Hemangioma glomeruloides D18.01

Chan JKC, 1990

Mostly multiple, acquired, lobar hemangioma, which is considered a marker for POEMS syndrome.

Mostly multiple, 2-5 mm, red, soft, asymptomatic papules (hardly distinguishable from tardive/senile angiomas) with a smooth surface. A common occurrence of glomeruloid and tardive hemangiomas has also been described.

• Capillary type: with large bright bubble-shaped cores (CD31+, CD34+, UEAI+, CD68-).
• Sinusoidal type: endothelia with small chromatin-tight nuclei (CD31+, CD34-, UEAI-, CD68+).

Clinical:

• Tardive (senile) hemangiomas.

Histologic:

• angiomatosis, bacillary; bartonellosis
• intravascular papillary endothelial hyperplasia

Case report v aried after Yuri Tet al. (2008).

A 64-year-old Japanese woman suffering from idiopathic thrombocytopenic purpura was treated with prednisolone. During the course of steroid withdrawal, she developed enlargement of the parotid gland and swelling of the cervical lymph nodes with multiple dome-shaped red papules on the trunk and upper limbs. On admission, the patient was found to have numbness in the lower limbs (polyneuropathy), lymph node swelling, elevated blood glucose levels, Bence Jones protein kappa in the urine (M protein). On the skin, there was hyperpigmentation, hypertrichosis and multiple glomeruloid hemangiomas. The overall symptomatology was consistent with polyneuropathy-organomegaly-endocrinopathy-M-protein skin abnormality syndrome(POEMS). The patient also had peripheral edema, ascites, and pleural effusion.

Histo: The glomeruloid hemangiomas exhibited intravascular capillary growth composed of conglomerates of capillaries resulting in structures resembling renal glomeruli. The cells within the capillary loops were lined by endothelial cells with little cytoplasm (CD31(+)/CD34(+)/CD68(-)/CD105(+)/UEA-1(+), whereas the outer surfaces of the loops were either covered by swollen endothelial cells containing PAS- and immunoglobulin-positive eosinophilic hyaline globules (CD31(+)/CD34(-)/CD68(-/+)/CD105(-)/UEA-1(-)) or cells without globules. These two phenotypically distinct endothelial cells were separated by alpha-smooth muscle actin-positive pericytes. Pericytes and endothelial cells covering the outer surface of the loops were bounded by a basement membrane. Biopsy of the parotid gland and lymph node revealed Sjögren's syndrome and Castleman's disease of the hyaline vascular type, respectively.

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