Hypersensitivity Vasculitis Workup
- Author: Jeffrey P Callen, MD; Chief Editor: William D James, MD more...
Laboratory Studies
Evaluation of patients with hypersensitivity vasculitis (leukocytoclastic vasculitis) serves 2 purposes: to determine the presence of systemic disease and to identify an associated disorder, which aids in predicting the patient's prognosis. No established routine exists, and testing should be prompted by signs or symptoms.
- A urinalysis should be performed in all patients to assess renal disease.
- Some authors also include a complete blood cell count, an erythrocyte sedimentation rate, and a blood chemistry panel.
- Check the stool for blood in patients with bowel symptoms.
- Obtain serologic studies (eg, antinuclear antibody; antineutrophil cytoplasmic antibody [ANCA], ie, circulating ANCA, perinuclear ANCA, atypical ANCA; rheumatoid factor) for patients without an obvious disease cause. In children and perhaps in some adults, serologic testing for a possible streptococcal infection should be considered (Streptozyme or ASO titer).
- Complement levels, including total hemolytic complement (CH100 or CH50), C3 levels, and C4 levels, may be obtained for patients suspected of having lupus erythematosus or patients who have urticarial vasculitis.
- Include immunofixation electrophoresis (IFE), cryoglobulins, and hepatitis C antibody in tests for paraproteins for patients without otherwise identified disease. Hepatitis B has been associated with vasculitis in the past; however, it appears that the association may have occurred by virtue of co-infection with hepatitis C (previously termed non-A, non-B). The measurement of hepatitis B surface antigen may not be required in all cases. Cryoglobulins are often not obtained properly; a positive rheumatoid factor should suggest the possibility of cryoglobulins.
- Perform HIV testing for patients at high risk for infection and possibly for those with otherwise unidentifiable cause of disease.
- Consider obtaining direct immunofluorescence microscopy for selected patients. The presence of IgA occurs in Henoch-Schönlein purpura.
Imaging Studies
Consider the following imaging studies:
- Chest radiography is part of the routine evaluation.
- Consider assessing the need for visceral angiography for patients with a severe vasculitic syndrome.
- Perform cardiac ultrasonography and blood cultures for patients with fever and/or a heart murmur.
Other Tests
Obtain pulmonary function tests for patients with hypocomplementemic urticarial vasculitis.
Procedures
Perform a skin biopsy of a relatively fresh (preferably < 24 hours of duration) lesion in most, if not all, adult patients. Biopsies are often not performed in children with suspected vasculitis whose clinical presentation is classic. Consider performing a biopsy of muscle or a biopsy of visceral organs in patients with severe vasculitic syndromes; however, most patients with hypersensitivity vasculitis of the skin do not require such tests.
Obtaining a bone marrow sample may be useful if the peripheral smear is abnormal.
Histologic Findings
A skin biopsy sample reveals the presence of vascular and perivascular infiltration of polymorphonuclear leukocytes with formation of nuclear dust (leukocytoclasis), extravasation of erythrocytes, and fibrinoid necrosis of the vessel walls. This process is dynamic; a biopsy sample of a lesion too early or too late in its evolution may not reveal these findings.
The picture of hypersensitivity vasculitis is a pattern that can occur in any vasculitic syndrome but may also occur in nonvasculitic diseases (eg, neutrophilic dermatoses), at the base of a biopsy sample of a leg ulceration, or in some insect bite reactions. Careful clinicopathologic correlation is necessary. Note the image below.
Histopathologic features of leukocytoclastic vasculitis. Mackel SE, Jordon RE. Leukocytoclastic vasculitis. A cutaneous expression of immune complex disease. Arch Dermatol. May 1982;118(5):296-301. [Medline].
Kevil CG, Bullard DC. Roles of leukocyte/endothelial cell adhesion molecules in the pathogenesis of vasculitis. Am J Med. Jun 1999;106(6):677-87. [Medline].
Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, Garcia-Fuentes M. Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients. Medicine (Baltimore). Nov 1998;77(6):403-18. [Medline].
Garcia-Porrua C, Gonzalez-Gay MA. Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch-Schönlein purpura in adults. Semin Arthritis Rheum. Jun 1999;28(6):404-12. [Medline].
Garcia-Porrua C, Gonzalez-Gay MA, Lopez-Lazaro L. Drug associated cutaneous vasculitis in adults in northwestern Spain. J Rheumatol. Sep 1999;26(9):1942-4. [Medline].
Gonzalez-Gay MA, Garcia-Porrua C, Pujol RM. Clinical approach to cutaneous vasculitis. Curr Opin Rheumatol. Jan 2005;17(1):56-61. [Medline].
Gonzalez-Gay MA, Garcia-Porrua C. Systemic vasculitis in adults in northwestern Spain, 1988-1997. Clinical and epidemiologic aspects. Medicine (Baltimore). Sep 1999;78(5):292-308. [Medline].
Sams WM Jr. Hypersensitivity angiitis. J Invest Dermatol. Aug 1989;93(2 Suppl):78S-81S. [Medline].
Zurada JM, Ward KM, Grossman ME. Henoch-Schönlein purpura associated with malignancy in adults. J Am Acad Dermatol. Nov 2006;55(5 Suppl):S65-70. [Medline].
Solans-Laque R, Bosch-Gil JA, Perez-Bocanegra C, Selva-O'Callaghan A, Simeon-Aznar CP, Vilardell-Tarres M. Paraneoplastic vasculitis in patients with solid tumors: report of 15 cases. J Rheumatol. Feb 2008;35(2):294-304. [Medline].
Fain O, Hamidou M, Cacoub P, et al. Vasculitides associated with malignancies: analysis of sixty patients. Arthritis Rheum. Dec 15 2007;57(8):1473-80. [Medline].
Xu LY, Esparza EM, Anadkat MJ, Crone KG, Brasington RD. Cutaneous manifestations of vasculitis. Semin Arthritis Rheum. Apr 2009;38(5):348-60. [Medline].
Piette WW, Stone MS. A cutaneous sign of IgA-associated small dermal vessel leukocytoclastic vasculitis in adults (Henoch-Schönlein purpura). Arch Dermatol. Jan 1989;125(1):53-6. [Medline].
Davis MD, Daoud MS, Kirby B, Gibson LE, Rogers RS 3rd. Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis. J Am Acad Dermatol. Jun 1998;38(6 Pt 1):899-905. [Medline].
Wisnieski JJ, Baer AN, Christensen J, et al. Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine (Baltimore). Jan 1995;74(1):24-41. [Medline].
Fujikawa K, Kawakami A, Hayashi T, et al. Cutaneous vasculitis induced by TNF inhibitors: a report of three cases. Mod Rheumatol. Feb 2010;20(1):86-9. [Medline].
Callen JP. Colchicine is effective in controlling chronic cutaneous leukocytoclastic vasculitis. J Am Acad Dermatol. Aug 1985;13(2 Pt 1):193-200. [Medline].
Sais G, Vidaller A, Jucgla A, Gallardo F, Peyri J. Colchicine in the treatment of cutaneous leukocytoclastic vasculitis. Results of a prospective, randomized controlled trial. Arch Dermatol. Dec 1995;131(12):1399-402. [Medline].
Keogh KA, Ytterberg SR, Fervenza FC, Carlson KA, Schroeder DR, Specks U. Rituximab for refractory Wegener's granulomatosis: report of a prospective, open-label pilot trial. Am J Respir Crit Care Med. Jan 15 2006;173(2):180-7. [Medline].
Chung L, Funke AA, Chakravarty EF, Callen JP, Fiorentino DF. Successful use of rituximab for cutaneous vasculitis. Arch Dermatol. Nov 2006;142(11):1407-10. [Medline].
Harper L. Recent advances to achieve remission induction in antineutrophil cytoplasmic antibody-associated vasculitis. Curr Opin Rheumatol. Jan 2010;22(1):37-42. [Medline].
Lunardi C, Bambara LM, Biasi D, Zagni P, Caramaschi P, Pacor ML. Elimination diet in the treatment of selected patients with hypersensitivity vasculitis. Clin Exp Rheumatol. Mar-Apr 1992;10(2):131-5. [Medline].
Tancrede-Bohin E, Ochonisky S, Vignon-Pennamen MD, Flageul B, Morel P, Rybojad M. Schönlein-Henoch purpura in adult patients. Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases. Arch Dermatol. Apr 1997;133(4):438-42. [Medline].
Callen JP. Cutaneous vasculitis: Relationship to systemic disease and therapy. Curr Probl Dermatol. 1993;5:45-80.
Carlson JA, Cavaliere LF, Grant-Kels JM. Cutaneous vasculitis: diagnosis and management. Clin Dermatol. Sep-Oct 2006;24(5):414-29. [Medline].
Carlson JA, Ng BT, Chen KR. Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. Am J Dermatopathol. Dec 2005;27(6):504-28. [Medline].
Ekenstam Eaf, Callen JP. Cutaneous leukocytoclastic vasculitis. Clinical and laboratory features of 82 patients seen in private practice. Arch Dermatol. Apr 1984;120(4):484-9. [Medline].
Fiorentino DF. Cutaneous vasculitis. J Am Acad Dermatol. Mar 2003;48(3):311-40. [Medline].
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. Nov 20 1997;337(21):1512-23. [Medline].
Lie JT. Nomenclature and classification of vasculitis: plus ça change, plus c'est la même chose. Arthritis Rheum. Feb 1994;37(2):181-6. [Medline].
Lotti T, Ghersetich I, Comacchi C, Jorizzo JL. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. Nov 1998;39(5 Pt 1):667-87; quiz 688-90. [Medline].
Martinez-Taboada VM, Blanco R, Garcia-Fuentes M, Rodriguez-Valverde V. Clinical features and outcome of 95 patients with hypersensitivity vasculitis. Am J Med. Feb 1997;102(2):186-91. [Medline].
Russell JP, Weenig RH. Primary Cutaneous Small Vessel Vasculitis. Curr Treat Options Cardiovasc Med. Apr 2004;6(2):139-149. [Medline].
Sanchez-Guerrero J, Gutierrez-Urena S, Vidaller A, Reyes E, Iglesias A, Alarcon-Segovia D. Vasculitis as a paraneoplastic syndrome. Report of 11 cases and review of the literature. J Rheumatol. Nov 1990;17(11):1458-62. [Medline].

