DermatologyTier 2Disease (DEADMAN)

Cutaneous manifestations of systemic disease - cryoglobulinemia

Description

Cryoglobulinaemia produces characteristic cutaneous findings - palpable purpura, livedo reticularis, and occasionally cutaneous ulceration - from immune complex/cryoglobulin-mediated small vessel vasculitis, most commonly associated with chronic hepatitis C infection

Type II (mixed) cryoglobulinaemic vasculitis is the subtype most strongly associated with hepatitis C infection; also occurs with underlying lymphoproliferative disease and some autoimmune conditions (Type I and III patterns respectively)

Cryoglobulins (immunoglobulins that reversibly precipitate in the cold) deposit in small dermal vessels, activating complement and triggering a leukocytoclastic vasculitis pattern - producing the characteristic purpuric skin findings, typically affecting the lower limbs (gravity-dependent, cooler peripheral areas)

Palpable purpura (typically lower limb, gravity-dependent) is the classic presenting sign; livedo reticularis and, in more severe disease, digital ulceration/necrosis from more significant vascular compromise
Correct cryoglobulin sample handling (keeping the sample warm from collection through to processing) is essential, since cryoglobulins can be missed (false-negative) if the sample is allowed to cool before testing - a critical, frequently overlooked pre-analytical point
Hepatitis C serology should be checked given the strong association

Treat the underlying cause specifically - antiviral therapy for hepatitis C-associated cryoglobulinaemic vasculitis is central to management, alongside immunosuppression (corticosteroids +/- rituximab) for significant organ-threatening manifestations
Cold avoidance may reduce symptom severity in some patients, reflecting the temperature-dependent precipitation mechanism

Hepatitis C infection, lymphoproliferative disease (Type I cryoglobulinaemia), other immune complex vasculitides (see immune complex vasculitis), rheumatoid factor positivity (commonly associated)

Correct cryoglobulin sample handling (kept warm, not allowed to cool during collection/transport) is a specific, easily-missed practical requirement - a standard-handled sample can produce a false-negative result even in genuine cryoglobulinaemic vasculitis, a frequently tested pitfall

6 more sections, plus exam facts

Premium unlocks every note across every specialty, and the full exam fact library behind it.