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AM/FM not synonymous with mycosis fungoides in children

Unlike many observations made in adults, a diagnosis of alopecia mucinosa/follicular mucinosis could not be regarded unequivocally as early follicular mycosis fungoides in children, according to findings in a recent study.

Researchers in Israel conducted a retrospective study of 31 children (16 boys) to determine the clinicopathologic features and natural history of pediatric alopecia mucinosa (AM)/follicular mucinosis (FM). Using medical records of children diagnosed with AM/FM from 1998 through 2009, the researchers defined AM/FM as the presence of well-demarcated hairless plaques with follicular prominence and an abundance of mucin during histopathologic examination. The mean age of the children at onset was 9 years.

Disease was found primarily (78%) on patients’ limbs. Eight children had a single lesion; 23 had multiple lesions. Ninety percent of patients presented with folliculotropism and 48% showed epidermotropism. Twelve patients met the diagnostic criteria of the International Society of Cutaneous Lymphomas (ISCL) for early mycosis fungoides (MF), yet histopathologic findings were typical of MF in only two cases.

Treatment consisted of mild- to moderate-potency topical corticosteroids in 30 patients, including one of the two cases defined as early follicular MF. All but one patient responded rapidly to treatment for follicular papules and the gradual disappearance of postinflammatory hypopigmentation. Mean duration of follow-up was 6.2 ± 3.7 years with all skin lesions resolved and no recurrence. One patient developed Hodgkin’s lymphoma 6 months after AM/FM diagnosis.

“Childhood AM/FM is not unequivocally synonymous with MF,” the researchers said. “ … the ISCL diagnostic algorithm for early MF should exclude children with AM/FM. Long-term follow-up of children with AM/FM is nevertheless warranted.”