Mucocutaneous Langerhans Cell Histiocytosis After Topical Therapy Has Not Achieved Improvement

This protocol addresses the next management step for patients with mucocutaneous Langerhans cell histiocytosis whose skin lesions did not respond to initial topical treatment.

Prior therapy — insufficient response

Topical therapy — including topical triamcinolone for disease limited to small areas of skin — did not produce any degree of improvement in cutaneous lesions on skin examination. This protocol defines the next line of treatment following that failure.

Next-line approach (partial overview)

Oral systemic therapy forms the basis of this next step. The approach involves low-dose oral methotrexate-based regimens and/or immunomodulatory agents — the specific agent selection, combinations, and sequencing are laid out in the full protocol.

Full regimen, all options, and the complete supporting evidence are available via the link below.

Response criterion

Any degree of improvement in cutaneous lesions on skin examination is considered a response.

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References

DOI: 10.1182/blood.2021014343

Locally extensive LCH of the skin is usually less sensitive to topical or systemic steroid therapy but usually responds to treatment with hydroxyurea or low-dose methotrexate.
For multifocal cutaneous LCH, recommended treatments are topical therapy, oral low-dose weekly methotrexate ± prednisone/6-MP, hydroxyurea, or IMiDs.
Multifocal skin disease is usually responsive to hydroxyurea, IMiDs, oral low-dose methotrexate therapy with or without prednisone/6-mercaptopurine.
Of the IMiDs, both thalidomide and lenalidomide have been reported to elicit responses, with some lasting over a year.
Any degree of improvement in clinical or radiographic findings is considered a response.

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