Generalized Eruptive Histiocytosis Associated With FIP1L1-PDGFRA-Positive Chronic Eosinophilic Leukemia.

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.

• 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)

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

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)