Generalized eruptive histiocytosis in Child
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Synopsis
![Generalized eruptive histiocytosis : Widespread distribution](/visualdx/sympticonswm/3669.png?r=1fdb69a28)
Generalized eruptive histiocytosis (GEH; also known as generalized eruptive histiocytoma) is a very rare form of non-Langerhans cell histiocytosis (non-LCH). It is usually a benign, self-limited condition that presents as multiple, asymptomatic, discrete red-brown papules located symmetrically on the face, trunk, and extremities. Successive crops of lesions erupt over years with eventual spontaneous resolution.
In a review of 25 cases of GEH, the mean age of onset for adults was 54 years, with onset ages ranging from 23-84 years. The mean age of onset for children was 4 years, with onset ages ranging from 10 months to 10 years. Males represented 57% of all cases that were reviewed.
The etiology of GEH is unknown. There are reports of GEH transforming into another subtype of non-LCH, such as xanthoma disseminatum, raising the question of whether GEH is actually an early phase of a more mature non-LCH.
GEH has been rarely reported to be associated with malignant hematologic disorders of myeloid lineages. As such, it is important to thoroughly screen GEH patients for associated hematologic malignancies.
In a review of 25 cases of GEH, the mean age of onset for adults was 54 years, with onset ages ranging from 23-84 years. The mean age of onset for children was 4 years, with onset ages ranging from 10 months to 10 years. Males represented 57% of all cases that were reviewed.
The etiology of GEH is unknown. There are reports of GEH transforming into another subtype of non-LCH, such as xanthoma disseminatum, raising the question of whether GEH is actually an early phase of a more mature non-LCH.
GEH has been rarely reported to be associated with malignant hematologic disorders of myeloid lineages. As such, it is important to thoroughly screen GEH patients for associated hematologic malignancies.
Codes
ICD10CM:
D76.3 – Other histiocytosis syndromes
SNOMEDCT:
110980006 – Generalized eruptive histiocytoma
D76.3 – Other histiocytosis syndromes
SNOMEDCT:
110980006 – Generalized eruptive histiocytoma
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Last Reviewed:08/27/2017
Last Updated:08/27/2017
Last Updated:08/27/2017