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.
Generalized eruptive histiocytosis in Adult
Alerts and Notices
Synopsis
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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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Urticaria pigmentosa – Lesions are often pruritic and can develop a wheal and flare when scratched or rubbed (Darier's sign).
- Juvenile xanthogranuloma – Typically occurs within the first year of life. Histology demonstrates foam cells and Touton cells in mature lesions.
- Xanthoma disseminatum – Papules may be grouped and favor intertriginous and flexural areas. Transitory diabetes insipidus and mucosal lesions are present in nearly 50% of cases. Histology demonstrates foam cells, Touton cells, and potentially siderosis in mature lesions.
- Benign cephalic histiocytosis – Affects children, with lesions developing first on the head with potential to evolve and involve the neck, upper trunk, and other parts of the body.
- Multicentric reticulocytosis – Papules frequently occur on the acral regions and are associated with arthropathy.
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Last Reviewed:08/27/2017
Last Updated:08/27/2017
Last Updated:08/27/2017
Generalized eruptive histiocytosis in Adult