The medical recommendations for adult LCH have been published in Orphanet Journal of Rare Diseases.

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History and physical examination

Author(s): Euro Histio Net Work Group for LCH Guidelines for adults (see introduction page), Created: 2012/07/26, last update: 2013/08/18

Complete history

Patients with LCH are often asymptomatic or show only mild symptoms. The most common symptoms are dyspnea, cough, bone pain, an abnormal growth of soft tissue over the affected bone, rash, pruritus, increased thirst, and lymphadenopathy. Additional signs are fatigue, generalized weakness, weight loss, night sweats, nausea, and fever.

A thorough history should be performed including the questioning about unexplained symptoms in the past such as “idiopathic” eczema, thyroid disease or diabetes insipidus, lung cysts or pneumothorax, or bony lesions, the smoking and family history with special attention to autoimmune disease. A very small number of familial cases are reported [Arico et al 1999].

Complete physical examination

A comprehensive physical examination is necessary. The skin and visible mucous membranes should be inspected. Supplemental neurological and/or psychological investigations are useful in patients presenting with neuromyopathy or cognitive impairment.

References

[Arico et al 1999] Aricò M, Nichols K, Whitlock JA, Arceci R, Haupt R, Mittler U, Kühne T, Lombardi A, Ishii E, Egeler RM, Danesino C: Familial clustering of Langerhans cell histiocytosis. British journal of haematology 1999, 107: 883 [PMID: 10606898]