Pediatric Mycosis Fungoides in Singapore: A Series of 46 Children

Abstract

Few studies have evaluated Asian children with mycosis fungoides (MF). We report a series of patients from a tertiary dermatologic institution in Singapore. A retrospective review was performed of patients younger than 16 years old diagnosed with MF between 2000 and 2008 at the National Skin Centre, Singapore. Forty-six patients were identified. At initial presentation, a provisional diagnosis of MF was made in 19 patients (41.3%), pityriasis lichenoides chronica (PLC) in 11 (23.9%) and postinflammatory hypopigmentation due to eczema or other causes in 11 (23.9%). After skin biopsy, the hypopigmented variant of MF was diagnosed in 42 patients (91.3%). There was one case each of PLC-like MF, pigmented purpuric dermatosis-like MF, classic MF, and solitary MF. Pityriasis lichenoides coexisted in three cases (6.5%). All except one patient presented with the early patch-plaque stage of disease (stage IA/B). The disease did not progress in any of our patients after a mean follow-up of 71.0 ± 52.5 months. Twenty-seven patients (58.7%) had complete disease clearance after a mean duration of 27.1 ± 28.1 months; 15 (49.7%) of 32 patients who received narrowband ultraviolet B treatment had complete clearance within an average of 8.9 ± 5.3 months, but 7 patients relapsed within 14.9 ± 14.8 months. One patient with solitary MF failed multiple treatment modalities before eventually achieving disease clearance with photodynamic therapy. Hypopigmented MF is the most common MF variant in Asian children. The diagnostic difficulty is in differentiating this from PLC, which may coexist with MF. Long-term prognosis is generally favorable.

Childhood Hypopigmented Mycosis Fungoides: A Rare Diagnosis

“…Fortunately, this condition is exceedingly rare, particularly in children who generally present with early stage disease consisting of limited or widespread patch or plaque stage. Palpable lymph nodes are usually free of disease, and the majority of affected children are diagnosed with stage Ia, Ib, or IIa MF, which is in line with the present case [4, 9–13]. An analysis of 36 pediatric patients from Kuwait reported that patch stage disease was the most common clinical variant (75%) and most patients had stage Ib disease [11].…”

“…Scarlett Boulos and coworkers showed that 41% and 56% of their 34 cases of juvenile-onset MF (median age at diagnosis 14 years) were at stages Ia and Ib, respectively [4]. Likewise, other studies reported identical findings [12, 13]. …”

“…Heng and coworkers showed that PUVA is now used much less often than NBUVB phototherapy, since it is considered to be safer [13]. Similarly, a retrospective study from Koh and Chong concluded that NBUVB phototherapy is an effective and safe treatment for early stage MF in children, yet recurrence is frequent.…”

Hypopigmented mycosis fungoides: a retrospective clinicohistopathologic study

“…It is important that the possibility of progression in this relatively indolent entity not be overlooked. There is lack of evidence to state that HMF predilects for darker skin; however, statistically, literature shows more cases in skin types IV‐V such as blacks, Asians, Hispanics and other ethnic minorities . This disparity could be for various reasons such as patients with lighter skin having difficulty noting the lesions and thus less likely to seek medical attention.…”

Childhood hypopigmented mycosis fungoides: a commonly delayed diagnosis

“…Histologically, the typical features of PL are parakeratosis, lymphocytic infiltrate, exocytosis, spongiosis and red blood cell extravasation. In the presented case, the patient had a continuous progressive course, the progression of erythematous papules to hypopigmented lesions was not observed and there were none of the histological features usually associated with PL or with posthypopigmented inflammation 3 7. Although not a common feature of MF, erythematous papules were already described particularly in HMF 8…”