HEALTH PROFESSIONAL · SOURCE READING

Isolated skin involvement

Source: Langerhans Cell Histiocytosis Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: January 6, 2025 · Captured 2026-09-09.

Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.

Context: Childhood LCH / Low-Risk Disease: Single-System or Multisystem LCH / Treatment of low-risk disease: single-system or multisystem LCH

Treatment options for patients with isolated skin involvement include the following:

Observation. Observation is recommended for all pediatric patients with asymptomatic skin-only LCH.[48]

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Therapy. Therapy is suggested only for patients with symptomatic disease, which includes extensive rashes, pain, ulceration, or bleeding.

Patients with skin-only involvement need to have a complete staging evaluation because 41% of these patients referred to one center had multisystem disease requiring treatment.[48] Careful clinical (but not radiological) follow-up of young infants with skin-only LCH is suggested because progression to high-risk multisystem disease is possible. Young children with skin-only LCH should be monitored periodically for many years because 1 of 19 children and 1 of 25 children in two series developed late diabetes insipidus.[31,49]

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For patients who require therapy, treatment options for symptomatic isolated skin lesions include the following:

Topical steroids. Medium- to high-potency steroids are effective, but recurrence after discontinuation is common.[50]

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Oral methotrexate. Oral methotrexate (20 mg/m2) weekly for 6 to 12 months.[86]

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Oral hydroxyurea. Oral hydroxyurea (20 mg/kg) daily for at least 12 months.[87]

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Oral thalidomide/lenalidomide.[88,89] Oral thalidomide 50 mg to 200 mg nightly.[88] Oral thalidomide/lenalidomide may be effective for both pediatric and adult patients.

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Topical nitrogen mustard. Topical application of nitrogen mustard can be effective for cutaneous LCH that is resistant to oral therapies, but not for disease involving large areas of skin.[90,91]

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Psoralen and long-wave ultraviolet A radiation (PUVA) or UVB. Psoralen and PUVA or UVB can be effective in skin LCH, but its use is limited by the potential for late skin cancers, especially in patients with light skin tones.[92,93]

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Chemotherapy. Systemic chemotherapy may be used in severe and symptomatic cases.

Radiation therapy. Although external-beam radiation therapy has been used, it has not proven to be reliably effective and may have severe side effects.[94,95]

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Preserved source evidence · Not medical advice