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.

Eosinophilic folliculitis, eosinophilic dermatosis of hematologic malignancy and acneiform follicular mucinosis: Two case reports and a review of the literature highlighting the spectrum of histopathology.

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.

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.

The clinical features and histopathologic patterns of folliculotropic mycosis fungoides in a series of 38 cases.

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.

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

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A case of idiopathic follicular mucinosis treated with bexarotene gel.

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.

Treatment of primary follicular mucinosis with imiquimod 5% cream.

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.

Prominent follicular mucinosis with diffuse scalp alopecia resembling alopecia areata.

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.

Pediatric follicular mucinosis: presentation, histopathology, molecular genetics, treatment, and outcomes over an 11-year period at the Mayo Clinic.

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.

Treatment of so-called idiopathic follicular mucinosis with hydroxychloroquine.

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.