Essential Type II Mixed Cryoglobulinemia Causing Pyoderma Gangrenosum–Like Ulcers

Abstract

A 47-year-old, otherwise healthy woman presented with multiple deep ulcers located primarily on her lower extremities that presented 6 days ago as blisters. A biopsy revealed livedoid vasculopathy, without any evidence of vasculitis. Extensive laboratory workup was positive only for type II cryoglobulins. Type II cryoglobulinemia is caused by monoclonal rheumatoid factors. Cutaneous manifestations are nearly always present in cryoglobulinemia. Lesions have a predilection for dependent areas and include erythematous macules and purpuric papules (90-95%), as well as ulcerations (10-25%). Livedo reticularis, Raynaud phenomenon, and nailfold capillary abnormalities are also noted. Presentation with deep ulcers may lead to a misdiagnosis of pyoderma gangrenosum.

Eradication of hepatitis C virus could improve immunological status and pyoderma gangrenosum‐like lesions

“…IT HAS BEEN reported that various kinds of extrahepatic disease could be induced by persistent infection of HCV . Among the extrahepatic diseases, cryoglobulin and autoimmune‐related diseases including pyoderma gangrenosum were most frequently detected in CHC patients . The relationship between cryoglobulin and Th17 cells has been studied in our group (unpubl.…”

“…15,16 Among the extrahepatic diseases, cryoglobulin and autoimmune-related diseases including pyoderma gangrenosum were most frequently detected in CHC patients. 12,17,18 The relationship between cryoglobulin and Th17 cells has been studied in our group (unpubl. data, Kondo et al).…”

Painful ulcerations: the sole clinical sign of mixed cryoglobulinaemia secondary to marginal zone lymphoma

“…While ulcerations with mixed cryoglobulinaemia has been reported in the literature, most cases also presented with extracutaneous clinical manifestations, multisystem involvement or HCV infection. Of 44 results identified from a PubMed search of case reports, combining the keywords ‘mixed cryoglobulinaemia’ and ‘ulcers’ through the Boolean Operator AND, only four remained after excluding reports that were non-English, were type 1 cryoglobulinaemia, had extracutaneous or multisystem involvement on diagnosis or known HCV 6–9. A case with chronic perianal skin ulceration as the presenting manifestation later developed peripheral neuropathies in his hands and feet 6.…”

Extrahepatic Manifestations of Hepatitis C Virus Infection: Mixed Cryoglobulinemia and Beyond

“…Palpable purpura is the main dermatologic finding, usually seen in the lower extremities where venous stasis favors the precipitation of cryoglobulins. Cutaneous findings include erythematous to purpuric macules, papules and urticarial plaques, livedo reticularis, acral necrotic infarction, hemorrhagic erosions, painful distal ulcers, extensive post-inflammatory hyperpigmentation, and pyoderma gangrenosum-like ulcers [10]. Biopsy of these lesions will show a nonspecific leukocytoclastic vasculitis involving small vessels.…”