Pediatric Follicular Mucinosis: Presentation, Histopathology, Molecular Genetics, Treatment, and Outcomes over an 11‐Year Period at the Mayo Clinic

Abstract

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.

Novel approaches to vitiligo treatment via modulation of mTOR and NF-κB pathways in human skin melanocytes

“…Vitiligo is the result of complex interactions of biochemical, environmental and immunological events in a permissive genetic milieu, while the precise mechanism of vitiligo pathogenesis has remained elusive3, 4. It has been recognized that this unique type of skin disorder is associated with melanocyte and possibly other skin cell apoptosis5, 6.…”

Well‐defined, slightly infiltrated, erythematosquamous plaque with focal alopecia of the eyebrow

“…lupus erythematodes, collagen vascular diseases, seborrheic keratosis, simple prurigo and acne vulgaris) and malignant lymphoproliferative diseases (especially MF, but also Hodgkin's disease, chronic lymphocytic leukemia and others) [3][4][5][6][7][8] . The latter occurs in the context of various benign infl ammatory diseases (e.g.…”

“…MF [ 3 ] . Typical clinical features are solitary or disseminated follicular-based papules and/or scaly patches and plaques with follicular prominence.…”

Primary follicular mucinosis – case report

“…7,8 Presença de epidermotropismo linfocitário, denso infiltrado perifolicular de células atípicas, idade avançada, múltiplas lesões disseminadas e recorrência apontam para MF associada a linfoma. 4,8,9 É importante que seja realizada uma avaliação dermatológica periódica dos pacientes com mucinose folicular, pela possibilidade de progressão para linfoma.…”