LCH of Skin
Skin Involvement in Langerhans Cell Histiocytosis (LCH): The skin lesions in LCH may be localized (unisystem LCH) or a component of multisystem disease (approx. 50% of cases). Patients with unifocal disease are usually older children and adults, whereas multisystem LCH develops in infants or during neonatal period.
Cutaneous lesions in multisystem disseminated LCH (Letterer-Siwe Disease and some cases of Hand-Schuller-Christian Disease) consist of widespread erythematous or reddish-brown, scaly patches and papules on scalp, chest, back, inguinal region, and axilla. Perineal and oral mucosal lesions may be present. Less frequently, the skin lesions are petechial, purpuric, vesicular, pustular, ulcerating, or nodular.
Other symptoms of disseminated LCH include fever, weight loss, lymphadenopathy, hepatosplenomegaly, and cytopenias. Imaging studies commonly show lytic bone lesions in skull, ribs, and femur.
The image shows skin lesions on the trunk of a 4 y/o male infant who was diagnosed with multisystem disseminated LCH (Letterer-Siwe Disease) (same case as images 5 and 6). Image courtesy of: Dr. Ian McColl, Global Skin Atlas; used with permission.