Polyneuropathy: A Rare and Challenging Presentation of Essential Mixed Cryoglobulinemia

A 49-year-old male presented with acute chronic sensory motor bilateral lower extremity polyneuropathy. Electromyography showed bilateral acute sensory motor axonal polyneuropathy. Lumbar spine magnetic resonance imaging showed diffuse bone marrow replacement and bilateral ankylosing spondylitis. Laboratory workup revealed elevated inflammatory markers and low G6PD (glucose-6-phosphate dehydrogenase) level. Due to elevated acute phase reactants, inflammatory polyneuropathy was suspected; patient was treated accordingly with resolution of neuropathy. Three months later, he relapsed and presented with disabling polyneuropathy and renal impairment, which prompted renal biopsy. Renal histopathology revealed the, otherwise mysterious, etiology, essential mixed cryoglobulinemia. Essential mixed cryoglobulinemia was not considered initially due to the absence of classic systemic manifestations of autoimmune disorders.

An unusual case of cryoglobulinemic purpura in elderly patient

Cryoglobulinemia is associated with infections, in particular chronic hepatitis C, autoimmune diseases, and lymphoproliferative disorders. Its frequency seems to be higher than is commonly considered, even in elderly patients.We describe the case of an elderly patient with diabetes, nephroangiosclerosis and purpura who presented marked and persistent increase in rheumatoid factor, hypocomplementemia and cryoglobulinemia with nephrotic disagreement unrelated to HCV. A thirteen‑month follow‑up showed neither immunorheumatologic nor neoplastic disorders. In literature, associations with hepatic pseudocyst is not described and a lack of association with HCV is very rare. Therefore, the hypothesis of “essential” mixed cryoglobulinemia (EMC) associated with nephrotic syndrome was formulated. Renal disease associated with EMC (unrelated to HCV) is characterized by the high prevalence of primary Sjögren syndrome and overt B‑cell non‑Hodgkin’s lymphoma for which repetitive clinical evaluation is necessary.

Leukocytoclastic Vasculitis in a Patient with Type 1 Cryoglobulinemia

Cutaneous manifestations of type 1 cryoglobulinemia are usually related to vascular occlusion by noninflammatory thrombosis; rarely is leukocytoclastic vasculitis seen in type 1 cryoglobulinemia. We report the case of a 64-year-old male who presented with isolated cutaneous leukocytoclastic vasculitis that was initially attributed to essential mixed cryoglobulinemia after thorough diagnostic evaluation. A lack of adequate clinical response to therapy prompted further investigation, including cryoprecipitate electrophoresis and immunofixation, which revealed an IgM kappa monoclonal gammopathy consistent with type 1 cryoglobulinemia. A renewed search for an underlying malignancy led to the discovery of early Waldenstrom's macroglobulinemia. Although leukocytoclastic vasculitis is more characteristic of mixed cryoglobulinemia, it can be a presenting manifestation of type 1 cryoglobulinemia.