138: Cutaneous Necrotizing Venulitis Flashcards
What is the most common subtype of cutaneous vasculitis?
The most common subtype of cutaneous vasculitis is cutaneous small-vessel vasculitis (45%) and IgA vasculitis (30%). Age >65 is associated with shorter survival.
What are the common infectious agents associated with cutaneous necrotizing venulitis?
Common infectious agents include: β-hemolytic Streptococcus, Staphylococcus aureus, Mycobacterium leprae, Neisseria meningitidis, Neisseria gonorrhoeae, Pseudomonas, Haemophilus influenzae, Rickettsia, Candida, and Rocky Mountain spotted fever.
What are the characteristic features of lesions in cutaneous necrotizing venulitis?
The characteristic features of lesions include polymorphous lesions, palpable purpura, erythematous to violaceous papules that do not blanch when pressed, and lesions that persist for 1-4 weeks and can resolve with hyperpigmentation and atrophic scars.
What is the most consistent abnormal laboratory finding in the diagnosis of cutaneous necrotizing venulitis?
The most consistent abnormal laboratory finding is an elevated ESR (Erythrocyte Sedimentation Rate).
What are the management strategies for cutaneous necrotizing venulitis?
Management strategies include: 1. H1 antihistamines 2. NSAIDs 3. Colchicine 4. Dapsone or hydroxychloroquine 5. If no response, consider systemic glucocorticoids, azathioprine, methotrexate, cyclosporine, mycophenolate mofetil, cyclophosphamide, plasmapheresis, intravenous immunoglobulin, infliximab, adalimumab, etanercept, and rituximab 6. For Hepatitis C: interferon-α 7. For Schnitzler syndrome: IL-1 inhibitors (anakinra, canakinumab, rilonacept) 8. For livedoid vasculitis: + stockings.
Which chronic disorders are associated with cutaneous necrotizing venulitis?
Associated chronic disorders include: Systemic Lupus Erythematosus, Sjögren Syndrome, Hypergammaglobulinemic Purpura, Dermatomyositis, Paraneoplastic Vasculitis, Cryoglobulins, Microscopic Polyangiitis, and others like Cystic fibrosis, Inflammatory bowel diseases, and Behçet disease.
What is the likely diagnosis for a 70-year-old patient with palpable purpura and elevated ESR?
The likely diagnosis is Cutaneous Necrotizing Venulitis. Systemic involvements to evaluate include synovia, GI tract, voluntary muscles, peripheral nerves, and kidneys.
What is the underlying mechanism for mixed cryoglobulinemia in a patient with hepatitis C?
The underlying mechanism involves immune complex deposition. The types of cryoglobulins involved are mixed types II and III.
What is the likely cause of hyperpigmentation and cutaneous ulcers in a patient with Sjögren Syndrome?
The likely cause is Cutaneous Necrotizing Venulitis associated with Sjögren Syndrome. Other systemic features might include articular involvement, peripheral neuropathy, Raynaud phenomenon, and renal involvement.
What does vasculitis of the GI tract indicate in a child with dermatomyositis?
This finding indicates systemic involvement of dermatomyositis. Complications to monitor include GI bleeding and perforation.
What antibody is associated with a higher risk of Cutaneous Necrotizing Venulitis in SLE patients?
The anti-Ro antibody is associated with a higher risk of Cutaneous Necrotizing Venulitis in patients with SLE.
What is the diagnosis for a patient with palpable purpura and IgA deposition around blood vessels?
The diagnosis is IgA vasculitis. The dominant subclass of IgA is IgA1.
What are the next-line treatment options for a patient unresponsive to NSAIDs and colchicine?
Next-line treatment options include systemic glucocorticoids, azathioprine, methotrexate, cyclosporine, mycophenolate mofetil, cyclophosphamide, plasmapheresis, intravenous immunoglobulin, infliximab, adalimumab, etanercept, and rituximab.
What specific antiviral therapy should be considered for a patient with hepatitis C and Cutaneous Necrotizing Venulitis?
Interferon-α should be considered for treating hepatitis C in the context of Cutaneous Necrotizing Venulitis.
What targeted therapy is recommended for a patient with Schnitzler Syndrome?
IL-1 inhibitors such as anakinra, canakinumab, or rilonacept are recommended for Schnitzler Syndrome.
What supportive therapy can help manage symptoms in a patient with livedoid vasculopathy?
Supportive therapy includes the use of compression stockings.
What laboratory test is most likely to be positive in a patient with microscopic polyangiitis?
ANCAs (antineutrophil cytoplasmic antibodies) are most likely to be positive in microscopic polyangiitis.
What is the causative organism in a patient with erythema nodosum leprosum and necrotizing vasculitis?
The causative organism is Mycobacterium leprae.
What is the diagnosis for a patient with a biopsy showing neutrophilic infiltrates and nuclear debris?
The diagnosis is Cutaneous Necrotizing Venulitis. The most consistent laboratory finding is elevated ESR.
What is the diagnosis for a patient with a biopsy showing fibrin deposition in venules?
The diagnosis is Cutaneous Necrotizing Venulitis. Systemic features to monitor include involvement of the synovia, GI tract, voluntary muscles, peripheral nerves, and kidneys.
What should be evaluated in a patient with palpable purpura and IgA deposition?
Organs to evaluate include the intestines and kidneys.
What biologic agents can be considered for a patient unresponsive to first-line treatments?
Biologic agents to consider include infliximab, adalimumab, etanercept, and rituximab.
What is the diagnosis for a patient with a biopsy showing endothelial-cell swelling and necrosis?
The diagnosis is Cutaneous Necrotizing Venulitis. This finding suggests endothelial activation and damage.
What does a biopsy showing hypogranulated mast cells and macrophages indicate?
The diagnosis is Cutaneous Necrotizing Venulitis. These findings indicate active inflammation and vessel damage.
What does basement membrane reduplication and thickening suggest in a biopsy?
The diagnosis is Cutaneous Necrotizing Venulitis. This finding suggests chronic vascular injury.
What does a biopsy showing mononuclear cells and extravasated erythrocytes indicate?
The diagnosis is Cutaneous Necrotizing Venulitis. This finding indicates vascular inflammation and hemorrhage.
What is the clinical significance of fibrinoid material deposition in a biopsy?
The diagnosis is Cutaneous Necrotizing Venulitis. This finding is characteristic of necrotizing vasculitis and indicates severe vessel damage.
What does a biopsy showing dermal cellular infiltrates of neutrophils suggest?
The diagnosis is Cutaneous Necrotizing Venulitis. This finding suggests acute inflammation.
What is the diagnosis for a biopsy showing nuclear debris?
The diagnosis is Cutaneous Necrotizing Venulitis. This finding indicates leukocytoclastic vasculitis.
What does perivenular and interstitial deposition of fibrin indicate?
The diagnosis is Cutaneous Necrotizing Venulitis. This finding indicates active vascular inflammation.
What does activation of endothelial nuclei in a biopsy suggest?
The diagnosis is Cutaneous Necrotizing Venulitis. This finding suggests endothelial activation and damage.
What does wrinkling of nuclear membranes indicate in a biopsy?
The diagnosis is Cutaneous Necrotizing Venulitis. This finding indicates endothelial cell injury.
What is the clinical significance of necrosis of blood vessels in a biopsy?
The diagnosis is Cutaneous Necrotizing Venulitis. This finding is a hallmark of necrotizing vasculitis and indicates severe vascular damage.
What does dermal cellular infiltrates of mononuclear cells suggest in a biopsy?
The diagnosis is Cutaneous Necrotizing Venulitis. This finding suggests chronic inflammation.
What is the most common form of cutaneous necrotizing venulitis in children?
IgA Vasculitis (Henoch-Schönlein Purpura) is the most common form of CNV in children (75% of CNV in children, 25% in adults).
What are the key features of Acute Hemorrhagic Edema of Infancy?
Acute Hemorrhagic Edema of Infancy affects children under 2 years old, with symptoms including painful, edematous petechiae and ecchymoses affecting the head and distal extremities, and resolution typically occurs within 1 to 3 weeks without sequelae.
What are the systemic features associated with Urticarial Venulitis?
Urticarial Venulitis features recurrent urticaria and angioedema, with systemic features including fever, malaise, myalgia, and involvement of multiple organ systems.
What are the characteristics of Schnitzler Syndrome?
Schnitzler Syndrome can occur with a monoclonal IgM K component and features episodes of urticarial vasculitis, systemic symptoms like fever and lymphadenopathy, and a risk of evolution into hematologic malignant conditions.
What are the key features of Eosinophilic Vasculitis?
Eosinophilic Vasculitis presents with recurrent, pruritic, and purpuric papular skin lesions, and biopsy findings include infiltrate of eosinophils.
What is the likely diagnosis for a 7-year-old child with palpable purpura and abdominal pain?
The most likely diagnosis is IgA vasculitis (Henoch-Schönlein Purpura). Systemic risks include progressive renal disease.
What is the likely condition for a 1-year-old infant with painful, edematous petechiae?
The likely condition is Acute Hemorrhagic Edema of Infancy. The prognosis is good, with resolution within 1 to 3 weeks without sequelae.
What condition should be suspected in a patient with recurrent urticaria and systemic symptoms?
Urticarial Venulitis should be suspected. Characteristic skin findings include pruritic, burning, or painful erythematous indurated wheals.
What syndrome is indicated by severe systemic manifestations and hypocomplementemia?
This is Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS). The underlying immunological abnormality involves low molecular weight 7s C1q precipitin.
What is the diagnosis for a patient with recurrent urticarial vasculitis episodes and monoclonal IgM?
The diagnosis is Schnitzler Syndrome. The long-term risk includes evolution into hematologic malignant conditions.
What is Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS)?
The underlying immunological abnormality involves low molecular weight 7s C1q precipitin and IgG autoantibody to the collagen-like region of C1q.
What is the diagnosis for a patient with monoclonal IgM κ component experiencing recurrent urticarial vasculitis episodes and systemic symptoms like bone pain and hepatosplenomegaly?
The diagnosis is Schnitzler Syndrome.
The long-term risk includes evolution into hematologic malignant conditions in 15% of cases.
What is the likely diagnosis for a 35-year-old woman with tender, red subcutaneous nodules on her calves, some of which are ulcerated?
The likely diagnosis is Nodular Vasculitis (Erythema Induratum).
Common triggers include Mycobacterium tuberculosis and hepatitis C virus infection.
What is the diagnosis for a patient with a history of hypercoagulable states presenting with recurrent painful ulcers on the lower extremities and persistent livedo reticularis?
The diagnosis is Livedoid Vasculopathy.
The hallmark of healing is the formation of atrophie blanche, which are sclerotic pale areas surrounded by telangiectasias.
What syndrome should be considered for a patient with systemic lupus erythematosus (SLE) who develops ischemic cerebrovascular lesions and livedo racemosa?
Sneddon Syndrome should be considered.
Antibodies that might be detected include antiendothelial cell, antiphospholipid, anti–β2-glycoprotein, and antiprothrombin antibodies.
What is the diagnosis for a patient presenting with recurrent pruritic, purpuric papular skin lesions and urticarial plaques, with a biopsy revealing eosinophilic infiltrates expressing CD40?
The diagnosis is Eosinophilic Vasculitis.
Supporting laboratory findings include depressed complement levels, peripheral eosinophilia, and elevated major basic protein levels.
