Elsevier

Clinics in Dermatology

Volume 23, Issue 3, May–June 2005, Pages 227-237
Clinics in Dermatology

Life-threatening purpura and vasculitis

https://doi.org/10.1016/j.clindermatol.2004.06.002Get rights and content

Abstract

Palpable purpura, the inflammation of blood vessels is the hallmark of vasculitis. It can be observed in a variety of settings, where vessels can be affected primarily or as a secondary event. Every patient with vasculitis should be considered to have a systemic disease unless proven otherwise. One or more systemic symptoms occur in at least 50% of patients and there is no way to predict systemic involvement. Patients may demonstrate mild systemic involvement like arthralgia and arthritis, fever and malaise or more severe symptoms such as massive proteinuria and raised creatinine leading to chronic renal failure, severe intestinal bleeding or perforation with a fatal outcome. In this article we will review the life-threatening aspects of purpura and vasculitis.

Introduction

Purpura, the discoloration of the skin due to extravasation of erythrocytes, manifested by the failure of blanching, is a physical sign and not a disease entity.

Clinically, purpuras may be divided into 3 main types: nonpalpable purpura, palpable purpura, and capillaritis of unknown cause. Nonpalpable purpura can be the result (a) of platelet disorders, due to defective production, survival, and function, excessive consumption, or platelet antibodies; (b) of coagulation defects (disseminated intravascular coagulation and purpura fulminans); and (c) of vascular endothelium pathology.1 It can be accompanied by gingival or gastrointestinal (GI) bleeding, hematuria, or by internal hemorrhage according to the etiology.2

Obviously, certain diseases unmasking with nonpalpable purpura may lead to death but they are beyond the scope of dermatologic interest.

Section snippets

Palpable purpura and vasculitis

Palpable purpura, which means inflammation of blood vessels, is the hallmark of vasculitis. It can be observed in a wide variety of settings where vessels can be affected primarily or as a secondary event. Depending on the infiltrating cells, vasculitis can be divided histopathologically into leukocytoclastic and lymphomonocytic. The division is not absolute because cellular infiltrates may evolve from neutrophilic to lymphocytic as lesions mature, although in certain diseases the pattern of

Henoch-Schönlein purpura

According to CHCC classification, HSP is a vasculitis affecting small vessels (capillaries, venules, or arterioles) with IgA-dominant immune deposits typically involving the skin, gut, glomeruli, and joints.4 It is most common in children with prevalence as high as 1.5 per 1000, but can also occur at any age.37 Both sexes are affected equally and in 50% of children an upper respiratory tract infection may precede the disease.38 A multitude of drugs and infectious agents may act as triggering

Urticarial vasculitis

Urticarial vasculitis is characterized clinically by persistent, burning, or painful urticarial lesions, involving mostly the trunk and proximal extremities, lasting more than 24 hours and leaving purpura or hyperpigmentation upon resolution.53 It has been associated with mixed cryoglobulinemia, monoclonal IgM gammopathy, drug ingestion, malignant diseases, and connective tissue disorders such as systemic lupus erythematosus or Sjögren's syndrome.54 Two subsets of UV exist: hypocomplementemic

Cryoblobulinemic vasculitis

Cryoblobulinemic vasculitis is a vasculitis associated with cryoglobulins in the serum, with cryoblobulin immune deposits affecting small vessels.4 Skin and glomeruli are predominantly affected. Cryoglobulins are cold precipitated monoclonal or polyclonal immunoglobulins occurring in conjunction with a variety of diseases including plasma cell or lymphoid neoplasms, chronic infections, and inflammatory diseases.63 The entity previously named mixed cryoglobulinemia has been found to be related

Polyarteritis nodosa

Polyarteritis nodosa (PAN) is a necrotizing inflammatory disease of medium or small arteries without glomerulonephritis, pulmonary capillaritis, or disease of other arterioles, capillaries, or venules.4 It is a rare entity with an annual incidence ranging from 2 to 9 cases per million annually,71 affecting equally men and women. It is well documented that PAN is associated with hepatitis B infection, usually within the first 6 months of the disease.72 Polyarteritis nodosa can also be seen in

Antineutrophilic cytoplasmic antibody–associated vasculitides

Antineutrophil cytoplasmic antibodies are believed to play a role in the pathogenesis of Wegener's granulomatosis (WG), CSS, and microscopic polyangitis through the activation of neutrophils.89 When detected by indirect immunofluorescence, 3 types of staining can be observed: a cytoplasmic (c-ANCA), a perinuclear (p-ANCA), or a more diffuse cytoplasmic staining (atypical ANCA).90 The main antigen producing a c-ANCA pattern as shown by direct enzyme-linked immunoassay is PR3, whereas the main

Wegener's granulomatosis

Wegener's granulomatosis is a multisystem necrotizing granulomatous vasculitis affecting small to medium vessels, involving the upper and lower respiratory tract and kidneys.92 The disease can occur at any age, affects equally both sexes, and has an estimated incidence of 5 to 10 per million annually.93

Cutaneous manifestations like palpable purpura, oral ulcers (most common), papulonecrotic lesions, subcutaneous nodules, and ulcers develop in 46% to 66% of the patients and can be the presenting

Microscopic polyangitis

Microscopic polyangitis is a pauci-immune nongranulomatous small and medium vessel vasculitis, which has many similarities to WG, with the difference that upper respiratory tract involvement is uncommon.43., 92.

Palpable purpura is found in 46% of patients at presentation, whereas nodular lesions should alert suspicion for WG or CSS.5 It is the most common cause of pulmonary-renal syndrome and if left untreated has a poor prognosis, mostly due to alveolar hemorrhage (12% of patients) or renal

Churg-Strauss syndrome

Churg-Strauss syndrome is a rare necrotizing vasculitis affecting small to medium vessels and is usually associated with asthma and eosinophilia.112 Its incidence is 3 per million.16 Criteria for patient identification include: asthma, blood eosinophilia, neuropathy, transient pulmonary infiltrates, paranasal sinus involvement, and biopsy specimen showing vasculitis with extravascular eosinophils.113 Usually, CSS unfolds in 3 distinct phases: (a) a prodromal phase, characterized by asthma or

Kawasaki disease

Kawasaki disease (KD) is an arteritis affecting large, medium, and small vessels and is associated with mucocutaneous, lymph node syndrome. Although its cause is unknown, an infectious agent like Staphylococcus aureus, Streptococcus, Candida, and Rickettsia species, retroviruses, and Epstein-Barr virus have been reported as possible triggering agents for the disease.120 A subtle immune immaturity leading to the inability to clear certain pathogens is hypothesized to be responsible for the

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      Dermatological, endocrine, and renal diseases in which there may be discordance between data acquired by medical history and findings of physical examination are also presented in Table 1. For example, the often impressive clinical picture of purpura is frequently thought to be attributable to thrombocytopenia, and so no palpable feeling of the lesions is expected; purpura that is palpable is due to vasculitis [44]. Transient rashes are often the mainstays of diagnosis, so history regarding them should not be easily disregarded; the details of such rash may provide helpful clues to diagnosis, as happens in the cases of erythema marginatum or the evanescent rash of Still's disease [45].

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