P035 Subtle skin rash was the only presenting symptom of a premalignant condition; monoclonal B-cell lymphocytosis driven cryoglobulinaemic vasculitis, a case report
Authors: Alkutobi Ziad , Laila Deena , Ansari Ghazal , Nandagudi Anupama 
Published: April 2022

Background/Aims

Intermittent episodes of subtle skin rash at the hands are commonly associated with dermatitis or allergy. We are reporting a case of cryoglobulinaemic vasculitis with a delayed diagnosis of more than two years because of subtle symptoms and normal investigations; highlighting important points to consider during the assessment of similar cases.

Methods

Case description: In June 2018, a 59-year-old man was initially assessed for intermittent episodes of pruritic skin rash usually on cold exposure, for the preceding 2 years. The patient described and showed pictures of non-blanching deep red-bluish plaques with raised irregular margins involving only the hands; usually self-resolving over 2-3 days without specific treatment and there were no defined predisposing factors. There was history of Raynaud’s, loss of appetite and weight loss of half a stone, but otherwise no other CTD/vasculitis symptoms. Clinical examination showed no skin, joints or systemic abnormalities. Investigations revealed positive RF at 30 U/mL, low C3 and C4 at 0.5 and &lt; 0.1 g/L respectively; whereas full blood count, renal function, liver function, urine protein, ESR, CRP, immunoglobulins, s. protein electrophoresis, ANA, ANCA, HIV and hepatitis screen were all negative. Type 1 cryoglobulinaemia was detected at room temperature and cryoprecipitating IgG kappa was 3g/L on polyclonal background. Peripheral blood immune-phenotyping confirms kappa light chain and monoclonal B-cell lymphocytosis of CLL phenotype. Nailfold capillaroscopy revealed coiled capillaries and corkscrew ramification. CT-Chest abdomen pelvis showed widespread generalised lymphadenopathy. Over 3 years post diagnosis; there was no transformation of MBL to CLL clinically or haematologically except for episodes of rash, thus the plan was to continue monitoring for ‘B symptoms’, and to consider rituximab if significant symptoms appear.

Results

Cryoglobulins (immunoglobulins that precipitate in cold) are classified into three types: type I (monoclonal), type II (mixed) and type III (polyclonal). Polyclonal cryoglobulins are associated with autoimmune rheumatic diseases, hepatitis B and C. Mixed essential cryoglobulinaemia presents with purpuric skin lesions showing a leucocytoclastic vasculitis on biopsy. Type I monoclonal IgG, IgM, IgA, or their κ or λ light chains represent 10-15 % of the cases of cryoglobulinaemia. It is characterised by overwhelming association with hematologic neoplasia including MGUS, smouldering multiple myeloma, multiple myeloma, waldenström's macroglobulinemia, and chronic lymphocytic leukemia. Treatment is generally directed towards treating the underlying pre-malignant or malignant disorder.

Conclusion

Cryoglobulinaemia has a wide variety of clinical presentation scenarios, but physicians can play a key role by early recognition. In addition to the routine investigations, patients with suspected vasculitic rash especially to cold exposure need to be assessed for rheumatoid factor, complements and cryoglobulins, and cases confirmed with type I cryoglobulinaemia should be investigated for haematological malignancies. Nailfold Capillaroscopy is crucial in distinguishing secondary Raynaud’s, another feature of cryoglobulinaemia.

Disclosure

Z. Alkutobi: None. D. Laila: None. G. Ansari: None. A. Nandagudi: None.