Abstract | IMPORTANCE: Generalized eruptive histiocytosis (GEH) is a rare non-Langerhans cell histiocytosis with a benign, self-healing course. Neoplastic hematologic disorders of the myeloid lineage have been reported in association with GEH in 4 patients. A clonal association between GEH and the underlying leukemia was suspected in these patients but could only be confirmed in one patient. OBSERVATIONS: A male patient in his 20s presented with asymptomatic red to brown macules and papules. A skin biopsy confirmed a diagnosis of GEH. His blood cell count revealed hypereosinophilia. Morphologic and molecular analyses from bone marrow and blood samples revealed FIP1L1-PDGFRA-positive chronic eosinophilic leukemia. The patient was treated with imatinib and achieved complete clinical remission of his leukemia and the GEH. CONCLUSIONS AND RELEVANCE: To our knowledge, this is the first report of a patient with GEH associated with FIP1L1-PDGFRA-positive chronic eosinophilic leukemia. Generalized eruptive histiocytosis in association with a myeloid neoplasm may occur in 2 variants: a reactive condition or a clonal derivative of the underlying leukemia. In this case, both diseases responded well after initiation of treatment with imatinib.
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Authors | Birgit Ziegler, Wiebke K Peitsch, Andreas Reiter, Alexander Marx, Sergij Goerdt, Cyrill Géraud |
Journal | JAMA dermatology
(JAMA Dermatol)
Vol. 151
Issue 7
Pg. 766-9
(Jul 2015)
ISSN: 2168-6084 [Electronic] United States |
PMID | 25923837
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Antineoplastic Agents
- Benzamides
- Oncogene Proteins, Fusion
- Piperazines
- Pyrimidines
- mRNA Cleavage and Polyadenylation Factors
- Imatinib Mesylate
- FIP1L1-PDGFRA fusion protein, human
- Receptor, Platelet-Derived Growth Factor alpha
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Topics |
- Adult
- Antineoplastic Agents
(therapeutic use)
- Benzamides
(therapeutic use)
- Histiocytosis, Non-Langerhans-Cell
(complications, drug therapy)
- Humans
- Hypereosinophilic Syndrome
(complications, drug therapy, genetics)
- Imatinib Mesylate
- Leukemia
- Male
- Oncogene Proteins, Fusion
(genetics)
- Piperazines
(therapeutic use)
- Pyrimidines
(therapeutic use)
- Receptor, Platelet-Derived Growth Factor alpha
(genetics)
- mRNA Cleavage and Polyadenylation Factors
(genetics)
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