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


METHODS
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
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.
Department of Dermatology, Venereology, and Allergology, University Medical Center, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany2Center of Excellence in Dermatology, Mannheim, Germany.
Birgit
B
Ziegler
Department of Dermatology, Venereology, and Allergology, University Medical Center, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany2Center of Excellence in Dermatology, Mannheim, Germany.
Wiebke K
WK
Peitsch
Department of Hematology and Oncology, University Medical Center, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany.
Andreas
A
Reiter
Institute of Pathology, University Medical Center, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany.
Alexander
A
Marx
Department of Dermatology, Venereology, and Allergology, University Medical Center, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany2Center of Excellence in Dermatology, Mannheim, Germany.
Sergij
S
Goerdt
Department of Dermatology, Venereology, and Allergology, University Medical Center, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany2Center of Excellence in Dermatology, Mannheim, Germany.
Cyrill
C
Géraud
D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides / Q000627:therapeutic use; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000150:complications* / Q000188:drug therapy / Q000235:genetics*; D000068877:Imatinib Mesylate; D007938:Leukemia; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D010879:Piperazines / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics*; D039221:mRNA Cleavage and Polyadenylation Factors / Q000235:genetics*
D000970:Antineoplastic Agents; D001549:Benzamides; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D011743:Pyrimidines; D039221:mRNA Cleavage and Polyadenylation Factors; D000068877:Imatinib Mesylate; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha