Cryoglobulinemic Vasculitis in a Patient with Trismus Pseudocamptodactyly

Cryoglobulinemia is a condition where immunoglobulins precipitate at temperatures < 37°C causing damage to blood vessels of the skin, joints, peripheral nervous system, and kidneys. Diagnosis is difficult due to various etiologies and clinical manifestations. We present a challenging case of idiopathic type III cryoglobulinemia. A 33-year-old man with a history of trismus pseudocamptodactyly syndrome (TPS), chronic pain, and polysubstance abuse presented with several days of worsening rash on both feet. The patient noted more restriction in his joints, increased from his baseline restriction due to TPS. Physical exam revealed palpable purpura on the dorsum of both feet. Extensive tests were performed (CBC, CMP, PT and INR, Lupus Anticoagulant (LA), Hepatitis B and C serology, HIV antibody, ANA panel, ANCA panel, serum complement) which were unremarkable. A punch biopsy of the right foot demonstrated epidermal necrosis with vascular thrombosis. The patient was discharged due to improvement of his condition. Two weeks later, the patient was readmitted for worsening pain in his feet. The purpuric lesions were more necrotic compared to his last visit. New petechiae appeared on the ventral forearms. Work-up for lupus, infectious etiologies, and malignancy were negative; however, a cryoglobulin test was positive. A follow-up SPEP revealed elevated polyclonal IgM of 622 mg/dL. A punch biopsy of the new lesions revealed leukocytoclastic vasculitis (LCV), which can be caused by mixed cryoglobulinemia (MC), further supporting the diagnosis. This case highlights the difficulty in making the diagnosis of cryoglobulinemia in a patient with a history of drug abuse and TPS. His clinical presentation was confounded by his inability to localize any particular joint involvement due to chronic pain, his history of polysubstance abuse raising concerns for drugseeking behavior, and his negative hepatitis panel making the diagnosis of MC less likely. Despite this, the patient did meet several classification criteria for cryoglobulinemia, which include: the presence of cryoglobulins, elevated monoclonal IgM, constitutional symptoms, and articular and vascular involvement.

Creator

• Ashack, Kurt
• Martin, Anthony
• Aleshaki, Joseph

Language

• English

Stichwort

• Cryoglobulinemia
• Cryoglobulinemic vasculitis
• trismus pseudocamptodactyly
• Trismus

Date created

• 1/1/2016

Resource type

• poster

Source

• mucom_rd_42

Rights statement

• http://rightsstatements.org/vocab/InC/1.0/