eMedicine Specialties > Dermatology > Diseases of the Vessels

Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)

Author: Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Contributor Information and Disclosures

Updated: Mar 4, 2009

Introduction

Background

Hypersensitivity vasculitis (leukocytoclastic vasculitis, or LCV) is a histopathologic term commonly used to denote a small-vessel vasculitis. Many possible causes exist for this condition, but a cause is not found in as many as 50% of patients.

Hypersensitivity vasculitis (leukocytoclastic vasculitis) may be localized to the skin, or it may manifest in other organs. The internal organs most commonly affected are the gastrointestinal tract and the kidneys. Joints are also commonly affected. The prognosis is good when no internal involvement is present. The disorder may be acute or chronic.

Pathophysiology

In the past, circulating immune complexes were believed to cause hypersensitivity vasculitis (leukocytoclastic vasculitis).1 Although immune complexes are involved in the pathogenesis of hypersensitivity vasculitis (leukocytoclastic vasculitis), other autoantibodies cause disease manifestations, such as antineutrophil cytoplasmic antibody (ANCA), other inflammatory mediators, and local factors that involve the endothelial cells and other adhesion molecules.2 The exact mechanisms remain to be elucidated.

Frequency

United States

The incidence of hypersensitivity vasculitis (leukocytoclastic vasculitis) is unknown, but the condition is presumed to be rare.

International

Several studies from Spain have been conducted.3,4,5,6,7 Hypersensitivity vasculitis reportedly has an incidence of 10-30 cases per million people per year. Henoch-Schönlein purpura reportedly has an incidence of 14 cases per million people per year.

Mortality/Morbidity

The prognosis of hypersensitivity vasculitis (leukocytoclastic vasculitis) is generally good, but mortality is possible if the kidneys, the gastrointestinal tract, the lungs, the heart, or the central nervous system is involved. Chronic cutaneous disease may involve ulceration or painful bouts of purpura. Some patients alter their lives because of recurrent purpuric eruptions.

Race

Hypersensitivity vasculitis (leukocytoclastic vasculitis) is reported most often in the white population.

Sex

Hypersensitivity vasculitis (leukocytoclastic vasculitis) affects men and women in approximately equal proportions. Some of the studies from Spain suggest that hypersensitivity vasculitis (leukocytoclastic vasculitis) is slightly more common in men than in women.

Age

Hypersensitivity vasculitis (leukocytoclastic vasculitis) may occur at any age. In children, hypersensitivity vasculitis (leukocytoclastic vasculitis) may be called Henoch-Schönlein purpura. This condition may also occur in adults. Another form of vasculitis that is reported in infancy is acute hemorrhagic edema.

Clinical

History

  • Patients with vasculitis of their skin may report itching, a burning sensation, or pain, or they may have asymptomatic lesions.
  • Vasculitis of the skin may occur in the absence of any systemic disease.
  • Vasculitis may manifest as an eruption only, or it may occur in conjunction with collagen vascular disorders, paraproteinemia, ingestants (drugs or foods), infections, or malignancy (rare).8,9,10,11
  • Elicit information about possible systemic manifestations from patients. Inquire about the presence or the absence of fever, arthralgia, arthritis, myalgia, abdominal pain, diarrhea, hematochezia, cough, hemoptysis, sinusitis, paresthesia, weakness, and hematuria.
  • Obtain information about symptoms of an associated disorder. Determine the patient's history of intravenous drug use, hepatitis, transfusion, and travel, along with symptoms or a history of inflammatory bowel disease and collagen-vascular disorder, particularly rheumatoid arthritis, lupus erythematosus, or Sjögren syndrome.

Physical

Palpable purpura is the most common manifestation of cutaneous vasculitis, but other manifestations may occur.12

  • Palpable purpura is the most frequent presentation of small-vessel vasculitis.
    • Lesions are usually round and 1-3 mm in diameter.
    • Lesions may coalesce to form plaques; they may ulcerate in some instances.
    • Retiform lesions were associated with immunoglobulin A (IgA)–related immune complex disease in one study; however, this result has not been validated in subsequent studies.13
    • Palpable purpura is most frequently observed on the legs, but any surface can be involved. Purpuric lesions are sometimes barely palpable.
  • Urticarial lesions may occur in some patients; rarely, this type of lesion can predate purpuric lesions.
    • Urticarial lesions are of a different character than routine urticaria, tending to be of longer duration (often >24 h) and tending to resolve with some residual pigmentation or ecchymosis. Patients complain of a burning sensation rather than itching.
    • To determine the duration of individual lesions, encircle several lesions and ask the patient to observe them periodically and note when they resolve or when they change shape and when a lesion is outside the encircled area.
  • Patients with hypocomplementemic urticarial vasculitis may develop chronic obstructive pulmonary disease; carefully examine the heart and the lungs.14,15
  • Livedo reticularis is a rare manifestation of small-vessel vasculitis. It is more frequent in patients with occlusive or inflammatory disease of medium-sized vessels.
  • Nodular lesions may occur in some patients with small-vessel vasculitis.
  • Ulceration is more common in vasculitis that affects larger vessels, but it may complicate intense purpura.
  • Perform a careful physical examination in patients with vasculitis, including specific observation of cardiopulmonary, musculoskeletal, and gastrointestinal systems.

Causes

  • Between one third and one half of cutaneous vasculitis cases are idiopathic; the remainder have a variety of causes.
  • Antibiotics are the most common drugs that can cause cutaneous vasculitis, particularly beta-lactams. Nonsteroidal anti-inflammatory drugs and diuretics also frequently cause vasculitis. However, almost all drugs are potential causes.
  • Various infections may be associated with vasculitis. Upper respiratory tract infections (particularly beta-hemolytic streptococcal infection) and viral hepatitis, particularly hepatitis C, are most often implicated. HIV infection may also be associated with some cases of cutaneous vasculitis. Ascertaining whether a drug (eg, antibiotic) or an infection (eg, upper respiratory infection) is responsible for the disease is impossible because the occurrence of vasculitis postdates infection and the drug used to treat the infection.
  • Foods or food additives may cause vasculitis.
  • Hepatitis C is a regularly recognized cause of vasculitis, probably through the presence of cryoglobulins. However, of 1614 patients with hepatitis C, vasculitis occurred in only 12 patients (9 with cryoglobulinemia, 3 without). Interestingly, cryoglobulins were present in roughly 40% of those tested; many patients with cryoglobulins (98%) did not have vasculitis despite an abnormal circulating paraprotein. Hepatitis B was implicated in some cases of vasculitis in the past.
  • Collagen vascular diseases account for 10-15% of cases of vasculitis.
    • In particular, rheumatoid arthritis, Sjögren syndrome, and lupus erythematosus may have an associated vasculitis.
    • The presence of vasculitis often denotes active disease and possibly a poorer prognosis.
  • Inflammatory bowel disease, ulcerative colitis, or Crohn colitis may be associated with cutaneous vasculitis.
  • Malignancy accounts for less than 1% of cases of cutaneous vasculitis.
    • Perhaps lymphoproliferative diseases are more common (particularly hairy cell leukemia); however, any type of tumor at any site may possibly be related to cutaneous vasculitis.
    • Effective tumor therapy in some patients has led to resolution of the vasculitis.
  • Small-vessel cutaneous vasculitis may be seen uncommonly in patients with a larger vessel vasculitis, such as Wegener granulomatosis, polyarteritis nodosa, or Churg-Strauss syndrome.

More on Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)

Overview: Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)
Differential Diagnoses & Workup: Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)
Treatment & Medication: Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)
Follow-up: Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)
Multimedia: Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)
References
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References

  1. Mackel SE, Jordon RE. Leukocytoclastic vasculitis. A cutaneous expression of immune complex disease. Arch Dermatol. May 1982;118(5):296-301. [Medline].

  2. 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].

  3. 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].

  4. 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].

  5. 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].

  6. Gonzalez-Gay MA, Garcia-Porrua C, Pujol RM. Clinical approach to cutaneous vasculitis. Curr Opin Rheumatol. Jan 2005;17(1):56-61. [Medline].

  7. 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].

  8. Sams WM Jr. Hypersensitivity angiitis. J Invest Dermatol. Aug 1989;93(2 Suppl):78S-81S. [Medline].

  9. Zurada JM, Ward KM, Grossman ME. Henoch-Schonlein purpura associated with malignancy in adults. J Am Acad Dermatol. Nov 2006;55(5 Suppl):S65-70. [Medline].

  10. 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].

  11. 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].

  12. Xu LY, Esparza EM, Anadkat MJ, Crone KG, Brasington RD. Cutaneous Manifestations of Vasculitis: A Visual Guide. Semin Arthritis Rheum. Mar 18 2008;[Medline].

  13. 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].

  14. 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].

  15. 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].

  16. Callen JP. Colchicine is effective in controlling chronic cutaneous leukocytoclastic vasculitis. J Am Acad Dermatol. Aug 1985;13(2 Pt 1):193-200. [Medline].

  17. 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].

  18. 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].

  19. 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].

  20. 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].

  21. Tancrede-Bohin E, Ochonisky S, Vignon-Pennamen MD, Flageul B, Morel P, Rybojad M. Schoenlein-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].

  22. Callen JP. Cutaneous vasculitis: Relationship to systemic disease and therapy. Curr Probl Dermatol. 1993;5:45-80.

  23. Carlson JA, Cavaliere LF, Grant-Kels JM. Cutaneous vasculitis: diagnosis and management. Clin Dermatol. Sep-Oct 2006;24(5):414-29. [Medline].

  24. 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].

  25. 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].

  26. Fiorentino DF. Cutaneous vasculitis. J Am Acad Dermatol. Mar 2003;48(3):311-40. [Medline].

  27. Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. Nov 20 1997;337(21):1512-23. [Medline].

  28. Lie JT. Nomenclature and classification of vasculitis: plus ca change, plus c'est la meme chose. Arthritis Rheum. Feb 1994;37(2):181-6. [Medline].

  29. 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].

  30. 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].

  31. Russell JP, Weenig RH. Primary Cutaneous Small Vessel Vasculitis. Curr Treat Options Cardiovasc Med. Apr 2004;6(2):139-149. [Medline].

  32. 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].

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Further Reading

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Keywords

hypersensitivity vasculitis, leukocytoclastic vasculitis, LCV, allergic angiitis, small-vessel vasculitis, small vessel vasculitis, Henoch-Schönlein purpura, acute hemorrhagic edema, hypocomplementemic urticarial vasculitis, livedo reticularis, cutaneous vasculitis, upper respiratory tract infections, beta-hemolytic streptococcal infection, viral hepatitis, HIV infection, hepatitis B, hepatitis C, collagen vascular disease, rheumatoid arthritis, Sjögren syndrome, lupus erythematosus, inflammatory bowel disease, ulcerative colitis, Crohn colitis

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Contributor Information and Disclosures

Author

Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Genetech Honoraria Consulting; Celgene Honoraria Consulting

Medical Editor

Michelle Pelle, MD, Clinical Assistant Professor, Division of Dermatology, Department of Medicine, University of California at San Diego
Michelle Pelle, MD is a member of the following medical societies: American Academy of Dermatology, California Medical Association, Medical Dermatology Society, and Pennsylvania Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey J Miller, MD, Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn State Milton S Hershey Medical Center
Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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