Follicular mucinosis successfully treated by photodynamic therapy: Two case reports.

Follicular mucinosis is an epithelial reaction pattern characterized by follicular mucin accumulation. Follicular mucinosis may occur in a primary form or a secondary form associated with skin lymphoma, especially mycosis fungoides. This report describes two patients with these two forms of follicular mucinosis, who both had an excellent response to photodynamic therapy. The condition changes of the secondary follicular mucinosis patient were followed up by repeated pathological biopsies. The expression of CD103, a specific marker of tissue-resident memory T cells, was found to decline when the lesions improved. These results indicate an association between efficacy and pathological changes during the treatment of secondary follicular mucinosis.

Combination of tacalcitol ointment and photodynamic therapy for the treatment of follicular mucinosis of the scalp.

Follicular mucinosis (FM) is a rare inflammatory disorder histologically characterized by mucin deposition in the follicular epithelium. There is no standard therapy for FM and several treatments have been described in the literature. We present the case of a 59 year-old female affected by a recalcitrant FM with diffuse scalp alopecia, in which complete clinical remission was achieved after a combination of topical tacalcitol and photodynamic therapy.

Acneiform follicular mucinosis: an indolent follicular mucinosis variant unrelated to mycosis fungoides?

Follicular mucinosis (FM) can present as an acneiform eruption, and is usually a benign variant of primary FM unrelated to cutaneous T-cell lymphoma (CTCL). We report two cases of women in their twenties who presented with an acneiform rash on the face, arms and back. In both cases, pathological evaluation of the facial papules revealed predominantly mucinous degeneration of the follicular epithelium, with insufficient lymphocytic infiltration or atypia to diagnose mycosis fungoides. These cases are similar to previous reports of acneiform FM. As none of the reported cases progressed to CTCL, we consider that overdiagnosis and overtreatment should be avoided in acneiform FM, but recommend long-term follow-up.

Childhood follicular mucinosis co-existed with alopecia universalis.

We report a case of a 6-year-old girl presented with diffuse scalp and body hair loss and developed multiple groups of follicular papules on the trunk. She was diagnosed as follicular mucinosis co-existed with alopecia universalis. Histopathological study supported the diagnosis and did not find malignancy cells.

Mucinosis folicular facial en la infancia / Facial follicular mucinosis in childhood

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Follicular mucinosis associated with nonlymphoid skin conditions.

BACKGROUND: Follicular mucinosis coexisting with lymphoproliferative disorders has been thoroughly debated. However, it has been rarely reported in association with inflammatory disorders. METHODS: Thirteen cases have been retrieved, and those with cutaneous lymphoma or alopecia mucinosa were excluded. RESULTS: Follicular mucinosis was found in the setting of squamous cell carcinoma, seborrheic keratosis, simple prurigo, acne vulgaris, dextrometorphan-induced phototoxicity, polymorphous light eruption (2 cases), insect bite (2 cases), tick bite, discoid lupus erythematosus, drug-related vasculitis, and demodecidosis. Unexpectedly, our observations revealed a preponderating accumulation of mucin related to photo-exposed areas, sun-associated dermatoses, and histopathologic solar elastosis. The amount of mucin filling the follicles apparently correlated with the intensity of perifollicular inflammatory infiltrate, which was present in all cases. The concurrence of dermal interstitial mucin was found in 7 cases (54%). CONCLUSIONS: The concurrence of interstitial dermal mucinosis or the potential role of both ultraviolet radiation and the perifollicular inflammatory infiltrates in its pathogenesis deserves further investigations. Precise recognition and understanding of this distinctive, reactive histological pattern may prevent our patients from unnecessary diagnostic and therapeutic strategies.

Follicular mucinosis presenting as an acneiform eruption: a follow-up study.

It has been proposed by many authors that follicular mucinosis is directly associated with mycosis fungoides (MF). Follicular mucinosis may be classified into 3 main clinical variants: a benign idiopathic form in children and young adults, which includes an acneiform presentation; an idiopathic form in older patients with a benign course; and a third variant that occurs in adults and is associated with MF. Our goal was to study the relationship between the acneiform variant of follicular mucinosis and MF. Eight patients previously diagnosed with the acneiform variant of follicular mucinosis were identified. Biopsy specimens were reviewed to evaluate the histopathologic attributes that characterize the disease and the infiltrate's immunohistochemistry. Also, patient follow-up was assessed to evaluate the clinical course of the disease. Median age of onset of disease was 29.5 years; 95% of lesions were located in the head and neck region. Biopsy specimens showed a moderate to dense perivascular, perifollicular, and interstitial infiltrate of lymphocytes with mucinous deposits within the follicular epithelium. On immunohistochemistry, the infiltrate showed prominent leukocyte common antigen (LCA) positivity and a CD3-positive and CD4-positive infiltrate with rare CD20-positive cells. None of the study patients showed evidence of MF after a mean follow-up of 3 years. The benign course of disease demonstrated in the study patients suggests that the acneiform variant of follicular mucinosis probably represents a subpopulation of the benign idiopathic form of the disease. However, given that histopathologically this variant cannot be distinguished from the lymphoma-associated variant of follicular mucinosis, longitudinal evaluation is still warranted in these patients.

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.

Adult T-cell leukemia/lymphoma with follicular mucinosis: an unusual histopathological finding and a commentary.

Follicular mucinosis is currently recognized as a histopathological finding characterized by the accumulation of mucin within follicular epithelium and is commonly associated with follicular mycosis fungoides (MF). We report the finding of follicular mucinosis in a cutaneous nodule of human T-lymphotropic virus type 1 (HTLV-1) associated adult T-cell leukemia/lymphoma (ATLL). The patient was a 69-year-old female of Caribbean descent with a history of ATLL who presented with erythematous nodules on the chest and abdomen. Histopathologic examination showed a pan-dermal infiltrate of medium-to-large sized atypical lymphocytes extending into follicular epithelium where they associated with large mucin deposits. Immunohistochemical stains showed that the atypical lymphocytes were positive for CD3, CD4 and CD25 and negative for CD30. Cutaneous lesions of ATLL, which often present histopathologically as an epidermotropic lymphoma with Pautrier-type collections, are often difficult to distinguish from MF. Until recently, lymphoma-associated follicular mucinosis seemed specific to MF and Sézary syndrome (SS), being reported only once in a lesion of ATLL. We report a second case of ATLL-associated follicular mucinosis to increase awareness of this possible association, and briefly review the literature of follicular mucinosis-associated hematologic malignancies, ultimately cautioning against the interpretation of all cutaneous lymphoma-related follicular mucinosis as MF/SS.

Adult T-cell leukemia-lymphoma associated with follicular mucinosis.

Follicular mucinosis (alopecia mucinosa) is often associated with malignancies including mycosis fungoides and Sézary syndrome, but not adult T-cell leukemia-lymphoma (ATLL). We report a 49-year-old male patient who had pruritic follicular papules and erythemas clinically, and follicular and perifollicular infiltrates and follicular mucin deposition histopathologically. The patient showed 11% of flower-shaped atypical lymphocytes in blood examination and positive human T-cell leukemia virus type 1 antibody in serology, consistent with the chronic type of ATLL. This case seems to be a very rare association of follicular mucinosis and chronic ATLL, suggesting that malignant T cells may have a feature of folliculotropism as well as epidermotropism.

Angiolymphoid hyperplasia with follicular mucinosis.

Follicular mucinosis occurring along with angiolymphoid hyperplasia with eosinophils (ALHE) has been described in a 54-year-old female. The patient presented with pruritic erythematous papules on the left frontoparietal scalp. Histopathological examination showed prominent blood vessels in the dermis lined by plump histiocytoid endothelial cells that were surrounded by a dense lymphoid infiltrate with numerous eosinophils; these findings are typical of angiolymphoid hyperplasia with eosinophils. Features of follicular mucinosis were observed in the same section with several hyperplastic follicular infundibula containing pools of mucin in the infundibular epithelium. The concurrent occurrence of these two distinct histopathological patterns in the same biopsy specimen has been described in only three cases to date.

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

Folliculotropic mycosis fungoides with follicular mucinosis--case report.

Reports on clinical and histologic follicular alterations in patients previously diagnosed with mycosis fungoides (MF) or at the time of MF diagnosis are rare. The clinical and histologic criteria to distinguish MF associated with follicular mucinosis from follicular MF are a matter of debate. A patient is described with advanced clinical and histologic alterations predominated by follicular lesions and presence of mucin. In the early stage of the disease, folliculotropism was clinically and histologically present but less pronounced than epidermotropism and classic plaque-like lesions. The patient died four years after the diagnosis. As the term 'folliculotropic' describes a particular histopathologic finding, we consider it correct to use the term "folliculotropic MF" to denote atypical lymphocyte folliculotropism in the absence or presence of mild epidermotropism, presence of mucin, or no evidence for intrafollicular mucin. Folliculotropic MF seems to represent a specific clinicopathologic entity which may have a poorer prognosis than classic MF.

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