Profile and Outcome of Childhood Mycosis Fungoides in Singapore

Abstract

Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphoma. It usually occurs in middle-aged and elderly persons, although several reports have described its occurrence in young children. The aim of this study was to review the profile and outcome of childhood MF in Singapore from 1989 to 1998. A total of nine patients (six males and three females) were diagnosed with MF before the age of 21 years. There were four Chinese, four Malay, and one Indian. The age at the time of histologic diagnosis ranged from 6 to 20 years (mean 14.3 years). Eight of the nine patients presented with hypopigmented patches and plaques. According to TNM staging, three were in stage 1A and six in stage 1B. The treatment modalities included psoralen plus ultraviolet A (PUVA) (n = 5), UVB (n = 2), and potent topical steroids (n = 2). We found that PUVA induced a faster clinical remission, but maintenance PUVA was required to prolong the relapse-free interval. This study also highlighted the need to consider MF in the differential diagnosis of hypopigmented dermatoses in dark-skinned individuals, especially if they occur on the buttocks.

A study of clinicopathologic profile of 15 cases of hypopigmented mycosis fungoides

“…In our study, all 15 patients were Asians with dark skin type, similar to the findings in the study by Tan et al, [12] that consisted of three Chinese, four Malays, and one Indian, whereas the study by Wain et al, [13] revealed 4 Asian, 3 African Caribbean (dark skinned) and 1 Caucasian (fair skinned) patient. All seven patients in a study by Lambroza et al, [14] too showed predilection for persons with brown or black skin [ Table 2].…”

“…Tan et al [12] Wain et al [13] Lambroza et al [14] Ardigo et al [18] No. occurrence of melanin incontinence was seen in 46.6% of our cases.…”

Outcome in 34 patients with juvenile‐onset mycosis fungoides

“…2,8 In the current study, 8 of 34 (24%) patients had hypopigmented lesions compared with a similar incidence of 5 of 24 (21%) patients in the juvenile series reported by Zackheim et al 2 In a small series (n ϭ 9) of patients with MF reported by Tan et al, 10 8 had purely hypopigmented lesions. This subset is most commonly reported in patients with skin types IV-V. 12 In the current study, of the eight patients with hypopigmented lesions, only one was Caucasian, four were Asian, and …”

Mycosis fungoides with a CD56+ immunophenotype

“…Despite this, the prognosis with regard to disease-specific survival at 5 and 10 years is similar for juvenile-versus adultonset disease. 2,[8][9][10][11] All 4 patients with CD56 1 cytotoxic MF in whom clinical details are available were younger than 40 years at disease onset with one (patient 2) developing MF at 3 years of age. These patients were all considerably younger than the typical age of MF disease onset; however, the small numbers of patients with CD56 1 MF preclude significant statistical analysis.…”