Follicular mucinosis in patients with hematologic malignancies other than mycosis fungoides: A clinicopathologic study.

BACKGROUND: Follicular mucinosis (FM), which is defined by mucin accumulation within follicular epithelium, may occur in mycosis fungoides (MF). FM without MF is occasionally reported in systemic hematologic malignancies and may be diagnostically challenging. OBJECTIVE: To describe clinicopathologic characteristics of FM in patients with hematologic malignancies other than MF. METHODS: Clinical data and histopathology features were analyzed in patients with FM and hematologic malignancies diagnosed between 1994 and 2017. RESULTS: A total of 18 patients with FM and systemic hematologic malignancies without cutaneous T-cell lymphoma (CTCL) were identified; 9 of them were discovered after hematopoietic stem cell transplantation. No patients with non-CTCL-associated FM (n = 46 [37 biopsy specimens]) developed CTCL during a mean follow-up of 4.3 years. Of the cases of CTCL associated with FM (n = 44 [31 biopsy specimens]), MF was the most common subtype (n = 38), although other CTCLs were identified. FM in patients with non-CTCL hematologic malignancies differed clinically from those with MF-associated FM, presenting most frequently with erythematous papules (P < .0001), without plaques (P <.0001), without alopecia (P = .001), and without histopathologically identified epidermal exocytosis (P = .013). LIMITATIONS: A retrospective study in a single cancer center. CONCLUSIONS: FM can present in systemic hematologic malignancies, including after hematopoietic stem cell transplantation. Papular lesional morphologic and histopathologic features may help to distinguish these cases from MF.

Juvenile-Onset Early-Stage Mycosis Fungoides-Associated Follicular Mucinosis: A Case Report.

We report a juvenile case of mycosis fungoides with prominent follicular mucinosis (FM). The patient was a 9-year old boy who presented with a 2-month history of enlarging alopecic patch with fine scales on the scalp. Dermatologic examination revealed orange-tan slightly palpable plaques with follicular prominence on his trunk. The patient and his family were not aware of these asymptomatic truncal plaques. Histopathologic examination of both-scalp and trunk-lesions revealed folliculotropic lymphocytic infiltration with mucin. Immunohistochemical study showed that lymphocytic infiltration was CD4 dominant. Flow cytometry analyses of peripheral blood were normal. Any abnormal populations and Sézary cells were not observed on blood smear. Polymerase chain reaction testing showed monoclonality for the T-cell receptor4-[Latin Small Letter Rams Horn] gene. Our patient had the clinical and histopathological diagnosis of follicular mycosis fungoides-associated follicular mucinosis.

Follicular Mucinosis in a Male Adolescent with a History of Acute Myelogenous Leukemia and Graft-versus-Host Disease.

Although many cases of follicular mucinosis are idiopathic, numerous others are associated with mycosis fungoides or, rarely, other neoplastic or inflammatory disorders. There are only three reported cases, all in adults, of follicular mucinosis arising in association with acute myelogenous leukemia, two of which involved mycosis fungoides-associated follicular mucinosis, including one case in which the patient had a preceding bone marrow transplant. We present the first reported case of follicular mucinosis arising in an adolescent with acute myelogenous leukemia and acute graft-versus-host disease after an allogeneic bone marrow transplantation.

Adult T-cell leukemia/lymphoma accompanying follicular mucinosis: a case report with review of the literature.

Follicular mucinosis is recognized as one of the histopathological reaction patterns characterized by the accumulation of mucin within follicular epithelium. It is induced by various causes including inflammatory diseases, and more than half of the cases are associated with malignant lymphoma, mainly mycosis fungoides. Herein, we describe the third documented case of adult T-cell leukemia/lymphoma (ATLL) accompanying follicular mucinosis. A 72-year-old Japanese male presented with persistent erythema in his arm and neck. Laboratory tests demonstrated positivity for human T-cell leukemia virus (HTLV)-1 antibodies. Histopathological study of the biopsy specimen from the neck revealed superficial perivascular, nodular, and intrafollicular lymphocytic infiltrations. These lymphocytes were small- to medium-sized and had convoluted nuclei. Mucoid material deposition was observed within the hair follicles, and it was digested by hyaluronidase. Immunohistochemically, these lymphocytes were positive for CD3, CD4, CD25, and Foxp3. Accordingly, an ultimate diagnosis of ATLL accompanying follicular mucinosis was made. The skin is the most common extralymphatic site of involvement of ATLL. The present case clearly demonstrated that albeit extremely rare, ATLL can cause follicular mucinosis. Therefore, ATLL should be included in the differential diagnostic consideration of follicular mucinosis.

Reactive benign follicular mucinosis: a report of 2 cases.

We report 2 cases of adolescents who developed follicular mucinosis following cutaneous infections. A 17-year-old adolescent boy was evaluated for a 2-week history of erythematous papules and plaques on his face and neck. One month prior to presentation a culture was taken that was positive for methicillin-sensitive Staphylococcus aureus-associated impetigo. Biopsies from 2 representative lesions demonstrated follicular mucinosis without evidence of folliculotropism or T cell gene rearrangements. A separate case involved a 17-year-old adolescent girl who presented with an edematous plaque on her right preauricular region and scattered erythematous papules and small annular plaques over her face 2 weeks following a herpes simplex virus type 2 (HHV-2) infection. on her face. Biopsy showed follicular mucinosis without evidence of epidermotropism or lymphocyte atypia. There was no herpesvirus cytopathic effect. The first case rapidly responded to an oral prednisone taper and the second case resolved over several weeks without further treatment.

A novel case of follicular mucinosis after autologous stem-cell transplantation for multiple myeloma.

Follicular mucinosis is a rare inflammatory disorder of unknown aetiology, characterized by mucin deposition in hair follicles and sebaceous glands. FM can occur as a benign idiopathic primary disorder or secondary to malignant lymphoproliferative processes, most notably mycosis fungoides. We report a novel case of FM developing after autologous stem-cell transplantation for multiple myeloma, a correlation not previously reported in the literature.

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.

Folliculitis, follicular mucinosis, and papular mucinosis as a presentation of chronic myelomonocytic leukemia.

Leukemias and Lymphomas can present in indolent and surprisingly unusual manners. Although uncommon, follicular lesions such as eosinophilic folliculitis have been reported in association with leukemia. However, follicular and papular mucinosis are novel associations for chronic myelomonocytic leukemia.

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

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.

Milia in regressing plaques of mycosis fungoides: provoked by topical nitrogen mustard or not?

OBJECTIVE: To report three cases of mycosis fungoides with milia formation in the regressing lesions. PATIENTS AND SETTING: Dermatology clinic of a university hospital (referral center). Three patients with mycosis fungoides with body surface involvement of 10% in one case (stage IIb) and exceeding 30% in two cases (stages IIb and III). All patients were treated with photochemotherapy and topical nitrogen mustard ointment in a concentration of 0.01%. After approximately 3 months multiple milia erupted on regressing plaques. RESULTS: The presence of milia was evident and was confirmed by histopathology. Regression of mycosis fungoides was noted in these plaques both clinically and in comparison with the pretreatment histologic appearance. Two of the patients showed a histological picture of follicular mucinosis. CONCLUSIONS: We do not know the significance of milia in mycosis fungoides (MF). However, we suggest that follicular rupture or a degenerative process might result in milia formation.

Follicular mucinosis occurring after bone marrow transplantation in a patient with acute lymphoblastic leukemia.

Follicular mucinosis (FM) is characterized histologically by mucinous degeneration of the outer root sheath of the hair follicle and sebaceous gland, accompanied by inflammatory infiltrate. It can occur as a primary idiopathic disorder or in association with benign or malignant diseases, most notably mycosis fungoides. In addition, it also can be found incidentally on histology. We describe an unusual case of follicular mucinosis in a 19-year-old man with acute lymphoblastic leukemia (ALL). One month after bone marrow transplantation, he developed cutaneous graft-versus-host disease (GVHD) involving the palms and soles, which was followed 12 days later by the appearance of multiple erythematous follicular papules and plaques on his face, auricles, and postauricular area. Biopsy of follicular plaque revealed changes of follicular mucinosis without evidence of graft-versus-host disease or leukemia cutis. The follicular rash was associated with prominent peripheral eosinophilia. The rash and eosinophilia resolved after 2 and 4 weeks, respectively. In conclusion, we report a case of FM occurring as a transient reaction during the course of cutaneous GVHD following bone marrow transplantation for ALL. Awareness of this condition may avoid undue concern that the rash might represent a manifestation of GVHD, cutaneous relapse of the hematological malignancy, or a drug allergy.

Follicular mucinosis associated with mycosis fungoides.

A 62-year-old man with a 13-year history of mycosis fungoides presented with a 2-month history of alopecia of the scalp. The mycosis fungoides had remained untreated for the previous 3.5 years. A biopsy specimen from the scalp showed follicular mucinosis in association with mycosis fungoides.

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.

Mucinosis folicular: nuevas entidades clínico-patológicas. Estudio clínico, histológico, inmunohistoquímico e inmunogenotípico de 23 pacientes* / Follicular mucinosis: new clinicopathologic entities. Clinical, histological, immunohistochemical and immunogenotypic study of 23 patients

En el presente trabajo se realiza una revisión clínica, histológica, inmunohistoquímica e inmunogenotípica de 23 pacientes con mucinosis folicular. Se incluyeron en el trabajo pacientes con mucinosis folicular primaria (MFP) y pacientes con mucinosis folicular secundaria (MFS) a linfoma sistémico o linfoma cutáneo de células T. Se seleccionaron del archivo de histopatología las biopsias con diagnóstico de mucinosis folicular desde 1975 al año 2000 con el único criterio de mostrar degeneración mucinosa en el epitelio folicular con un infiltrado inflamatorio acompañante. En todas las biopsias se efectuaron tinciones inmunohistoquímicas con marcadores pan B, pan T, T4 y T8. En casos seleccionados se realizó detección de reordenamiento clonal del receptor T mediante PCR de cortes en parafina de las biopsias. Se encontraron seis casos de MFS (30,4% del total), todos ellos varones de una edad media de 50,4 años. El linfoma más frecuentemente asociado fue la micosis fungoide. Dieciséis casos correspondían a MFP (ocho varones y ocho mujeres; edades entre 8 y 72 años). En este grupo de pacientes se identificaron cuatro formas clínicas de presentación: MFP localizada, MFP difusa, MFP acneiforme y MFP urticarial. No existieron diferencias histológicas sustanciales entre la MFS y la MFP. Tan sólo la forma urticarial de la mucinosis folicular presentaba características histológicas diferenciales, como fueron un abundante infiltrado inflamatorio y abundantes eosinófilos. Los hallazgos inmunohistoquímicos confirman que el infiltrado inflamatorio está formado preferentemente por linfocitos T helper. Los estudios de reordenamiento demostraron tres casos de MFP con clonalidad, sin que se pudieran encontrar características diferenciales entre éstos y las formas no clonales. Pese a la clonalidad ninguno de estos tres casos evolucionó posteriormente a linfoma, lo que sostiene la teoría de las dermatosis benignas clonales, recientemente propuesta. No obstante, parece aconsejable un seguimiento prolongado de los casos de mucinosis folicular primaria con clonalidad en el estudio genético (AU)

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.

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.