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Within the literature, there is overlap in the histopathological features described in eosinophilic folliculitis associated with chronic lymphocytic leukemia (CLL), eosinophilic dermatosis of hematologic malignancy, and acneiform follicular mucinosis. These disorders are described with varying degrees of superficial and deep lymphocytic and eosinophilic inflammation demonstrating perivascular, perifollicular, and folliculocentric involvement with or without follicular mucin deposition. Given significant histopathological overlap, these diagnoses may represent a continuum on a spectrum of dermatoses. Here, we present two cases with histopathological elements that reflect components of this clinicopathological spectrum and compare our findings with previously reported cases to compare and contrast reported features. Our first case is a 71-year-old African American man with long-standing CLL who developed a pruritic erythematous papular eruption on the face and chest with biopsy revealing a dense folliculotropic lymphocytic infiltrate with conspicuous eosinophils and follicular mucinosis. Our second case is a 70-year-old Caucasian man recently diagnosed with CLL/small lymphocytic lymphoma who developed an erythematous papular rash on the neck and face with biopsy revealing superficial and deep perivascular and periadnexal lymphocytic inflammation with scattered eosinophils. Characterization of our two cases and comparison with available literature suggest that these disorders may represent a continuum of dermatoses., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

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

Background: The recognition of folliculotropic mycosis fungoides (FMF) may pose diagnostic challenges, owing to the variety of histopathological findings., Objective: In this study, our aim is to describe the broad spectrum of the histopathological patterns in a total of 86 biopsies from 38 patients with FMF, together with the clinical features., Results: The most frequent histopathologic pattern was the folliculocentric/folliculotropic pattern, with or without follicular mucinosis. Keratin-filled cysts and comedones were the second most common pattern in the biopsies. Other less common findings included widening of the hair follicle orifis with keratotic plugging, reminiscent of keratosis pilaris, granuloma formation, eosinophilic or suppurative folliculitis and basaloid folliculolymphoid hyperplasia. Coexisting syringotropism was present in some biopsies. The CD4 : CD8 ratio was at least 4 : 1 or more in most biopsies. Grouped follicular papules and patch/plaque lesions with follicular prominence were the most frequent clinical findings. Folliculocentric lesions such as milia, cysts and acneiform lesions, alopecia, loss of hair or eyebrows were also seen. In 6 out of 38 (15.8%) patients, transformation to large-cell lymphoma was observed during the follow-up., Conclusion: The awareness and the identification of the various histopathological presentations of FMF by pathologists, as well as by clinicians, are imperative to prevent diagnostic errors., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Topical bexarotene 1% gel is currently FDA-approved for early stage (IA and IB) persistent or refractory cutaneous T-cell lymphoma (CTCL). No uniformly effective therapy exists for follicular mucinosis, although several treatments are routinely used. There are no known reports of topical bexarotene being used in the treatment of idiopathic follicular mucinosis when there is no association with CTCL. This article reports the first case of bexarotene gel to successfully treat persistent idiopathic follicular mucinosis., Materials and Methods: This study describes a 34-year-old Caucasian male with idiopathic follicular mucinosis. The patient had treatment failure with clobetasol 0.05% ointment and narrow-band UVB. Intralesional injections with triamcinolone 5 mg/ml were successful for treating the plaques in the beard area. The patient was treated with bexarotene 1% gel applied twice a day to the persistent plaques on the lower extremities. The patient reported significant improvement in hair growth after only six weeks of treatment. The treatment was decreased to once a day due to erythema, and he had complete hair regrowth at 26 weeks., Discussion: Several treatments have been described in the literature, such as corticosteroids, psoralen plus ultraviolet A (PUVA) light therapy, topical nitrogen mustard, and radiation therapy. Isolated cases have documented the beneficial responses of pimecrolimus, dapsone, indomethacin, minocycline, isotretinoin, hydroxychloroquine and interferons. No single treatment has been shown to be consistently effective., Conclusion: Topical bexarotene 1% gel should be considered for patients with idiopathic follicular mucinosis resistant to standard treatment., (© 2013 The International Society of Dermatology.)

Follicular mucinosis can develop as a primary form or associated with other benign or malignant skin conditions. We present a case of primary follicular mucinosis in which complete clinical remission was achieved after treatment with topical imiquimod 5%. Primary follicular mucinosis (PFM), also called idiopathic alopecia mucinosa, is considered a distinct entity, the clinical course of which is usually benign, but its pathogenesis remains unclear. There is no standard treatment for PFM, and a wide variety of therapies have been described. There are no previous reports of treatment of PFM with imiquimod., (© 2012 Wiley Periodicals, Inc.)

A 56-year-old Caucasian female presented with a 2-month history of alopecia. On examination, she had diffuse hair loss of her scalp with some discrete patches of nonscarring alopecia. Histopathology revealed an inflammatory nonscarring alopecia with prominent follicular mucinosis and findings suggestive of alopecia areata. The patient's alopecia completely resolved with oral prednisone. The histopathologic findings and clinical presentation are most consistent with a diagnosis of alopecia areata with follicular mucinosis, although the differential diagnosis is broad. As follicular mucinosis may be associated with both benign and malignant conditions, it is important to be cautious regarding the clinical diagnosis when this reaction pattern is observed histopathologically., (© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Follicular mucinosis (FM) and folliculotropic mycosis fungoides (MF) are rare in children, and data regarding long-term outcomes are limited. We sought to describe clinical and histopathologic findings of children with FM with and without MF, as well as treatments administered and clinical outcomes. We conducted a retrospective chart review of patients younger than 22 years (at time of diagnosis) with a biopsy demonstrating FM who were seen in the Dermatology Department at the Mayo Clinic from September 1, 1999, to September 1, 2010. Eleven patients (six male, five female) ages 11 to 19 years at the time of diagnosis met the inclusion criteria. Follow-up data were available for 10 patients, with a mean duration of 4.9 years. The head, neck, and extremities were the most common sites of involvement, and lesions were follicular-based papules (18%), scaly alopecic patches and plaques (45%), or a combination of the two (36%). Overall, three patients were confirmed to have MF. T-cell receptor gene rearrangement demonstrated clonality in two cases and was equivocal in one case. Treatments included topical corticosteroids, topical retinoids, oral minocycline, and, in patients with MF, ultraviolet light and topical bexarotene. Lesions resolved completely in seven patients, partially in one, and not at all in two (no follow-up data on one patient). Of the three patients with MF, two had complete resolution, and one has intermittent flares. To our knowledge, no patients developed other lymphoproliferative disorders. FM in children is rare. A histopathologic diagnosis of FM does not equate to folliculotropic MF in all cases. Most patients responded to treatment with topical steroids, topical retinoids, or phototherapy. In our series of patients, the disease ran a benign course., (© 2012 Wiley Periodicals, Inc.)

There exists no treatment of choice for follicular mucinosis (FM). Historically two distinct entities of FM have been proposed: FM of children and young adults not associated with other diseases ('idiopathic' FM), and FM in elderly patients associated with mycosis fungoides and Sézary syndrome ('lymphoma-associated' FM). Nowadays it is suggested that 'idiopathic' FM might represent a localized form of cutaneous T-cell lymphoma. Six patients with 'idiopathic' FM were treated with hydroxychloroquine (HCQ) at a dose of 200 mg three times daily for 10 days followed by a dose adjusted to the ideal body weight, usually 200 mg twice daily. All patients showed an improvement of 'idiopathic' FM already after 6 weeks and a complete remission with full hair regrowth after 2-5 months of HCQ therapy. In all patients no relapse occurred during follow up of between 3 and 23 years and no patient developed lymphoma. We conclude that HCQ is a highly effective therapy without significant side-effects in the treatment of so-called 'idiopathic' FM.

Follicular mucinosis is an uncommon inflammatory disorder characterized histologically by mucin accumulation in the follicular epithelium. The condition is generally divided into primary and secondary forms, the latter being frequently associated with mycosis fungoides. Lesional skin T-cell clonality has been documented in some patients with follicular mucinosis, even those with no histologic evidence of cutaneous lymphoma. In this report, we describe a patient with clonal idiopathic primary follicular mucinosis who had complete clinical, histologic, and molecular remission with minocycline therapy.

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 mucinosis is a rare disorder of unknown etiology characterized by accumulation of mucin in the sebaceous glands and outer root sheaths of the hair follicles. It is divided into a primary benign type and a secondary type mostly associated with lymphomas. No effective standard therapy for follicular mucinosis is available. We describe the case of a 21-year-old Caucasian male who had papules, nodules, and erythematous plaques on his left shoulder, left arm, and right scapular region. He was diagnosed as primary benign generalized follicular mucinosis, and treated with isotretinoin. Almost complete remission was achieved in 4 months.

Follicular mucinosis is a rare chronic inflammatory disease of unknown aetiology, presenting as mucin deposits around the follicles and sebaceous glands. It can progress to alopecia of the scalp and other hairy areas. Follicular mucinosis may be a benign primary idiopathic disorder or secondary to malignant lymphoproliferative disorders. It can present with shiny papules or sharply marginated infiltrated erythematous scaling plaques, with follicular accentuation on the scalp, neck, trunk and limbs. There are many local and systemic treatments. This paper discusses the case of an adult with an uncommon acneiform follicular mucinosis controlled with systemic corticosteroids.

Background: Alopecia mucinosa has been shown to be associated with many benign and malignant conditions. It can be seen in childhood but is seen more commonly in adulthood. Alopecia mucinosa is generally felt to occur in three settings: a primary idiopathic form, a form associated with malignancy, and a form secondary to inflammatory conditions. The histologic hallmark is the accumulation of mucin in the follicular epithelium, called follicular mucinosis. Therapy for alopecia mucinosa remains problematic., Objective: We present a representative case of alopecia mucinosa and discuss the etiology, histology, epidemiology, treatment, and prognosis., Conclusion: We report a case of idiopathic alopecia mucinosa that responded to minocycline with a complete remission and we review the literature on alopecia mucinosa.

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.

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.

We describe the case of a 33-year-old Caucasian male with follicular mucinosis successfully treated with isotretinoin. Follicular mucinosis is a primary idiopathic disease or a secondary, lymphoma-associated dermatosis. An effective standard therapy for this disease is unknown. In our case, isotretinoin led to a dramatic improvement of the skin lesions in about two weeks. To the best of our knowledge, the benefits of isotretinoin in the treatment of follicular mucinosis have never been reported previously. The efficacy of this drug could be mediated by a regulatory effect on the infiltrating cells and/or by a modulation of the target organ (skin) response to the infiltrating cells.

Follicular mucinosis can be a primary idiopathic disease or a secondary disease associated with lymphoma. When it appears in childhood or adolescence, it is usually primary and self-limited. We describe four cases of follicular mucinosis occurring in early adulthood that have had protracted courses. Each presented as an unusual acneiform eruption. Two of the cases demonstrated a clonal genetic rearrangement of the T-cell receptor within the cutaneous lymphoid infiltrate, a finding not previously reported. Although its significance is not clear, the clonal lymphocytic expansion indicates a need for continued surveillance of these patients.

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.

A patient with generalized follicular mucinosis is presented. On first attendance, the 63-year-old woman had been suffering from generalized pruritus for a year and from general loss of hair for 6 months. The pathognomonic histological features of follicular mucinosis in a histopathological examination of the skin allowed the diagnosis. The lack of a malignant dermal infiltrate and the rapid response to treatment with diamino-diphenylsulphone are, we feel, good prognostic indicators. The histology of alopecia mucinosa, its distinction from the other cutaneous forms of mucinosis by the limitation of mucinous change to the pilosebaceous follicles and the classification of follicular mucinosis into three clinical and prognostic patterns are discussed. The possibility that more widespread disease might be associated with an overt cutaneous lymphoma (mycosis fungoides) is emphasized.

A patient with primary follicular mucinosis is described, who developed multiple foci of involvement on the skin of the trunk and limbs. Combined therapy including thyroidin and liver function-normalizing drugs was fairly effective.

The present paper reports a study on a case of follicular mucinosis exacerbated by sunlight exposure. Provocation with standardized light testing was carried out on both normally pigmented skin and on areas of hypopigmentation representing a residual state after earlier skin lesions but without any signs of active mucinosis follicularis. Typical clinical and histologically verified lesions were provoked by the light test procedure, best seen in hypopigmented areas but with similar changes in normally pigmented skin. Characteristic findings developed gradually, assuming an appearance identical with that of the observed spontaneously elicited lesions after 3--4 weeks. Treatment with carotenoids periodically over several years has provided a proper light protective effect, as only minimal lesions have been noted during these periods, compared with extensive changes during similar periods without treatment.

We cared for nine patients who were less than 21 years old at the time of diagnosis of follicular mucinosis. Four had clearing of the lesions, four did not, and one was lost to follow-up. Follicular mucinosis, even in childhood, may be persistent and be associated with lymphoma, which can result in death. Because there is no clinical pattern or single histopathologic criterion that correlates with the outcome, clinical follow-up and assessment of several histopathologic criteria must be relied on when evaluating this condition in children.

Follicular mucinosis is usually regarded as an eruption consisting of follicular papules or infiltrated plaques with prominent follicular openings associated with loss of hair. We describe a patient who presented with an acute extensive dermatitis confined to the face which was later diagnosed as follicular mucinosis. We believe that such a florid presentation has not previously been reported in the literature and serves to illustrate the difficulty in distinguishing acute follicular mucinosis from acute eczematous dermatitis, pyogenic infections and inflammatory fungal infections.