(Figures 3.41C and 3.42A) - Entity with clinical presenta tion resembling urticaria but with histopathological findings of LCV; may be associated with autoimmune connective tissue disease (i.e. SLE), infection (HBV, HCV, EBV), serum sickness, malignancy or medication (KI, NSAIDs, fluoxetine)
- Majority normocomplementemic with benign course (average duration 3 years); approximately 25% with hypocomplementemic UV and increased likelihood of systemic involvement
- Presents with urticarial papules and plaques lasting >24 h with associated burning or pain, ± residual hemorrhage; ± fever, malaise, intermittent arthralgias, pulmonary, and GI symptoms (hypocomplementemic)
- Labs (hypocomplementic form): ↓ complement levels, anti-C1q antibody
- Histology: leukocytoclastic vasculitis, but typically subtle
- Treatment: antihistamines, NSAIDs, colchicine, dapsone, antimalarial, oral corticosteroid
| | | | | | Schnitzler’s syndrome: urticarial vasculitis + monoclonal IgM gammopathy + at least 2 of the following: fever, arthralgias, HSM, ↑ ESR, ↑ WBC, bone abnormality, bone pain) | | | | | |
|
| | Figure 3.41
A: CSVV
(Courtesy of Dr. Paul Getz)
B: CSVV
(Courtesy of Dr. Paul Getz)
C: Urticarial vasculitis
(Courtesy of Dr. Iris K.
Aronson) |
| | Figure 3.42
A: Urticarial vasculitis
(Courtesy of Dr. Iris K.
Aronson)
B: EED*
C: Granuloma faciale*
* Reprint from Burgdorf WH,
Plewig G, Landthaler M,
Wolff HH, eds. Braun-Falco’s
Dermatology. 3rd ed.
Heidelberg: Springer; 2009 |
|
