Original articleCutaneous manifestations of Erdheim-Chester disease (ECD): Clinical, pathological, and molecular features in a monocentric series of 40 patients
Section snippets
Study design and setting
We performed a single-center retrospective observational study that included patients with biopsy-proven ECD who were referred to dermatologists at the Internal Medicine Department of Pitié-Salpêtrière Hospital in Paris, France, during a 31-year period (1982 to December 2013).
Eligibility criteria
We reviewed records of all patients followed up in our center for ECD. ECD was diagnosed according to published criteria4: (1) a tissue biopsy specimen showing the typical pathological signs of ECD (infiltration with foamy
Characteristics of ECD
The clinical features of patients are summarized in Table I. Among 123 patients with ECD, 40 (32%) had cutaneous manifestations of histiocytosis. They were 27 men and 13 women (sex ratio [SR] 2.1), and median age at diagnosis was 51 (range 23-80) years. Among these 40 patients, 31 had isolated cutaneous ECD lesions and 9 had mixed histiocytosis involving both ECD and LCH manifestations. Patients with mixed histiocytosis had cutaneous manifestations from at least 1 type of histiocytosis (Table II
Discussion
Cutaneous involvement has been reported in around 20% of ECD cases in the literature14 and the clinical description has been mostly limited to XLL.10 To our knowledge, this study is the first to describe various cutaneous manifestations associated with ECD. Consistent with previous reports, we found that XLL were the most frequent cutaneous manifestations that occurred in 25% of ECD cases. These lesions presented as multiple yellowish patches most commonly located on the inner canthus of the
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Cited by (0)
Drs Chasset and Barete contributed equally to this work as co-first authors.
Funding sources: None.
Disclosure: Dr Emile participated on an advisory board for Roche. Drs Chasset, Barete, Charlotte, Cohen-Aubart, Arnaud, Le Pelletier, Francès, Amoura, and Haroche have no conflicts of interest to declare.