Hypersensitivity Vasculitis Clinical Presentation

  • Author: Jeffrey P Callen, MD; Chief Editor: William D James, MD   more...
 
Updated: May 2, 2012
 

History

Patients with hypersensitivity 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 detectable systemic disease. Vasculitis may occur in conjunction with collagen-vascular disorders, paraproteinemia, ingestants (drugs or foods), infections, or malignancy (rare).[8, 9, 10, 11]

The clinician should elicit information about possible systemic manifestations from patients. Additionally, the clinician should 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 or a collagen-vascular disorder, particularly rheumatoid arthritis, lupus erythematosus, or Sjögren syndrome.

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Physical

Palpable purpura is the most common manifestation of cutaneous vasculitis, but other manifestations may occur, as described below and shown in the images that follow[12] :

Henoch-Schönlein purpura. Henoch-Schönlein purpura. Urticarial vasculitis. These lesions differ from rUrticarial vasculitis. These lesions differ from routine hives by lasting longer (often >24 h), being less pruritic, and often resolving with a bruise or residual pigmentation. Erythema elevatum diutinum, a rare cutaneous vascuErythema elevatum diutinum, a rare cutaneous vasculitis.
  • 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 have been 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 report 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. Livedo reticularis 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 medium and large vessels, but it may complicate intense purpura.
  • Perform a careful physical examination in patients with hypersensitivity vasculitis, including specific observation of cardiopulmonary, musculoskeletal, and gastrointestinal systems.
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Causes

From one third to one half of cutaneous vasculitis cases are idiopathic; the remainder have a variety of causes, as follows:

  • Antibiotics are the most common drugs to cause cutaneous vasculitis, particularly beta-lactams. Nonsteroidal anti-inflammatory drugs and diuretics also frequently cause vasculitis. However, almost all drugs are potential causes.[16]
  • 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. Hepatitis C is a commonly 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 has been implicated in some cases of vasculitis in the past. HIV infection may also be associated with some cases of cutaneous vasculitis.
  • Ascertaining whether a drug (eg, antibiotic) or an infection (eg, upper respiratory tract infection) is responsible for the disease is impossible because the occurrence of vasculitis postdates infection and the drug therapy used to treat the infection.
  • Foods or food additives may cause vasculitis.
  • Collagen-vascular diseases account for 10-15% of cases of vasculitis. In particular, rheumatoid arthritis, Sjögren syndrome, and lupus erythematosus may have an associated hypersensitivity 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 hypersensitivity 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 hypersensitivity vasculitis.
  • Small-vessel hypersensitivity vasculitis may be seen uncommonly in patients with a larger-vessel vasculitis, such as Wegener granulomatosis, polyarteritis nodosa, or Churg-Strauss syndrome.
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Contributor Information and Disclosures
Author

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), 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; Celgene Honoraria Safety Monitoring Committee

Specialty Editor Board

Michelle Pelle, MD  Clinical Assistant Professor, Division of Dermatology, Department of Medicine, University of California, San Diego, School of Medicine

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.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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.

Jeffrey J Miller, MD  Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania 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.

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 Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

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

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Hypersensitivity vasculitis.
Henoch-Schönlein purpura.
Histopathologic features of leukocytoclastic vasculitis.
Urticarial vasculitis. These lesions differ from routine hives by lasting longer (often >24 h), being less pruritic, and often resolving with a bruise or residual pigmentation.
Erythema elevatum diutinum, a rare cutaneous vasculitis.
 
 
 
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