Cutaneous vasculitis (CV) is a condition in which inflammation of the vessel wall leads to hemorrhage and ischemia [1]. It can be a primary or idiopathic condition (e.g. polyarteritis nodosa (PAN), Wegener's granulomatosis (WG), Churg-Strauss syndrome (CSS), idiopathic cutaneous leukocytoclastic angiitis) or secondary to drugs (e.g. NSAIDs), infections or systemic diseases (e.g. connective tissue disease, rheumatoid arthritis) and malignancy [2].

Another classification of CV is based on the size of the vessel wall affected - small vessel vasculitis (SVV) and medium vessel vasculitis (MVV) [3]. SVV is associated with urticaria, palpable purpura, target lesions as well as vesiculobullous lesions while lesions in MVV include subcutaneous nodules, livedo reticularis, ulcer, infarct, digital pitted scars and gangrene [1] [2] although overlapping clinical features of SVV and MVV have also been reported [4] in the same patient. The cutaneous lesions are usually found on the lower extremity and their appearance on the upper extremity is an indication of severe and/or systemic disease [5]. Constitutional symptoms like fever, malaise, arthralgias are common to CV of the primary as well as secondary types. Although CV can occur in individuals of all ages, it is commonly seen in adults and has a female preponderance. A majority of the children with CV have Henoch-Schönlein purpura (HSP).

Three patterns of CV presentation and progression have been reported [2]:

1. A self-limiting, single episode following an infection or intake of drug which resolves within six months. This is the most common form of the presentation of CV.
2. Waxing and waning symptoms are seen in HSP and connective tissue associated vasculitis
3. Chronic, progressive vasculitis associated with cryoglobulinemia, hypergammaglobulinemia, and malignancy

CV may be associated with extracutaneous (visceral) vascular inflammation in 20% of the patients and this affects mainly the kidneys [1] [5] [6]. Severe forms of life-threatening CV have been reported in < 7% patients [2] [6].

• Infectious Mononucleosis

  Other conditions that may cause or be associated with small-vessel vasculitis include: infections (hepatitis B and C, infectious mononucleosis, Lyme disease), drugs (diltiazem, cimetidine, procarbazine, fluoxetine, etanercept, methotrexate), hematological [clinicaldermatology.eu]

• Cat Scratch

  Bartonella henselae is the aetiological agent of cat-scratch disease. [ncbi.nlm.nih.gov]

• Vascular Disease

  Reduced blood flow :: Stasis, High fat content reduces blood flow in thigh and buttocks :: medication that constricts blood :: vessels :: Varicose veins :: Peripheral vascular disease :: Cold weather Malignancy Auto immune disorders like SLE, Dermatomyositis [dramitdutta.com]

  Vascular diseases in McKee′s Pathology of the skin with clinical correlations. In: Calonje E, Brenn T, Lazar A, editors. Vol. 1, 4 th ed. China: Elsevier Saunders; 2012. p. 658-710. 2. [ijdpdd.com]

  disease (eg, rheumatoid arthritis, Sjögren's syndrome, SLE) Inflammatory bowel disease Neoplastic (eg, hairy cell leukaemia) Causes of larger-vessel vasculitis (uncommon) Wegener's vasculitis Churg-Strauss syndrome Polyarteritis nodosa Kawasaki disease [patient.info]

• Left Ventricular Dysfunction

  A 61-year-old patient who had been treated with lisinopril in the past without any problems was commenced on ramipril for left ventricular dysfunction. He developed a painful symmetrical purpuric eruption over both feet after three days. [ncbi.nlm.nih.gov]

• Cutaneous Manifestation

  Cutaneous manifestations are frequent during the course of many systemic vasculitis. Lesions are often not specific, the most frequent being palpable purpura. [ncbi.nlm.nih.gov]

• Eczema

  I have eczema, so I thought maybe he was having an allergic reaction to something. His rashes looked somewhat similar to the breakouts I get when something triggers my eczema. [wisegeek.org]

  ITCHING PURPURATOUS (Eczematide-like purpura ) Itching purpura is an eczema like purpura, which begins usually as an eczematous purpuric reaction around the ankles and spreads peripherally. [drmhijazy.com]

• Malar Rash

  The vasculitis group had a higher frequency of malar rash (97.9% vs. 87.4%, p   0.004), photosensitivity (91.4% vs. 81.6%, p   0.030), and Raynaud phenomenon (RP; 27.7% vs. 7.5%, p [ncbi.nlm.nih.gov]

• Dysarthria

  Neurologic features in all five siblings included oral motor deficits, dysarthria, low average IQ (70-80), and essential myoclonus. Four had documented ataxia and/or mild sensory loss with increased patellar but diminished ankle reflexes. [ncbi.nlm.nih.gov]

The diagnosis of CV can be challenging due to its variable clinical presentation. The physician has to not only diagnose the vasculitis but should also identify the underlying condition leading to the vasculitis, order the appropriate laboratory workup, treat the patient and follow-up carefully. After eliciting a detailed history and performing a multi-system physical examination, routine laboratory tests like complete blood count, erythrocyte sedimentation rate, C-reactive protein, renal function tests, and urinalysis are ordered. Specific tests like antinuclear antibody, anti-neutrophil cytoplasmic antibodies (ANCA), perinuclear ANCA (pANCA), rheumatoid factor, and complement levels should be obtained in CV patients despite the absence of clinical features of connective tissue disorders or rheumatoid arthritis or systemic lupus erythematosus. Adult patients with HSP have been reported to have immunoglobulin (Ig)A-type antiphospholipid antibodies [7] and should be tested for it.

If the etiology of CV has been identified then laboratory tests like serum protein electrophoresis (for paraproteinemia), cryoglobulins, hepatitis C antibody and, HIV should be performed. Bone marrow biopsy is indicated in patients with abnormal peripheral blood smears [8]. Although testing for malignancies is not routinely recommended, it should be performed if the clinical presentation in CV is indicative of it [9]. Blood culture and echocardiography may be indicated in patients with endocarditis.

Skin biopsy of the lesion is the mainstay of CV diagnosis as it differentiates between authentic versus pseudo-vasculitis. The timing of the biopsy, multiple biopsies taken from the most reddish, tender and purpuric cutaneous lesions and extending into the subcutaneously help improve identification of the lesion [2]. Almost all biopsies can detect immunoglobulins in the first 48 hours, although complement can still be detected in a majority of cases after 72 hours [10]. Histology demonstrating pan-dermal small-vessel vasculitis as well as subcutaneous muscular-vessel vasculitis is indicative of a connective tissue disorder, ANCA-associated vasculitis, Behçet disease, or malignancy-associated vasculitis. A concomitant biopsy for direct immunofluorescence evaluation is useful to distinguish between IgA-associated vasculitis (Henoch-Schönlein purpura) and IgG-/IgM-associated vasculitis.

• Chlamydia Trachomatis

  Unlike Chlamydia trachomatis and C. psittaci, the association of C. pneumoniae infection with immunological complications, such as reactive arthritis (ReA) or erythema nodosum (EN) has been rarely reported. [ncbi.nlm.nih.gov]

• Mycobacterium Chelonae

  Mycobacterium chelonae is a non-tuberculous, rapidly growing mycobacteria and is widely distributed in the natural environment. [ncbi.nlm.nih.gov]

• Bartonella Henselae

  Bartonella henselae is the aetiological agent of cat-scratch disease. [ncbi.nlm.nih.gov]

• HLA-B27

  A 45-year-old HLA B27 negative male developed an asymmetric and additive arthritis and a cutaneous leukocytoclastic vasculitis with IgM and complement papillary deposition along hypodermic vessel walls about three weeks after the onset of respiratory [ncbi.nlm.nih.gov]

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10. Sais G, Vidaller A, Jucgla A, et al. Prognostic factors in leukocytoclastic vasculitis: a clinicopathologic study of 160 patients. Arch Dermatol 1998; 134 (3): 309-15