Three different MGCS-associated skin manifestations. (A) Normolipemic xanthoma with marked periorbital involvement in a patient with decreased total complement activity (CH50) and serum C4 level suggesting classical complement pathway activation by immune complexes. (B) Glomeruloid hemangiomata in a patient with POEMS syndrome, likely resulting from the effects of VEGF on angiogenesis. (C-E) Skin lesions of Schnitzler syndrome. (C) Typical pseudourticarial aspect predominantly involving the back. (D-E) Close-up view of lesions of the upper limb in another patient illustrating the efficacy of anakinra and showing initial lesions (D) and normal aspects (E) of the skin obtained after the first injection of the IL-1 antagonist. Skin lesions did not recur after 2 years of follow-up from diagnosis on continuously maintained daily treatment.