Follicular mucinosis and follicular mycosis fungoides: clinicopathological evaluation of seven cases.

OBJECTIVE: Follicular mucinosis is a disease characterized by follicular degeneration and mucin accumulation. It can be seen in mycosis fungoides, although idiopathic or forms associated with other diseases are also known. Follicular mycosis fungoides is a type of mycosis fungoides with different clinicopathological and prognostic features. MATERIAL AND METHOD: Seven cases with follicular centered lesions and multiple biopsies (2-6) were included. Cases were evaluated according to their clinical, histological and immunophenotypical features and follow-up data. RESULTS: All cases were male, and the mean age was 40.3 (range 18-61). Clinical complaints were follicular prominence, erythema and alopecia at head and neck, trunk, and lower limbs. Follicular mucinosis (6/7), and dermal lymphoid infiltration showing minimal-intensive folliculotropism accompanied by eosinophils was seen. Lymphoid infiltration was composed of small-medium sized cells, with scattered hyperchromatic nuclei in six cases. In one case there was only minimal cytological atypia. Intense folliculotropism of atypical lymphocytes and dense dermal infiltration without follicular mucinosis was seen in one case. Local and/or systemic treatments were applied and partial remission was achieved histologically. In three cases new and increasing lesions were seen. Density of infiltration and atypia were increased. CONCLUSION: The findings supported the opinion that follicular mucinosis is an important finding seen in mycosis fungoides. There can be important differences concerning the amount of infiltration and degree of atypia. In cases where the density of infiltration associated with follicular mucinosis is not diagnostic for MF, there can be progression over time. Long-term follow up is necessary in such cases where the differential diagnosis is difficult.

Alopecia mucinosa / Alopecia mucinosa

La mucinosis epitelial (mucinosis folicular) es un patrón histológico reactivo caracterizado por la acumulación de mucina en el epitelio infundibular, folicular o sebáceo, que puede ocurrir en múltiples procesos cutáneos, y que no puede ser considerada una enfermedad específica. La alopecia mucinosa es una entidad clinicopatológica con lesiones cutáneas distintivas, depósitos de mucina en los folículos pilosos e infiltrados linfoides perianexiales. Se han descrito tres tipos de alopecia mucinosa: idiopática, persistente o crónica benigna y asociada a linfoma. Para algunos autores, estos tipos de alopecia mucinosa no son enfermedades independientes, sino que representan variantes de micosis fungoides con mucinosis epitelial

Epithelial mucinosis (follicular mucinosis) is a reactive histological pattern characterized by the accumulation of mucin in the infundibular, follicular, and sebaceous epithelium, that may occur in multiple skin conditions and cannot be considered a specific disease. Alopecia mucinosa is a clinicopathological entity with distinctive skin lesions, mucin deposits in the hair follicles and periadnexal lymphoid infiltrates. Three types of mucinous alopecia have been described: idiopathic, persistent or benign chronic, and associated to lymphoma. For some authors, these types of mucinous alopecia are not independent diseases, but rather represent variants of mycosis fungoides with epithelial mucinosis

Mucinosis folicular secundaria a fotoalergia por captopril / Follicular mucinosis secondary to captopril-induced photoallergy

El captopril es uno de los inhibidores de la enzima convertidora de la angiotensina que ha sido ampliamente utilizado como antihipertensivo y vasodilatador. Aunque se producen efectos adversos cutáneos en el 10 % de las prescripciones, no existen muchos datos publicados sobre fotosensibilidad. Por otro lado, la mucinosis folicular se ha descrito asociada a numerosos procesos, principalmente linfomas, lupus eritematoso y con menos frecuencia a erupciones fotoinducidas y reacciones a fármacos. Se presenta el caso de una paciente tratada con captopril que desarrolló una erupción en áreas fotoexpuestas. Histológicamente mostró cambios compatibles con mucinosis folicular, con un fotoparche positivo al captopril y con resolución de las lesiones tras retirar el agente hipotensor

Captopril is an angiotensin-converting enzyme inhibitor which has been widely used as an antihypertensive agent and vasodilator. Although adverse skin effects occur in 10% of patients prescribed this drug, there is little published data about photosensitivity. On the other hand, follicular mucinosis has been described in association with numerous processes, mainly lymphomas, lupus erythematosus and, less frequently, with photoinduced eruptions and drug reactions. We present the case of a female patient treated with captopril who developed an eruption in photoexposed areas. Histologically, the patient showed changes consistent with follicular mucinosis, with a positive captopril photopatch test and with resolution of the lesions after the antihypertensive agent was withdrawn

Mycosis fungoides with follicular mucinosis displaying aggressive tumor-stage transformation : successful treatment using radiation therapy plus oral bexarotene combination therapy.

Follicular mucinosis is a tissue reaction pattern characterized by mucin deposition with follicular sebaceous units and is found as an idiopathic, primary, benign process (alopecia mucinosa), or as a secondary process due to inflammatory and neoplastic disorders (mycosis fungoides). When associated with follicular mucinosis, mycosis fungoides commonly pursues an aggressive course, often undergoing large-cell transformation, which is associated with resistance to therapy and poor prognosis. We present a case of mycosis fungoides with follicular mucinosis that was treated with incomplete courses of interferon, isotretinoin, and polychemotherapy with subsequent rapid progression to tumor-stage mycosis fungoides with large cell transformation and nodal and bone marrow involvement. In this setting, the patient was treated with local radiation therapy, total-skin electron beam therapy, and therapy and maintenance with the oral retinoid-X-receptor retinoid bexarotene, and achieved a durable complete remission.

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.

[Cutaneous mucinoses]. / Les mucinoses cutanées.

Cutaneous mucinoses are a heterogeneous group of disorders in which mucin accumulates in the skin or in the follicles. Mucin is a gelatinous substance composed of glycosaminoglycanes, especially hyaluronic acid and dermatan sulfate bound to small quantities of chondoitin sulfate and heparin sulfate. Though the causes of mucinoses remain unknown, they can be divided into distinctive cutaneous (primary) mucinoses, in which mucin deposition is the distinctive histological sign resulting in clinically distinctive lesions, and cutaneous disorders, in which mucin deposition is an epiphenomenon (secondary mucinoses). Histologically, mucin is recognized after special staining techniques using alcian blue and colloidal iron. The microscopic localization of the mucin deposit is used to distinguish between dermal and follicular forms of primary mucinoses and between epidermal, dermal and follicular forms of secondary mucinoses. We present here the clinical and histological features of primary cutaneous mucinoses and an updated classification. The main therapeutic schemas are outlined.

Mucinosis pápulo-nodular asociada a Lupus Eritematoso Sistémico con poliserositis, esplenomegalia y adenomegalias

Describimos el caso de un paciente de 24 años, con un cuadro clínico de 33 meses de evolución de un lupus eritomatoso sistémico asociado a lesiones pápulo-nodulares en miembros superiores e inferiores en el tronco. La biopsia de piel de las lesiones papulo-nodulares demostró acúmulos de mucina y abundantes mastocitos en la dermis intercalados con tejido colágeno. Revisamos la literatura y sólo se han informado 18 casos, ninguno en Latinoamérica. A diferencia de los casos descritos en la literatura médica, no hay informe de poliserositis, adenomegalias y esplenomegalia asociados a la mucinosis pápulo-nodular como se describe en este paciente. Las lesiones cutáneas se manifestaron con la actividad lúpica y la respuesta a la prednisona fue adecuada.

Successful treatment of primary progressive follicular mucinosis with interferons.

Follicular mucinosis is a primary idiopathic disease or a secondary, lymphoma-associated dermatosis. An effective standard therapy for the benign group is unknown. We describe a patient with primary benign disseminated progressive follicular mucinosis who was successfully treated with recombinant interferon alfa-2b and interferon-gamma. Interferons might act by down-regulation of activated inflammatory cells and/or by induction of enhanced elimination of extracellular mucin via increasing phagocytosis by macrophages.