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.

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.

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.