Treatment of single-system skin disease

Surgical excision. Localized lesions are rarely treated by surgical excision. Mutilating surgery, including hemivulvectomy, should be avoided unless the disease is refractory to all available therapy.

Topical therapy. Topical therapies are described in greater detail in the childhood isolated skin involvement section and include the following:

Topical therapy. Topical therapies are described in greater detail in the childhood isolated skin involvement section and include the following:

Topical or intralesional corticosteroid.

Topical therapy. Topical therapies are described in greater detail in the childhood isolated skin involvement section and include the following:

Topical tacrolimus.[62][Level of evidence C2]

Topical therapy. Topical therapies are described in greater detail in the childhood isolated skin involvement section and include the following:

Topical imiquimod.[63,64][Level of evidence C3]

Topical therapy. Topical therapies are described in greater detail in the childhood isolated skin involvement section and include the following:

Psoralen and long-wave ultraviolet A radiation (PUVA) and UVB. Therapies such as PUVA/UVB may be more useful in adults because long-term toxicity may be reduced.[62,65][Level of evidence C3]

Systemic therapy. Systemic therapy for severe skin LCH includes oral methotrexate, hydroxyurea, oral thalidomide, oral interferon-alpha, or combinations of interferon and thalidomide.[66-68][Level of evidence C3] Interferon and thalidomide are also used to treat chronic skin LCH in adults.[69][Level of evidence C3] Recurrences are possible after treatment is stopped but lesions usually respond to re-treatment.Oral isotretinoin has induced remissions in some adult patients with refractory skin LCH.[70][Level of evidence C3]