Cutaneous Langerhans cell histiocytosis: study of Asian children shows good overall prognosis.

AIM

To describe an Asian population with isolated cutaneous Langerhans cell histiocytosis (LCH), presenting a review on previous studies on this subgroup of patients and comparing our cohort with other studies of cutaneous LCH with systemic involvement.

METHODS

All patients diagnosed as LCH with cutaneous involvement presenting to a tertiary paediatric hospital (KK Women's & Children's Hospital) between January 2001 and December 2011 were reviewed. Information recorded included clinical presentation, investigation results, treatment and outcome.

RESULTS

We identified 10 patients with cutaneous manifestations of LCH. Six had isolated cutaneous LCH with no visceral or bony involvement, while four had cutaneous lesions with involvement of other organ systems. Of the patients with isolated skin involvement, three were treated with surgical excision, one with topical corticosteroids and two with multi-agent chemotherapy. The four patients with concomitant systemic involvement were treated with multi-agent chemotherapy. None of our patients with isolated cutaneous LCH progressed to multisystem disease during the follow-up period.

CONCLUSION

Cutaneous LCH, with or without other organ involvement at diagnosis has a good overall prognosis. However, long-term follow-up is recommended in view of possible recurrence.