Previously, GH were believed to be found only in patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, multiple myeloma, and skin changes and sclerotic bone changes) syndrome. Increased serum vascular endothelial growth factor (VEGF) levels are found in almost all patients with POEMS syndrome, and this is thought to play a role in the pathogenesis of GH. However, cases of patients with GH but without POEMS syndrome have been reported. This remains somewhat controversial as it is not known whether GH can occur in otherwise healthy individuals, if the cases in the literature describe patients who later displayed more of the findings of POEMS syndrome, or if the vascular lesions described were actually papillary hemangiomas, which can be challenging to distinguish from GH.
There also have been a small number of reports describing GH in the setting of Crow-Fukase syndrome and TAFRO (thrombocytopenia, anasarca, fever, renal dysfunction, and organomegaly) syndrome.
D18.01 – Hemangioma of skin and subcutaneous tissue
403976007 – Glomeruloid hemangioma
Differential Diagnosis & Pitfalls
- Angiosarcoma – Larger, more infiltrative, and frequently appearing on the face and scalp.
- Angiolymphoid hyperplasia
- Kaposi sarcoma in HIV-infected and HIV-uninfected individuals.
- Lobular capillary hemangiomas (pyogenic granulomas)
- Acquired tufted angioma – Usually flat.
- Cutaneous reactive angiomatosis (angioendotheliomatosis, acroangiodermatitis)
- Malignant endovascular papillary angioendothelioma (Dabska tumor)
- Glomus tumors
- Papillary hemangioma – Usually on the head and neck of otherwise healthy adults. In challenging cases, immunostaining with collagen type IV can be helpful.