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The histiocytic disorders Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD), can both present with multisystem involvement, with the central nervous system and the bone, skin, neuroendocrine, cardiac, respiratory, and gastrointestinal systems potentially affected. The 2 entities occasionally can be difficult to distinguish. Both rarely affect the orbit and the central nervous system, and although there are rare reports of patients with coexistent LCH and ECD, there are no reported cases of the 2 diseases that involve both the orbital and neuroendocrine systems. We report 2 such cases, and review the literature of cases of LCH and ECD occurring in the same patient. The presentation of LCH and ECD in certain patients suggests a possible abnormality in the common CD34 progenitor cell. The coexistence of the 2 disease states should be suspected in patients with atypical presentations of either disorder.

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An 85-year-old man had a bilateral periorbital rash and conjunctivitis leading to lower eyelid ectropion and epiphora within 6 weeks of treatment with erlotinib (Tarceva, Genentech, Inc., San Francisco, CA, and OSI Pharmaceuticals, Melville, NY), a second-line antineoplastic agent. The treatment was discontinued secondary to toxicity, and the periorbital rash completely resolved within 6 weeks of cessation of the drug. To our knowledge, the periorbital rash resulting in bilateral lower eyelid ectropion associated with epiphora is a newly recognized side effect of erlotinib that is completely reversible with discontinuation of the drug. The rash and ectropion should be treated palliatively, and surgical intervention should be avoided unless the patient cannot be removed from treatment.

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