A 48 year old man with an onset of cutaneous vasculitic lesions at the age of 45 years. This patient came to our attention due to a widespread eruption of purpuric macules and papules, ulcers and eschars scattered on the upper and lower extremities, hands, genitals, face, as well as nasal and oral mucosae.
The vasculitic rash was accompanied by severe malaise, fever, arthralgia, paraesthesia, and microhematuria.
No ANA, ENA, nDNA or ANCA antibodies were found whereas cryoglobulins and high titers of circulating immune complexes were noted.
Steroid treatment was instituted together with plasma-apheresis with good results.