy, tuberculoid; Mucinosis, follicular. Open in a separate window. Complete remission of lesion after treatment with topical corticoid.

Footnotes Financial Support: None. References 1. Pinkus H. Alopecia mucinosa: inflammatory plaques with alopecia characterized by root-sheath mucinosis. AMA Arch Derm. Mucinosis follicularis.

Actas Dermosifiliogr. Pediatr Dermatol. Follicular mucinosis: literature review and case report. Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoidesassociated follicular mucinosis. J Cutan Pathol. J Am Acad Dermatol.

Reactive Benign Follicular Mucinosis: a report of two cases. Joshi R, Gopalani V. Alopecia mucinosa responding to antileprosy treatment: are we missing something? Indian J Dermatol. Ann Dermatol. Support Center Support Center. External link. Please review our privacy policy.

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2018, Number 4

Primary follicular mucinosis is a rare dermatosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. Clinically, it is characterized by the presence of papules or well-circumscribed and infiltrated plaques. In this paper, we report the case of a female patient, seven years old, evolving for three months with an asymptomatic, erythematous and infiltrated plaque located in the chin region. The research of thermal, pain and tactile sensitivity was inconclusive.

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2018, NĂºmero 4

Follicular mucinosis- Case report. An Bras Dermatol ;89 2 Pediatric follicular mucinosis: Presentation, histopathology, molecular genetics, treatment, and outcomes over an year period at the Mayo Clinic. Pediatr Dermatol ;30 2 Int J Clin Exp Pathol ;6 12 Piel ;28 6 Mucinosis folicular.

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Follicular mucinosis

Key words:. Follicular mucinosis- Case report. An Bras Dermatol ;89 2 Pediatric follicular mucinosis: Presentation, histopathology, molecular genetics, treatment, and outcomes over an year period at the Mayo Clinic. Pediatr Dermatol ;30 2 Int J Clin Exp Pathol ;6 12

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