How is single-system Langerhans cell histiocytosis (LCH) treated?

Updated: Jun 12, 2020
  • Author: Christopher R Shea, MD; Chief Editor: William D James, MD  more...
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Solitary bone lesions are treated locally with curettage or excision. Painful bone lesions may require intralesional steroid injection (triamcinolone acetonide). Bisphosphonates such as zoledronic acid can also be used to reverse bone destruction and mitigate the pain of bony lesions. [63] Early treatment with vinblastine and prednisolone has been suggested for bony lesions at vital anatomic locations requiring prompt resolution. [28] Rarely, lesions that are unusually large and painful occur in inaccessible sites or involve vital structures require radiation therapy (3-6 Gy [300-600 rad]).

Polyostotic bone lesions are best treated with indomethacin or a short course of systemic steroids. [64] A case report on pharmacologic management of single-system bony disease using naproxen indicates that multiple COX antagonists may be used in treating this form of LCH. [65]

Localized skin disease is best treated with moderate-to-potent topical steroids (eg, mometasone furoate [Elocon] cream 0.1%, triamcinolone [Kenalog] cream 0.1%, fluocinolone [Synalar] ointment 0.025%) or superpotent topical steroids (eg, clobetasol propionate 0.05%). In cases of severe cutaneous involvement, topical nitrogen mustard (20% solution) may be used. Acitretin may also be an effective agent for patients with primarily cutaneous manifestations of LCH. [66]

Psoralen plus ultraviolet A (PUVA) is another effective modality for cutaneous-only LCH or for cutaneous involvement in multisystemic disease. PUVA consists of photosensitizing psoralens (8-methoxypsoralen or 5-methoxypsoralen) either applied topically or ingested systemically 2 hours prior to treatment with long-wave ultraviolet A (320-400 nm). The purpose of this treatment is to induce remission of skin diseases by inducing a repeated and controlled phototoxic reaction. The photoconjugation of psoralens with DNA produces an antiproliferative reaction in the skin, generates programmed cell death (apoptosis), and induces down-regulation of the cutaneous immune system. [67]

Ultraviolet B excimer laser has been found in at least one case report to offer effective adjuvant therapy in the management of cutaneous LCH, and it may be particularly useful for patients with comorbidities who cannot tolerate more aggressive treatment. [68]

For single lymph node infiltration, excision is the treatment of choice. Regional lymph node enlargement can be treated with a short course of systemic steroids. Treatment-resistant nodes with sinus tracts to the skin may require systemic chemotherapy.

Smoking cessation is an important intervention in cases of pulmonary LCH. [40]

Single-agent chemotherapy with cladribine (2-chlorodeoxyadenosine/2-CdA) may be a promising treatment for single-system pulmonary LCH. [69, 70]


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