[Associated Langerhans cell histiocytosis and Erdheim-Chester disease].

[erdheim-chester disease]

Erdheim-Chester disease (ECD ) is a rare non-Langerhans cell histiocytosis with multiple organ involvement affecting middle -aged adults . A case of ECD associated with Langerhans cell histiocytosis (LCH ) is reported herein .A 75 -year -old woman presented maculopapular skin lesions on her trunk , associated with constrictive pericarditis and pleurisy present for 1 year . The skin biopsy militated in favour of LCH since it revealed a histiocytic infiltrate with a positive CD 1 a marker at immunohistochemistry (IHC ). The association with ECD was diagnosed on the basis of pericarditis , periaortitis , pleurisy , pulmonary involvement and retroperitoneal fibrosis . The patient was treated with interferon-α 2 a with good initial results , but died from septic shock a year and a half later , a few months after discontinuing interferon due to poor tolerability .The clinical , radiographic and histological arguments in favour of ECD clearly differ from those for LCH . However , as already reported , the two illnesses may be associated , thus underlining the possible existence of a link between these two histiocytic proliferations emanating from the same medullary precursor . Two hypotheses have been advanced in an attempt to explain this association : the first involves a stimulus that might lead to independent proliferation of the two cell lines while the second suggests the existence of a transformation pathway from one form of proliferation to the other .Screening for associated ECD should be routinely performed in patients presenting LCH with signs evocative of ECD .

Diseases
Validation

Diseases presenting "skin biopsy" symptom

adrenomyeloneuropathy

cadasil

cutaneous mastocytosis

dentinogenesis imperfecta

dracunculiasis

epidermolysis bullosa simplex

erdheim-chester disease

erythropoietic protoporphyria

fabry disease

harlequin ichthyosis

inclusion body myositis

junctional epidermolysis bullosa

kindler syndrome

lamellar ichthyosis

malignant atrophic papulosis

monosomy 21

oligodontia

primary effusion lymphoma

primary hyperoxaluria type 1

pyruvate dehydrogenase deficiency

sneddon syndrome

waldenström macroglobulinemia

x-linked adrenoleukodystrophy

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