138: Cutaneous Necrotizing Venulitis

Question 1

What is the most common subtype of cutaneous vasculitis?

Answer 1

The most common subtype of cutaneous vasculitis is cutaneous small-vessel vasculitis (45%) and IgA vasculitis (30%). Age >65 is associated with shorter survival.

Question 2

What are the common infectious agents associated with cutaneous necrotizing venulitis?

Answer 2

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.

Question 3

What are the characteristic features of lesions in cutaneous necrotizing venulitis?

Answer 3

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.

Question 4

What is the most consistent abnormal laboratory finding in the diagnosis of cutaneous necrotizing venulitis?

Answer 4

The most consistent abnormal laboratory finding is an elevated ESR (Erythrocyte Sedimentation Rate).

Question 5

What are the management strategies for cutaneous necrotizing venulitis?

Answer 5

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.

Question 6

Which chronic disorders are associated with cutaneous necrotizing venulitis?

Answer 6

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.

Question 7

What is the likely diagnosis for a 70-year-old patient with palpable purpura and elevated ESR?

Answer 7

The likely diagnosis is Cutaneous Necrotizing Venulitis. Systemic involvements to evaluate include synovia, GI tract, voluntary muscles, peripheral nerves, and kidneys.

Question 8

What is the underlying mechanism for mixed cryoglobulinemia in a patient with hepatitis C?

Answer 8

The underlying mechanism involves immune complex deposition. The types of cryoglobulins involved are mixed types II and III.

Question 9

What is the likely cause of hyperpigmentation and cutaneous ulcers in a patient with Sjögren Syndrome?

Answer 9

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.

Question 10

What does vasculitis of the GI tract indicate in a child with dermatomyositis?

Answer 10

This finding indicates systemic involvement of dermatomyositis. Complications to monitor include GI bleeding and perforation.

Question 11

What antibody is associated with a higher risk of Cutaneous Necrotizing Venulitis in SLE patients?

Answer 11

The anti-Ro antibody is associated with a higher risk of Cutaneous Necrotizing Venulitis in patients with SLE.

Question 12

What is the diagnosis for a patient with palpable purpura and IgA deposition around blood vessels?

Answer 12

The diagnosis is IgA vasculitis. The dominant subclass of IgA is IgA1.

Question 13

What are the next-line treatment options for a patient unresponsive to NSAIDs and colchicine?

Answer 13

Next-line treatment options include systemic glucocorticoids, azathioprine, methotrexate, cyclosporine, mycophenolate mofetil, cyclophosphamide, plasmapheresis, intravenous immunoglobulin, infliximab, adalimumab, etanercept, and rituximab.

Question 14

What specific antiviral therapy should be considered for a patient with hepatitis C and Cutaneous Necrotizing Venulitis?

Answer 14

Interferon-α should be considered for treating hepatitis C in the context of Cutaneous Necrotizing Venulitis.

Question 15

What targeted therapy is recommended for a patient with Schnitzler Syndrome?

Answer 15

IL-1 inhibitors such as anakinra, canakinumab, or rilonacept are recommended for Schnitzler Syndrome.

Question 16

What supportive therapy can help manage symptoms in a patient with livedoid vasculopathy?

Answer 16

Supportive therapy includes the use of compression stockings.

Question 17

What laboratory test is most likely to be positive in a patient with microscopic polyangiitis?

Answer 17

ANCAs (antineutrophil cytoplasmic antibodies) are most likely to be positive in microscopic polyangiitis.

Question 18

What is the causative organism in a patient with erythema nodosum leprosum and necrotizing vasculitis?

Answer 18

The causative organism is Mycobacterium leprae.

Question 19

What is the diagnosis for a patient with a biopsy showing neutrophilic infiltrates and nuclear debris?

Answer 19

The diagnosis is Cutaneous Necrotizing Venulitis. The most consistent laboratory finding is elevated ESR.

Question 20

What is the diagnosis for a patient with a biopsy showing fibrin deposition in venules?

Answer 20

The diagnosis is Cutaneous Necrotizing Venulitis. Systemic features to monitor include involvement of the synovia, GI tract, voluntary muscles, peripheral nerves, and kidneys.

Question 21

What should be evaluated in a patient with palpable purpura and IgA deposition?

Answer 21

Organs to evaluate include the intestines and kidneys.

Question 22

What biologic agents can be considered for a patient unresponsive to first-line treatments?

Answer 22

Biologic agents to consider include infliximab, adalimumab, etanercept, and rituximab.

Question 23

What is the diagnosis for a patient with a biopsy showing endothelial-cell swelling and necrosis?

Answer 23

The diagnosis is Cutaneous Necrotizing Venulitis. This finding suggests endothelial activation and damage.

Question 24

What does a biopsy showing hypogranulated mast cells and macrophages indicate?

Answer 24

The diagnosis is Cutaneous Necrotizing Venulitis. These findings indicate active inflammation and vessel damage.

Question 25

What does basement membrane reduplication and thickening suggest in a biopsy?

Answer 25

The diagnosis is Cutaneous Necrotizing Venulitis. This finding suggests chronic vascular injury.

Question 26

What does a biopsy showing mononuclear cells and extravasated erythrocytes indicate?

Answer 26

The diagnosis is Cutaneous Necrotizing Venulitis. This finding indicates vascular inflammation and hemorrhage.

Question 27

What is the clinical significance of fibrinoid material deposition in a biopsy?

Answer 27

The diagnosis is Cutaneous Necrotizing Venulitis. This finding is characteristic of necrotizing vasculitis and indicates severe vessel damage.

Question 28

What does a biopsy showing dermal cellular infiltrates of neutrophils suggest?

Answer 28

The diagnosis is Cutaneous Necrotizing Venulitis. This finding suggests acute inflammation.

Question 29

What is the diagnosis for a biopsy showing nuclear debris?

Answer 29

The diagnosis is Cutaneous Necrotizing Venulitis. This finding indicates leukocytoclastic vasculitis.

Question 30

What does perivenular and interstitial deposition of fibrin indicate?

Answer 30

The diagnosis is Cutaneous Necrotizing Venulitis. This finding indicates active vascular inflammation.

Question 31

What does activation of endothelial nuclei in a biopsy suggest?

Answer 31

The diagnosis is Cutaneous Necrotizing Venulitis. This finding suggests endothelial activation and damage.

Question 32

What does wrinkling of nuclear membranes indicate in a biopsy?

Answer 32

The diagnosis is Cutaneous Necrotizing Venulitis. This finding indicates endothelial cell injury.

Question 33

What is the clinical significance of necrosis of blood vessels in a biopsy?

Answer 33

The diagnosis is Cutaneous Necrotizing Venulitis. This finding is a hallmark of necrotizing vasculitis and indicates severe vascular damage.

Question 34

What does dermal cellular infiltrates of mononuclear cells suggest in a biopsy?

Answer 34

The diagnosis is Cutaneous Necrotizing Venulitis. This finding suggests chronic inflammation.

Question 35

What is the most common form of cutaneous necrotizing venulitis in children?

Answer 35

IgA Vasculitis (Henoch-Schönlein Purpura) is the most common form of CNV in children (75% of CNV in children, 25% in adults).

Question 36

What are the key features of Acute Hemorrhagic Edema of Infancy?

Answer 36

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.

Question 37

What are the systemic features associated with Urticarial Venulitis?

Answer 37

Urticarial Venulitis features recurrent urticaria and angioedema, with systemic features including fever, malaise, myalgia, and involvement of multiple organ systems.

Question 38

What are the characteristics of Schnitzler Syndrome?

Answer 38

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.

Question 39

What are the key features of Eosinophilic Vasculitis?

Answer 39

Eosinophilic Vasculitis presents with recurrent, pruritic, and purpuric papular skin lesions, and biopsy findings include infiltrate of eosinophils.

Question 40

What is the likely diagnosis for a 7-year-old child with palpable purpura and abdominal pain?

Answer 40

The most likely diagnosis is IgA vasculitis (Henoch-Schönlein Purpura). Systemic risks include progressive renal disease.

Question 41

What is the likely condition for a 1-year-old infant with painful, edematous petechiae?

Answer 41

The likely condition is Acute Hemorrhagic Edema of Infancy. The prognosis is good, with resolution within 1 to 3 weeks without sequelae.

Question 42

What condition should be suspected in a patient with recurrent urticaria and systemic symptoms?

Answer 42

Urticarial Venulitis should be suspected. Characteristic skin findings include pruritic, burning, or painful erythematous indurated wheals.

Question 43

What syndrome is indicated by severe systemic manifestations and hypocomplementemia?

Answer 43

This is Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS). The underlying immunological abnormality involves low molecular weight 7s C1q precipitin.

Question 44

What is the diagnosis for a patient with recurrent urticarial vasculitis episodes and monoclonal IgM?

Answer 44

The diagnosis is Schnitzler Syndrome. The long-term risk includes evolution into hematologic malignant conditions.

Question 45

What is Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS)?

Answer 45

The underlying immunological abnormality involves low molecular weight 7s C1q precipitin and IgG autoantibody to the collagen-like region of C1q.

Question 46

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?

Answer 46

The diagnosis is Schnitzler Syndrome. ## Footnote The long-term risk includes evolution into hematologic malignant conditions in 15% of cases.

Question 47

What is the likely diagnosis for a 35-year-old woman with tender, red subcutaneous nodules on her calves, some of which are ulcerated?

Answer 47

The likely diagnosis is Nodular Vasculitis (Erythema Induratum). ## Footnote Common triggers include Mycobacterium tuberculosis and hepatitis C virus infection.

Question 48

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?

Answer 48

The diagnosis is Livedoid Vasculopathy. ## Footnote The hallmark of healing is the formation of atrophie blanche, which are sclerotic pale areas surrounded by telangiectasias.

Question 49

What syndrome should be considered for a patient with systemic lupus erythematosus (SLE) who develops ischemic cerebrovascular lesions and livedo racemosa?

Answer 49

Sneddon Syndrome should be considered. ## Footnote Antibodies that might be detected include antiendothelial cell, antiphospholipid, anti–β2-glycoprotein, and antiprothrombin antibodies.

Question 50

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?

Answer 50

The diagnosis is Eosinophilic Vasculitis. ## Footnote Supporting laboratory findings include depressed complement levels, peripheral eosinophilia, and elevated major basic protein levels.