Absence of human herpesvirus-8 in glomeruloid haemangiomas associated with POEMS syndrome and Castleman's disease.

Abstract

Sir, POEMS syndrome is considered to be a form of plasma cell dyscrasia presenting as osteosclerotic myeloma or multicentric angiofollicular lymph node hyperplasia (= Castleman's disease) and evokes a large variety of symptoms including polyneuropathy, organomegaly (lymphadenopathy, splenomegaly or hepatomegaly), endocrinopathy (frequently as hypothyroidism or hypogonadism), monoclonal gammopathy and protean skin changes. Synonyms are Crow–Fukase syndrome, Takatsuki syndrome and PEP syndrome (polyneuropathy, endocrinopathy, plasma cell dyscrasia).1 Skin signs in POEMS syndrome are usually nonspecific and include generalized hyperpigmentation, hypertrichosis and diffuse sclerodermiform skin thickening.2 Glomeruloid haemangiomas, however, are regarded to be highly specific for POEMS syndrome. To date, no case of glomeruloid haemangioma has been reported outside POEMS syndrome.3 A 36‐year‐old man presented with multiple dome‐shaped, firm purple‐red angiomas measuring up to 2 cm in diameter on the upper back and abdomen. They had appeared suddenly 1 year previously within a 2‐month period and had remained unchanged and asymptomatic thereafter (Fig. 1A). The patient was in poor general condition. He had lost 10 kg of weight over the past 2 years, and he had a disabling sensorimotor polyneuropathy of the lower extremities, hepatosplenomegaly, ascites, pleural effusions, generalized lymphadenopathy and multiple endocrine dysfunctions (primary hypothyroidism, hypogonadotrophic hypogonadism with sexual impotence, hyperparathyroidism, vitamin D deficiency and pathological oral glucose tolerance). Skin biopsy of a haemangioma revealed endothelial cell proliferation with ectatic vascular spaces lined by a single layer of flat endothelial cells, filled with red blood cells and interspersed stromal cells resulting in structures reminiscent of renal glomeruli (Fig. 1B). In immunohistochemistry, two distinct endothelial cell populations were observed: one was predominantly CD31‐positive and formed sinusoidal to slit‐like vascular channels, and the other type was CD34‐positive, exhibited a plumper morphology, and outlined small round to oval vessels within the glomeruloid haemangioma.4 Macrophage‐like KP1 (CD68)‐positive cells were interspersed in the stroma.