Life-threatening 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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Cited by (5)
Discordance between data acquired by history and findings of physical examination: A phenomenal paradox
2008, European Journal of Internal MedicineCitation Excerpt :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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