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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., Competing Interests: Declaration of Competing Interest None., (Copyright © 2022 Elsevier B.V. All rights reserved.)

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., (Copyright © 2019. Published by Elsevier B.V.)

Competing Interests: None

Competing Interests: None

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., (© 2018 British Association of Dermatologists.)

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.

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.

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.

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., (© 2013 Wiley Periodicals, Inc.)

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., (Copyright © 2012 John Wiley & Sons A/S.)

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.

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.

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.

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.

Objective: To determine the clinicopathologic features and the disease course of patients with follicular mycosis fungoides (MF)., Design: A multicenter, 14-year, retrospective cohort analysis., Setting: Dutch Cutaneous Lymphoma Group., Patients: Fifty-one patients with the clinicopathologic features of follicular MF with (n = 49) or without (n = 2) associated follicular mucinosis. Follow-up data were compared with those of 158 patients with the classic epidermotropic type of MF, including 122 patients with generalized plaque-stage MF (T2 N0 M0) and 36 patients with tumor-stage MF (T3 N0 M0)., Observations: Characteristic clinical features not or rarely observed in classic MF were the preferential localization of the skin lesions in the head and neck region (45 of 51 patients), the presence of follicular papules, alopecia, acneiform lesions, mucinorrhoea, and often severe pruritus. Characteristic histologic findings were the presence of perifollicular neoplastic infiltrates with a variable degree of folliculotropism, but generally no epidermotropism, follicular mucinosis (49 of 51 cases), and often a considerable admixture of eosinophils and plasma cells. Response on initial treatment, risk of disease progression (development of extracutaneous disease and/or death from lymphoma), and disease-specific and overall survival of patients with follicular MF were worse than in classic MF patients. The actuarial disease-specific survival was 68% at 5 years and 26% at 10 years., Conclusions: Follicular MF shows distinctive clinicopathologic features, is more refractory to treatment, and has a worse prognosis than the classic type of MF; it should be considered a distinct type of cutaneous T-cell lymphoma. Based on these results and those of other studies, we suggest the term follicular MF for cases with or without associated follicular mucinosis.

Context: Beginning in 1957, patients have been described with localized alopecia characterized histopathologically by mucin deposition within hair follicles (follicular mucinosis [FM]). At least 2 distinct diagnostic entities have been proposed: one occurring in children and young adults without association with other diseases ("idiopathic" FM), the other occurring in elderly patients and associated with mycosis fungoides or Sézary syndrome ("lymphoma-associated" FM)., Objective: To determine whether idiopathic and lymphoma-associated FM are distinct or related entities., Design: Case series., Setting: Department of Dermatology, University of Graz, Graz, Austria., Patients: Forty-four patients with FM were divided into 2 groups. Group 1 comprised 16 patients (mean age, 37.5 years) with no associated mycosis fungoides or Sézary syndrome; group 2 was made up of the other 28 (mean age, 52.2 years), who had clinicopathologic evidence of cutaneous T-cell lymphoma., Results: Mean age was lower in patients with idiopathic FM, but a considerable overlapping among the 2 groups was present. Location on the head and neck region was common in both groups, but most patients with lymphoma-associated FM had lesions also on other body sites. In fact, solitary lesions at presentation were common in patients with idiopathic FM (11 [68.8%] of 16 patients), but uncommon in those with lymphoma-associated FM (2 [7.1%] of 28 patients). Histopathologic findings did not allow clear-cut differentiation of the 2 groups. Finally, a monoclonal rearrangement of the T-cell receptor gamma gene was demonstrated by polymerase chain reaction analysis in about 50% of tested cases from each group., Conclusions: Criteria previously reported to differentiate idiopathic from lymphoma-associated FM proved ineffective. In analogy to localized pagetoid reticulosis (Woringer-Kolopp disease), small-plaque parapsoriasis, and so-called solitary mycosis fungoides, idiopathic FM may represent a form of localized cutaneous T-cell lymphoma.

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.

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.

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.

Ofuji papuloerythroderma is a distinctive clinical entity of unknown etiology, which may occasionally be associated with B cell and T cell lymphoma or visceral malignancy. We report a case of papuloerythroderma associated with follicular mucinosis in mycosis fungoides (MF) that raises the possibility of papuloerythroderma as a form of prelymphomatous skin eruption. This specific papuloerythroderma responded well to the Re-PUVA treatment, which is a combination of etretinate and PUVA photochemotherapy.

The term HIV-associated eosinophilic folliculitis (EF) designates an idiopathic dermatitis that appears in HIV-infected patients with different clinical manifestations but with a distinctive histological feature characterized by a predominantly eosinophilic infiltrate in the follicular infundibula. On the other side, follicular mucinosis (FM) is a reaction pattern in the follicular epithelium, characterized by a mucinous degeneration of the outer sheath of follicles and sebaceous glands. It has been described in association with a variety of unrelated conditions. We report 2 HIV-infected patients with a pruritic papular eruption. Histopathological study revealed the coexistence of EF and FM. To our knowledge, this is the first report that describes this association. The possible relationship between these two entities is discussed.

Xanthoerythrodermia perstans (XEP) is a distinctive variant of large-plaque parapsoriasis. Along with alopecia mucinosa and lymphomatoid papulosis, there is a strong association between large-plaque parapsoriasis and evolving cutaneous T-cell lymphoma (CTCL). In some reports, large-plaque parapsoriasis was suggested to be a precursor lesion that converted to CTCL in 10 to 30 percent of cases. We describe a patient who presented clinically with both XEP and alopecia mucinosa and was subsequently shown to have CD-30 CTCL.

The diagnosis of cutaneous T-cell lymphoma in patients under 20 years of age is extremely rare. We report five patients diagnosed before 20 years of age who illustrate the striking variations in clinical and histologic features as well as disease progression. We feel this information stresses the importance of multiple biopsies in young patients with chronic dermatoses.

We present a case of pityriasis rubra pilaris (PRP)-like eruption, in association with human immunodeficiency virus (HIV) infection, clinically characterized by follicular papules with some elongated spines and comedo-like lesions. The lesions were located on the back, flanks, and proximal extremities. Orthokeratotic follicular plugs and perifollicular mucinous degeneration were consistently observed on three biopsies. The most characteristic histological features of PRP were absent. To our knowledge, this is the first reported case of this kind. Such a case could suggest an atypical PRP modified by HIV infection. It is also possible that it represents a new entity associated with advanced HIV infection (horny perifollicular mucinosis).

The case of a 40-year-old man with follicular mucinosis and simultaneous cutaneous T-cell lymphoma is reported. This observation emphasizes 1) the difficulties to interpret the lymphoid infiltration which accompanies a follicular mucinosis, 2) the necessity of immunochemistry and multiple biopsies in order not to ignore an associated lymphoma.

The pathological features of alopecia areata were described in association with those of follicular mucinosis in the scalp pathology of an 18-year-old woman. The immunohistochemical picture of the inflammatory infiltrate showed a high CD4/CD8 ratio (25:1), which was significantly different from the CD4/CD8 ratio in patients with alopecia areata. The abundance of helper/inducer cells along with the involvement of the upper part of the hair follicles might explain the development of follicular mucinosis in this case. Different possibilities were discussed, but the clinical presentation and the follow-up favored the clinical and pathological interpretation of alopecia areata with incidental findings of follicular mucinosis.

Among 33 patients with the histologic diagnosis of follicular mucinosis (alopecia mucinosa) made at our institution between 1982 and 1989, 9 had mycosis fungoides diagnosed concomitantly. Three other patients had lymphoproliferative disorders, and two had Kaposi's sarcoma. Analysis of biopsy features such as epidermal lymphocytic exocytosis, periappendageal infiltrate, and deposition of mucin revealed no predominant finding that distinguished a benign course from mycosis fungoides. A predominance of eosinophils in the infiltrate was suggestive of benign follicular mucinosis rather than mycosis fungoides. Gene rearrangement studies detected three clones in three patients with follicular mucinosis; two were in patients with mycosis fungoides, and one was in a patient with dermatitis. The outcome of these three patients is pending further follow-up. No histopathologic or clinical features distinguished these patients from the others.

This is a case report of a patient with mycosis fungoides coexisting with follicular mucinosis. The simultaneous occurrence of these two conditions has been documented. There is controversy as to whether mycosis fungoides in patients with follicular mucinosis arises de novo or whether follicular mucinosis can evolve into mycosis fungoides. The relationship between the two conditions is discussed.

Two cases of follicular mucinosis which developed into lymphoma are reported. The infiltrative atypical lymphocytes proved to be T cells, which were identified by monoclonal antibodies. The association of follicular mucinosis and lymphoma in Japan is estimated to be 9.4%.

Follicular mucinosis is described to our knowledge for the first time in angiolymphoid hyperplasia. In general, follicular mucinosis may be regarded as a peculiar, nonspecific histological reaction pattern in follicular epithelium that may occur on its own or in association with other pathological processes, particularly lymphomas. The unusual and characteristic features of angiolymphoid hyperplasia revealed by electron microscopy are irregular vessels lined by atypical endothelial cells with convoluted nuclei and large cytoplasmic vacuoles.

Association of angiolymphoid hyperplasia (pseudopyogenic granuloma type) and follicular mucinosis in the same lesion on the scalp of a 30-year-old woman. A similar case has been reported recently.

Fifty-nine patients with the histologic diagnosis of follicular mucinosis (alopecia mucinosa) were evaluated retrospectively. Thirty-seven were male and 22 were female; ages ranged from 10 to 76 years. Of 19 patients with mycosis fungoides, 16 had initial lesions of follicular mucinosis on the trunk or extremities. Two patients had Hodgkin's disease and follicular mucinosis; both were younger than 20 years of age. Seven other patients were 20 years of age or younger. Two of the nine adolescent patients had persistent plaques of follicular mucinosis up to 18 years after diagnosis. Evaluation of biopsy specimens for lymphocytes, eosinophils, nonlymphoid cell infiltration, epidermal lymphocytic exocytosis, mucin deposition, and epidermal hyperplasia revealed no predominant feature that differentiated the group with benign disease from the group with mycosis fungoides. We conclude that no single clinical or histopathologic observation predicts which patients with follicular mucinosis will have a benign course and that evaluation of multiple clinical and histologic variables is necessary.

A study was undertaken in an attempt to identify useful histologic criteria that may allow differentiation between benign idiopathic and mycosis-fungoides-associated follicular mucinosis. We chose young patients because no person under 20 years of age with coexisting follicular mucinosis and mycosis fungoides disease has ever been reported. Our three most important observations in benign juvenile idiopathic follicular mucinosis were as follows: The lymphocytic infiltrate was generally confined to follicular, perifollicular, or perivascular zones with no extension of either normal or atypical mononuclear cells into the epidermis or into papillary/reticular dermis. Within follicular epithelium there were dense collections of lymphocytes with occasionally atypical-appearing nuclei in three of the eight patients, but never as Pautrier microabscesses. There was absence of a significant associated plasma cell or eosinophil-containing inflammatory dermal infiltrate. These findings are in contrast to those of older patients with follicular mucinosis and mycosis fungoides.

Alopecia mucinosa was found in the hypopigmented skin of two black patients. Alopecia mucinosa should be included in the differential diagnosis of hypopigmented papular skin lesions.