Which clinical history findings are characteristic of Langerhans cell histiocytosis (LCH)?

Updated: Jun 12, 2020
  • Author: Christopher R Shea, MD; Chief Editor: William D James, MD  more...
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Answer

The clinical presentation of Langerhans cell histiocytosis (LCH) depends on the extent of dissemination.

Unifocal bony LCH is characterized by the development of solitary osseous lesions at any site. Unifocal bony LCH is least common in the hands and the feet. These lesions are often asymptomatic and are detected incidentally during investigation for unrelated disorders.

Patients with multisystem disease may have a protean history depending on the location of osteolytic lesions and the degree of organ dysfunction. Patients with Hand-Schüller-Christian syndrome (which occurs in 25% of patients with multifocal LCH) often present with recurrent episodes of otitis media and mastoiditis or with polyuria and polydipsia.

Letterer-Siwe disease presents with symptoms suggestive of a systemic infection or malignancy, including a generalized skin eruption, anemia, and hepatosplenomegaly.

The congenital form of LCH manifests as skin lesions at birth or during the early postnatal period. Cutaneous nodules and ulceration have onset early in life. Rarely, patients with purpuric lesions present with a blueberry-muffin appearance. [38] Symptoms of organ involvement may also occur.


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