Glomeruloid hemangioma - a specific cutaneous marker of POEMS syndrome

In December 1998, a 49-year-old woman was referred by the Department of Neu rology to the Dermatology Clinic for evaluation of multiple, bright red pap ules on her face and trunk of 6 months' duration. Two years prior to the ap pearance of the cutaneous eruption she was, seen for intermittent numbness and burning sensation of both hands of several months' duration and was fou nd to have decreased sensory and motor nerve conduction velocities. Electro myography suggested axongenic demyelinating polyneuropathy. On admission fo r further studies in April 1996, she was noted to have generalized skin hyp erpigmentation and mild splenomegaly. Laboratory studies at that time revea led anemia [hemoglobin, 9.6 g/dL (normal male, 13.5-17.5 g/dL; normal femal e, 12.0-16.0 g/dL)], hypothyroidism [thyroxine, 3.86 ng/dL (normal, 5.0-12. 4 ng/dL); triiodothyronine, 35.4 ng/dL (normal, 62-168 ng/dL); thyroid-stim ulating hormone, 12.3 mIU/L (normal, 0.4-4.5 mIU/L)], and lambda-type immun oglobulin A (IgA) paraprotein in the serum. A bone marrow biopsy showed mar ked plasma cell infiltration. POEMS (polyneuropathy, organomegaly, endocrin opathy, M-protein, skin changes) syndrome was diagnosed at that time and sh e was treated with prednisolone (15-30 mg/day) and melphalan (75 mg/day). I n 1997, she developed pulsatile headache and blurred vision. Increased intr acranial pressure and hydrocephalus were found. Increased intracranial pres sure was relieved by placement of a lumbar peritoneal shunt. In January 199 8, she suffered from recurrent pleural effusion and ascites, which were tre ated by intermittent pleurocentesis and ascites tapping, respectively. Physical examination revealed generalized, diffuse, brown pigmentation of t he skin. Multiple erythematous papules of 4-7 mm were noted on the face and trunk (Fig. 1). The skin on the hands, wrists, feet, and lower legs was ta ut, swollen, shiny, and sclerodermatous, Ascites and pitting edema of the l ower legs, toes, and fingers were noted. A skin biopsy of a chest papule showed dilated dermal vascular spaces conta ining groups of vessels resembling renal glomeruli. The vessels were conges ted and hyaline globules were seen within the cytoplasm of the endothelial cells. Some endothelial cells were vacuolated (Fig. 2a,b). The eosinophilic hyaline globules were weakly positive to periodic acid-Schiff (PAS) stain after diastase digestion, but were negative for immunoglobulin staining (in cluding IgA, IgG, IgM, and kappa, lambda light chain). The endothelial cell s lining the capillary loops showed positive immunostaining for factor VIII -related antigen, CD34, and Ulex europaeus agglutinin (UEA)-1. These findin gs were consistent with glomeruloid hemangioma, as described by Chan et al. in 1990 (Chan JKC, Fletcher CDM, Hicklin GA, et al. Glomeruloid hemangioma . A distinctive cutaneous lesion of multicentric Castleman's disease associ ated with POEMS syndrome. Am J Surg Pathol 1990; 14: 1036-1046), except for negative immunoglobulin staining.