Cases reported "Mucinosis, Follicular"

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1/48. mycosis fungoides with mucinosis follicularis in childhood.

    mycosis fungoides is a cutaneous T-cell lymphoma (CTCL) usually observed in mid to late adulthood. It occurs only rarely during childhood. Follicular mucinosis is a chronic dermatosis involving the sebaceous glands and outer root sheaths. It is normally differentiated into a juvenile benign form and an adult form possibly associated with mycosis fungoides. We report a 12-year-old boy who presented with an 8-month history of erythematous mucinous plaques on the scalp. Three months later, he developed erythematous patches and plaques on his whole body, accompanied by cervical lymphadenopathy. A biopsy showed follicular mucinosis and epidermotropism of the lymphocytic infiltrate. immunophenotyping and a PCR clonality test were consistent with CTCL. The patient received PUVA treatment and local steroids, resulting in partial remission. mycosis fungoides should be considered in the differential diagnosis of chronic, scaling dermatoses in childhood. Moreover, follicular mucinosis in childhood can be associated with mycosis fungoides. ( info)

2/48. Syringolymphoid hyperplasia and follicular mucinosis in a patient with cutaneous T-cell lymphoma.

    Syringolymphoid hyperplasia with alopecia is an uncommon chronic dermatosis of which 9 cases have been reported, with or without follicular mucinosis or cutaneous T-cell lymphoma. We report a patient with cutaneous T-cell lymphoma and syringolymphoid hyperplasia and follicular mucinosis and review the previously reported cases. All reported cases with syringolymphoid hyperplasia were men (10 of 10), with the clinical findings of alopecia (9 of 10) and anhidrosis (3 of 10). Only 3 of 10 cases had associated follicular mucinosis. Of the 7 cases investigated, 6 were found to hve cutaneous T-cell lymphoma. Three patients were not investigated for cutaneous T-cell lymphoma. Although syringolymphoid hyperplasia can be idiopathic, it can also reflect a syringotropic cutaneous T-cell lymphoma. Careful follow-up with a biopsy of persistent lesions is recommended to evaluate for the presence of lymphoma. ( info)

3/48. Folliculotropic T-cell lymphocytosis (mucin-poor follicular mucinosis).

    A 48-year-old man presented with multiple asymptomatic patches of hair loss over his trunk and limbs associated with focal keratotic follicular plugs. Multiple skin biopsies showed a panfollicular lymphocytic infiltrate associated with follicular hyperkeratinization, minimal follicular spongiosis, focal basaloid follicular hyperplasia but no overt follicular mucinosis. The lymphocytes were small and there was no atypia. Immunoperoxidase stains showed that the follicular lymphocytes were T cells and predominantly CD4 positive with HLADr (LN3) expressed on their surface. There were insufficient clinical or histopathological features to make a diagnosis of folliculotropic T-cell lymphoma. This case currently may be classified best as folliculotropic T-cell lymphocytosis and may represent a mucin-poor counterpart of follicular mucinosis. Such cases may pursue an indolent course or may evolve to folliculotropic T-cell lymphoma, mycosis fungoides or anaplastic lymphoma. The term folliculotropic T-cell lymphocytosis may be useful for similar cases lacking clinical or histological criteria for lymphoma and lacking follicular mucinosis. ( info)

4/48. Rapidly progressing mycosis fungoides presenting as follicular mucinosis.

    Follicular mucinosis can occur as a primary idiopathic disorder or can arise in association with benign or malignant disease, most notably mycosis fungoides. We describe a patient with an aggressive folliculotropic variant of mycosis fungoides that initially presented as follicular mucinosis with alopecia. One month after the diagnosis of follicular mucinosis, a diagnosis of mycosis fungoides was made, and 3 months later inguinal lymph node involvement with mycosis fungoides developed. A skin biopsy specimen demonstrated prominent follicular mucinosis with folliculotropism of atypical cells and intrafollicular Pautrier's microabscesses. As demonstrated in this case, follicular mucinosis can be a presenting sign of rapidly progressive mycosis fungoides. In our review of follicular mucinosis and its association with mycosis fungoides, we found that the folliculotropic variant of mycosis fungoides appears more commonly to have an aggressive course than classic mycosis fungoides. ( info)

5/48. alopecia mucinosa and lymphoma. Report of two cases and review of literature.

    A total of 90 cases of alopecia mucinosa (follicular mucinosis) have been reported in the world literature since Pinkus described the entity in 1957. alopecia mucinosa appears morphologically in any variety of three basic clinical types: grouped folliculopapules, plaque forms of folliculopapules, and as boggy nodular masses. Characteristic histochemical changes of follicular mucinosis have been demonstrated in alopecia mucinosa as well as in selected cases of mycosis fungoides and lymphoma. The majority of case reports of alopecia mucinosa have been in children and young adults (58%), and the course of the dermatosis was usually uneventful. Beyond age 40, the disease appears to behave differently in that it follows a prolonged chronic course, and of 28 cases in this category four (15%) developed into either mycosis fungoides or lymphoblastoma. ( info)

6/48. 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. ( info)

7/48. A case of follicular mycosis fungoides with follicular mucinosis: a rare association.

    Follicular mycosis fungoides (FMF) is a rare cutaneous T cell lymphoma characterized by an atypical lymphoid infiltrate spreading within and around hair follicles without epidermotropism or follicular mucin deposits. Its occasional presentation with minimal epidermal involvement and/or follicular mucinosis suggests the need for uniform histologic criteria. We describe a new case of FMF associated with follicular mucinosis and discuss its morphologic spectrum of presentation. ( info)

8/48. Follicular mucinosis in suspected leprosy.

    Four cases of suspected leprosy showed, on biopsy, follicular mucinosis without any granulomatous inflammation. All the patients were adolescents (12-17 years) with a single lesion on the face. Three patients showed complete clearing after anti-leprosy treatment, and the fourth patient is currently taking anti-leprosy treatment and showed good clinical response. ( info)

9/48. Purely follicular mycosis fungoides without mucinosis: report of two cases with review of the literature.

    Follicular lesions can either associate with typical patch-/plaque-type mycosis fungoides or, more rarely, be the only clinical manifestation of the disease. We describe 2 adult patients who presented with alopecia and disseminated follicular erythematous papules, and comedones and cysts, respectively. In both patients, histology showed a folliculotropic infiltrate of atypical lymphocytes that spared the epidermis, in the absence of follicular mucinosis. Molecular genetic analysis confirmed the oligo/monoclonal nature of the T-cell infiltrate. Reported cases of purely follicular mycosis fungoides without mucinosis are reviewed. ( info)

10/48. Acneiform follicular mucinosis of the head and neck region.

    Follicular mucinosis has a protean clinical presentation and can be differentiated in a benign-idiopathic type and an evolutive lymphoma-associated type. Young age and single localization, especially in the head and neck region, are suggestive of the benign type. Reviewing the literature of follicular mucinosis appearing as an acneiform eruption of the face and neck, we were unable to find any case evolving in lymphoma, suggesting that this form is a particular clinical sub-type of follicular mucinosis with a favorable prognosis. We report two cases of this variant of follicular mucinosis and discuss the clinical characteristics and differential diagnosis. ( info)
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