Primary Follicular Mucinosis Research Articles

Clinicopathological features, therapeutic options, and significance of CD103 expression in 15 patients with follicular mucinosis.

Follicular mucinosis (FM) is generally divided into a primary benign idiopathic form and a secondary form associated with mycosis fungoides. To analyze the clinical and pathological features of FM and explore the pathological significance of CD103 expression. In this case series, we retrospective analysis the clinical, pathological, treatment and follow-up treatment of 15 cases of FM. The expression of CD103 in all cases was detected by immunohistochemistry. A total of 15 patients were enrolled, 7 were primary follicular mucinosis (P-FM) and 8 were mycosis fungoides-associated follicular mucinosis (MF-FM). Lesions of both P-FM and MF-FM are difficult to distinguish, present with red or dark red plaques and follicular papules. Pathologically, MF-FM showed more significant infiltrates of folliculotropic lymphoid cells, and the amount and proportion of CD103+ cells were significantly higher than that in P-FM. Follow-up data were available for 13 patients. Three cases were resolved after surgical resection, two patients were marked improved after oral administration of hydroxychloroquine and three times ALA photodynamic therapy respectively. The rest patients showed only modest efficacy. FM should be differentiated based on pathological characteristics and treatment response, CD103 is helpful in differential diagnosis of FM.

Primary cutaneous T-cell lymphomas in childhood and adolescence.

Primary cutaneous lymphomas are extranodal non-Hodgkin lymphomas of T- or B- cell origin, that predominantly affect older patients but have been reported in all age groups and as early as in the first years of life. Diagnosis of cutaneous lymphomas is challenging and requires high clinical suspicion and close collaboration between dermatologists, pediatric oncologists and pathologists. Skin involvement of non-Hodgkin lymphomas in children or adolescents can either be primary cutaneous or secondary due to an underlying nodal lymphoma. The most common primary cutaneous lymphomas encountered in children are of T-cell origin, with mycosis fungoides being the most prevalent cutaneous T-cell lymphoma, followed by CD30+ lymphoproliferative disorders. While cutaneous lymphomas share clinicopathologic characteristics between juvenile and adult forms, there are important differences in terms of clinical presentation, diagnosis and treatment. The hypopigmented variant of mycosis fungoides seems to be overrepresented in the pediatric age group. Prognosis and treatment of mycosis fungoides are stage dependent. The majority of children present with early-stage disease and respond well to topical corticosteroids and phototherapy.

Primary follicular mucinosis – case report

Of unknown etiology, acute follicular mucinosis is clinically characterized as an inflammatory disease coursing with more or less infiltrated and scaly plaques, with or without hair loss. In the chronic form, it usually has more numerous and diffuse lesions with variable morphology, from plaques to ulcerated nodules. It is characterized by localized or diffuse deposits of mucin in the skin or hair follicles. Objective: To present an unusual case of primary follicular mucinosis, the importance of differentiation from the secondary form of follicular mucinosis, to discuss the clinical and histopathological aspects used in the diagnosis, its characteristics and classifications as well as the possible therapeutic choices.

Primary follicular mucinosis: Case report

Primary follicular mucinosis: Case report

Pediatric Primary Follicular Mucinosis: Further Evidence of its Relationship with Mycosis Fungoides

Follicular mucinosis (FM) is an uncommon reaction pattern in which the accumulation of mucin in the follicular epithelium is the main pathologic finding. FM may be idiopathic (primary follicular mucinosis [PFM]), in association with mycosis fungoides or cutaneous T-cell lymphoma, or in association with other neoplastic and inflammatory conditions. Herein we report a case of PFM with identical T-cell clone rearrangement at anatomically distinct sites, supporting the idea that some authors have proposed, that FM may represent a low-grade lymphoproliferative disease related to mycoses fungoides with favorable prognosis.

Treatment of Primary Follicular Mucinosis with Imiquimod 5% Cream

Follicular mucinosis can develop as a primary form or associated with other benign or malignant skin conditions. We present a case of primary follicular mucinosis in which complete clinical remission was achieved after treatment with topical imiquimod 5%. Primary follicular mucinosis (PFM), also called idiopathic alopecia mucinosa, is considered a distinct entity, the clinical course of which is usually benign, but its pathogenesis remains unclear. There is no standard treatment for PFM, and a wide variety of therapies have been described. There are no previous reports of treatment of PFM with imiquimod.

Secondary follicular mucinosis associated with systemic lupus erythematosus

A 61-year-old woman presented with a five-month history of an intermittent eruption of papules and nodules on her face and neck. Past medical history included systemic lupus erythematosus. Histopathologic examination was consistent with secondary follicular mucinosis in association with systemic lupus erythematosus. This rare entity has been described in one prior report as a precursor to the clinical onset of systemic lupus erythematosus. Follicular mucinosis occurs as either a primary idiopathic form or a secondary form associated with either benign inflammatory processes or malignant conditions, such as cutaneous T-cell lymphoma. Numerous treatments for primary follicular mucinosis have been described, which include isotretinoin and glucocorticoids, whereas treatment of the underlying disease is necessary in the secondary form. The association with lymphoma mandates long-term clinical monitoring of patients with particularly recalcitrant, widespread, or chronic follicular mucinosis.

Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides‐associated follicular mucinosis

To determine (i) whether primary (idiopathic) follicular mucinosis (PFM) and lymphoma-associated follicular mucinosis (LAFM) are distinct or related entities and whether there are reliable criteria that allow the two forms to be distinguished, (ii) the histochemical properties and consequently the type of mucin that accumulates in the follicle in PFM and LAFM, and (iii) whether there is any difference between the staining properties of mucin in patients with PFM and LAFM. Thirty-one patients were divided into two groups. Group 1 comprised 20 patients with no associated mycosis fungoides or Sézary syndrome (PFM) and group 2 was made up of the other 11 patients who had clinicopathological evidence of cutaneous T-cell lymphoma (LAFM). The biopsy specimens of the patients were studied with histopathological, histochemical and immunohistochemical methods. Molecular biology studies were also performed. Patients with PFM were more frequently younger (mean age 39 years), women (F:M=3:1), and presented with a solitary lesion involving the head/neck area more often than patients with LAFM who were older (mean age 54 years), men (M:F=2:1), and presented with multiple lesions on areas of the body other than the head/neck area. As for histopathological findings, large cystic spaces filled with mucin and a slight perivascular and periadnexal polyclonal infiltrate of mostly non-atypical lymphocytes without epidermotropism and with an equivalent CD4+/CD8+ cell rate were more suggestive of PFM. On the contrary, patients with LAFM were more probably to present with a dense band-like infiltrate with some atypical lymphocytes and sign of epidermotropism, a prominent CD4+ immunophenotype and a monoclonal rearrangement of the infiltrate. Mucin proved to be a dermal-type mucin, composed of both hyaluronic acid and sulfated glycosaminoglycans. No differences were found in the composition of the follicular mucin in the PFM compared with LAFM. Although no single, indisputable feature can reliably differentiate PFM from LAFM and a considerable overlapping among the two groups exists, the use of multiple clinical, histological and immunopathological criteria associated with gene rearrangement analysis can be useful in evaluation of those patients.

Follicular mucinosis: Clinical, histologic, and molecular remission with minocycline

Follicular mucinosis is an uncommon inflammatory disorder characterized histologically by mucin accumulation in the follicular epithelium. The condition is generally divided into primary and secondary forms, the latter being frequently associated with mycosis fungoides. Lesional skin T-cell clonality has been documented in some patients with follicular mucinosis, even those with no histologic evidence of cutaneous lymphoma. In this report, we describe a patient with clonal idiopathic primary follicular mucinosis who had complete clinical, histologic, and molecular remission with minocycline therapy.

Successful Treatment of Recalcitrant Primary Follicular Mucinosis with Indomethacin and Low-dose Intralesional Interferon Alpha

Follicular mucinosis (FM) is an epithelial reaction pattern that is characterized by the accumulation of mucinous material in the epithelial hair follicle sheath and the sebaceous glands. Although various pharmacological agents have been employed in an attempt to treat FM, effective therapeutic options have remained elusive. We experienced a recalcitrant form of primary FM that we successfully treated with indomethacin and low-dose intralesional interferon alpha (IFN alpha), respectively. To the best of our knowledge, the primary type of FM that responded to indomethacin and low-dose IFN alpha, respectively, in a single case has not been reported in the English medical literature.

Primary follicular mucinosis: excellent response to treatment with photodynamic therapy

Primary follicular mucinosis: excellent response to treatment with photodynamic therapy

Primary follicular mucinosis: Long-term follow-up of patients younger than 40 years with and without clonal T-cell receptor gene rearrangement

Since the original descriptions of follicular mucinosis, accumulating experience shows that patient age, distribution of lesions, and duration or extent of disease do not reliably distinguish benign primary follicular mucinosis from secondary follicular mucinosis, associated with cutaneous lymphoma. More recently, it has been suggested that individuals with follicular mucinosis demonstrating a clonal T-cell receptor gene rearrangement may be at higher risk for the development of lymphoma. Long-term follow-up of 7 patients younger than 40 years with primary follicular mucinosis are reported. In all cases, there was no clinical or histologic evidence of associated dermatoses or lymphoma at the time of diagnosis. Five of the patients have clonal T-cell gene rearrangement as determined by Southern blot analysis. Clinically, at the time of diagnosis, lesions of primary follicular mucinosis ranged from papules confined to the face to widespread cutaneous plaques. After a mean follow-up of 10 years (range, 5-23 years) from the onset of disease, the majority of patients continue to have cutaneous manifestations of follicular mucinosis despite various treatments. There is no evidence of progression to cutaneous T-cell lymphoma in any patient despite the presence of a clonal T-cell receptor gene rearrangement. Continued prolonged follow-up of patients with clonal primary follicular mucinosis is necessary to determine the significance of infiltrates harboring a T-cell receptor gene rearrangement. However, in our experience with this group of selected patients, primary follicular mucinosis has been a clonal disorder with limited or “benign” cutaneous manifestations. (J Am Acad Dermatol 2002;47:856-62.)

Mucinosis folicular: nuevas entidades clínico-patológicas. Estudio clínico, histológico, inmunohistoquímico e inmunogenotípico de 23 pacientes

A clinical, histopathologic, inmunohistochemical and genotypic analysis review of twenty-three patients with follicular mucinosis (FM) is reported.Patients with primary follicular mucinosis (PFM) and secondary follicular mucinosis (SFM) associated with a systemic or cutaneous T-cell lymphoma were included in the study. Biopsy specimens of FM between the period 1975–2000 were selected based solely on the histopathologic finding of folicular mucin deposition from the dermatopathology files. Inmunostaining with monoclonal antibodies against pan T, pan B, T helper and T8 cells as performed in all biopsies. Detection of clonal T-cell receptor gene rearrangements was performed using a polymerase chain reaction (PCR) amplification of the T-cell ©gene from paraffin-embedded tissue in selected cases.Six cases with SFM (30,4%) were evaluated (all men; mean age: 54,1 y). The most frequent associated lymphoma was mycosis fungoides. Sixteen patients with PFM were evaluated (8 men and 8 women: 8 to 72 years). Four different clinical presentations of PFM could be identified after clinical review: localized PFM, diffuse PFM, acneiforme PFM and urticaria-like PFM.No histopathologic features separated clearly PFM from SFM. Only urticaria-like follicular mucinosis showed specific findigns, consisting in intense inflammatory infiltrate with abundant eosinophils. Inmunohistochemical findings showed predominance of T helper cells in all cases. Three cases of clonal PFM were observed. No differences could be detected between clonal cases and non-clonal cases of PFM. With long follow-up no evolution to cutaneous lymphoma was observed in clonal cases of PFM.Detection of PFM with clonal T-cell populations reinforces and expands the recently proposed concept of clonal benign dermatosis. Continued, long-term surveillance of patients with clonal PFM seems advisable.

Mucinosis folicular: un caso de presentación clínica inusual

Few cases of primary follicular mucinosis, not related to mycosis fungoides, are described in healthy adults. A 65-year-old woman presented with an erythematous patch on her right face. Histological examination revealed mucin depositions in the hair follicles. No other hematological or cutaneous diseases were detected.The aim of this case is to reinforce the concept of primary follicular mucinosis and to expand the spectrum of its clinical manifestations.

Follicular mucinosis associated with early stage cutaneous T-cell lymphoma: successful treatment with interferon alpha-2b and acitretin

BACKGROUND: Follicular mucinosis (FM) is a rare dermatosis characterized by mucin deposits in the pilosebaceous units. It is divided into a primary-benign type and a secondary type associated mostly with lymphomas. No standard effective therapy is available for the primary FM while in the secondary form treatment is aimed against the underlying disease. METHODS: We report a case of secondary FM in which a cutaneous T-cell lymphoma was detected 6 years after the initial eruption. RESULTS: Complete remission was achieved with combination therapy of interferon alpha-2b at a dose of 6 million U subcutaneously three times a week, and acitretin 35 mg/day, for 6 months. CONCLUSION: Regular clinical and histopathological evaluation is suggested for all patients with FM. For cases associated with cutaneous T-cell lymphoma the combination of interferon alpha and acitretin seems to be a good therapeutical approach.