Clinical and histopathologic spectrum of alopecia mucinosa/follicular mucinosis and its natural history in children

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• 作者：Alex Zvulunov ; MD ; MHA^a ; ^e ; ^{azvulun@bgu.ac.il} ; Vered Shkalim ; MD^b ; ^d ; Dan Ben-Amitai ; MD^a ; ^d ; Meora Feinmesser ; MD^c ; ^d
• 关键词：alopecia mucinosa ; cutaneous T-cell lymphoma ; follicular mucinosis ; mycosis fungoides
• 刊名：Journal of the American Academy of Dermatology
• 出版年：2012
• 出版时间：December, 2012
• 年：2012
• 卷：67
• 期：6
• 页码：1174-1181
• 全文大小：1141 K

文摘

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Background

Some authorities consider alopecia mucinosa (AM)/follicular mucinosis (FM) to invariably represent mycosis fungoides (MF). This understanding of AM/FM derives from observations in adults.

Objectives

We sought to explore the clinicopathologic features and natural history of pediatric AM/FM.

Methods

Medical records were searched for children given the diagnosis of AM/FM from 1998 through 2009. Diagnosis of AM/FM was defined as the presence of well-demarcated hairless plaques with follicular prominence plus an abundance of mucin on histopathologic examination.

Results

Forty children with a clinical diagnosis of AM/FM were identified. Nine did not meet the inclusion criteria. In the 31 remaining cases (16 boys, 15 girls) the mean age at onset was 9 ¡À 3.5 years. Histopathologic examination showed folliculotropism in 28 patients (90 % ) and epidermotropism in 15 (48 % ). Twelve cases fulfilled the International Society of Cutaneous Lymphomas (ISCL) diagnostic criteria for early MF. The histopathologic findings were typical of MF in only in two of these cases. T-cell receptor gene rearrangement was positive in 3 of 6 (50 % ) of tested samples, one in a patient who fulfilled the ISCL criteria for early MF. Mean duration of follow-up was 6.2 ¡À 3.7 years. All skin lesions resolved and none persisted or recurred. Hodgkin lymphoma was diagnosed 6 months after diagnosis of AM/FM in one patient.

Limitations

This was a retrospective study.

Conclusions

Although some pediatric cases meet the diagnostic criteria for MF, AM/FM cannot be regarded unequivocally as early follicular MF in this age group. We suggest the current diagnostic criteria for early MF should exclude children with AM/FM. Long-term follow-up of children with AM/FM is nevertheless warranted.