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.
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Paola C. Luis Eduardo A. Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the pilosebaceous follicle and sebaceous glands. It presents in two forms, a primary or idiopathic form and a secondary form associated with various benign or malignant processes. Among the malignant processes, the main association is with mycosis fungoides.
The frequent overlap of clinical, histopathological, immunohistochemical and molecular biology characteristics makes the correct classification of these conditions difficult, therefore a long follow-up of all cases is recommended. We report the case of an adolescent with disseminated lesions and discuss the difficulty of early identification of secondary follicular mucinosis associated with cutaneous lymphoma.
Follicular mucinosis FM is a cutaneous mucinosis in which there is a build-up of mucin at the external root sheath and with much less frequency in the sebaceous gland. Two variants are found, one idiopathic or primary form, usually present in children and young adults, of spontaneous remission, and a secondary form that affects a higher age range and is associated with other benign and malignant processes, the mycosis fungoides MF being the main association.
Clinical and histopathological criteria are fundamental for the distinction between primary and secondary forms; however, they are not specific enough for such definition, thus making the followup of patients necessary. Male patient, white, 13 years old, complained about spots on his body associated with pruritus for six months.
At the physical examination he presented erythematous and hypopigmented macules, well defined, alopecic, diameter of 5 to 10 cm, topped by follicular papules with thorny spicules which detached easily, on the cervical region, back, abdomen and flexural creases axillae, antecubital and popliteal fossae Figures 1 - 3.
Organomegaly was not found and the patient was previously healthy. Histopathology showed mucin inside the follicular epithelium, detected by HE coloration and Coloidal Iron, with disconnection of keratinocytes, perivascular and periannexal inflammatory infiltrate and without atypical lymphocytes. Figures 4 e 5. Follicular wall area, clear, with mucin deposition. Three follicles with mucin deposition on wall. The exams for investigation of associated systemic diseases blood count, renal function, liver function, thyroid function, anti-nuclear factor were normal.
FM is a rare condition, of unknown cause, which affects all races, ages and both sexes. FM is considered a reaction pattern to several conditions, being either idiopathic or secondary to benign diseases lupus erythematosus, insect bites, eczema, alopecia areata, hypertrophic lichen planus or malignant mycosis fungoides and Sezary syndrome, leukemia cutis, cutaneous B-cell lymphoma and Hodgkin's disease. The association of FM with the development of lymphoproliferative diseases is well documented and association with mycosis fungoides and its variants is more frequent.
The morphology of lesions on idiopathic and secondary forms is identical. Lesions present as single or multiple plaques, usually erythematous and alopecic, well delimited, discretely elevated, sometimes scaly and on top of them there may be follicular papules.
The follicular ostia may be dilated or filled with keratin and the pressure of lesion cause the exit of mucin. However, clinical manifestation may not be typical and there are reports of nodulary forms and presentations that simulate folliculitis, alopecia areata, scarring alopecia, chronic eczema, acne, urticaria and erythrodermic forms. Histopathology is essential for the diagnosis, showing mucin accumulation at the hair follicle external root sheath and sebaceous gland, besides inflammatory infiltrate of lymphocytes, macrophages and eosinophils with follicle tropism of lymphocytes.
The onset of MF might precede, occur concomitantly or years after the diagnosis of FM. In almost all cases in which there is development of MF this process happens within 5 years, one year on average. Patients are classified into two phenotypes with different evolution. It is accepted that when FM occurs in adults older than 40 years with infiltrative, persistent and diffuse lesions there is more serious risk of progression to MF; nevertheless, cumulative experience shows that the age of the patient, distribution of lesions, duration and extension of the disease do not distinguish primary benign FM from FM secondary to mycosis fungoides in a reliable way.
In contrast, the presence of a single lesion is a strong indication of primary FM; the development of lymphomas in single lesions, even though possible, is not expected.
Something similar occurs in histological evaluation of these cases. There is no standard conduct for the treatment of FM and it is possible to adopt an expectant management watchful waiting in cases of primary FM, since many of them resolve spontaneously between 2 and 24 months.
Several therapeutic modalities have been reported with variable results: topical corticoids, intralesional or oral, topical retinoids, oral isotretinoin, dapsone, indomethacin, minocycline, PUVA, cyclophosphamide and methotrexate, but no drug has a consistent result and the evaluation is difficult since there may be spontaneous involution. The patient with idiopathic FM must be oriented regarding the necessity of a follow-up for the early detection of alterations signs secondary to malignancies.
Such orientation is mandatory, and also suggested is patient follow-up for a minimal period of 5 years. J Am Acad Dermatol. Follicular mucinosis: a clinico pathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides - associated follicular mucinosis. J Cutan Pathol. Follicular mucinosis: literature review and case report. Primary follicular mucinosis: Long-term follow up of patients younger than 40 years with and without clonal T-cell receptor gene rearrangement.
Follicular Mucinosis. Arch Dermatol. Follicular mucinosis: clinical and histopathologic study. Parker SR, Murad E. Follicular mucinosis: clinical, histologic, and molecular remission with minocycline.
Successful treatment of recalcitrant primary follicular mucinosis with indomethacin and low-dose intralesional interferon alpha. Ann Dermatol. Treatment of so-called idiopathic follicular mucinosis with hydroxychloroquine. Br J Dermatol. Follicular mucinosis - Case report.
An Bras Dermatol. E-mail: manuzuchi hotmail. 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.
Services on Demand Journal. ABSTRACT Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the pilosebaceous follicle and sebaceous glands. Received: July 17, ; Accepted: July 23, Rio Branco, 39 How to cite this article.
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.
2018, Número 4
Paola C. Luis Eduardo A. Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the pilosebaceous follicle and sebaceous glands. It presents in two forms, a primary or idiopathic form and a secondary form associated with various benign or malignant processes. Among the malignant processes, the main association is with mycosis fungoides. The frequent overlap of clinical, histopathological, immunohistochemical and molecular biology characteristics makes the correct classification of these conditions difficult, therefore a long follow-up of all cases is recommended. We report the case of an adolescent with disseminated lesions and discuss the difficulty of early identification of secondary follicular mucinosis associated with cutaneous lymphoma.
2018, Number 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.