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Cutaneous Vasculitides: Clinico-Pathological Correlation Original CCutaneousutaneous vasculitides:vasculitides: Clinico-pathologicalClinico-pathological Article ccorrelationorrelation SSuruchiuruchi Gupta,Gupta, SSanjeevanjeev HHanda,anda, AmrinderAmrinder J.J. Kanwar,Kanwar, BBishanishan DDassass RadotraRadotra 1, RRanjanaanjana WW.. MMinzinz 2 Departments of Dermatology, ABSTRACT Venereology, Leprology, 1Histopathology and Background: Cutaneous vasculitis presents as a mosaic of clinical and histological 2Immunopathology, Postgraduate Institute of Þ ndings. Its pathogenic mechanisms and clinical manifestations are varied. Aims: To Medical Education and study the epidemiological spectrum of cutaneous vasculitides as seen in a dermatologic Research, Chandigarh, India clinic and to determine the clinico-pathological correlation. Methods: A cohort study was conducted on 50 consecutive patients clinically diagnosed as cutaneous vasculitis in the AAddressddress forfor ccorrespondence:orrespondence: dermatology outdoor; irrespective of age, sex and duration of the disease. Based on the Prof. Sanjeev Handa, clinical presentation, vasculitis was classiÞ ed according to modiÞ ed Gilliam’s classiÞ cation. Departments of Dermatology, All patients were subjected to a baseline workup consisting of complete hemogram, Venereology and Leprology, Postgraduate Institute of serum-creatinine levels, serum-urea, liver function tests, chest X-ray, urine (routine and Medical Education and microscopic) examination besides antistreptolysin O titer, Mantoux test, cryoglobulin levels, Research, Chandigarh, India antineutrophilic cytoplasmic antibodies and hepatitis B and C. Histopathological examination E-mail: was done in all patients while immunoß uorescence was done in 23 patients. Results: Out [email protected] of a total of 50 patients diagnosed clinically as cutaneous vasculitis, 41 were classiÞ ed as leukocytoclastic vasculitis, 2 as Heinoch−Schonlein purpura, 2 as urticarial vasculitis and one each as nodular vasculitis, polyarteritis nodosa and pityriasis lichenoid et varioliforme acuta. Approximately 50% of the patients had a signiÞ cant drug history, 10% were attributed to infection and 10% had positive collagen workup without any overt manifestations, while 2% each had Wegener granulomatosis and cryoglobulinemia. No cause was found in 26% cases. Histopathology showed features of vasculitis in 42 patients. Only 23 patients could undergo direct immunoß uorescence (DIF), out of which 17 (73.9%) were positive for vasculitis. Conclusions: Leukocytoclastic vasculitis was the commonest type of vaculitis presenting to the dermatology outpatient department. The workup of patients with cutaneous vasculitis includes detailed history, clinical examination and investigations to rule out multisystem involvement followed by skin biopsy and DIF at appropriate stage of evolution of lesions. Follow up of these patients is very essential as cutaneous manifestations may be the forme fruste of serious systemic involvement. DOI: 10.4103/0378-6323.53130 PMID: ***** Key words: Cutaneous vasculitis, Skin biopsy, Direct immunoß ourescence IINTRODUCTIONNTRODUCTION the dermatologist who must diagnose and treat this challenging condition. The classification of vasculitis is Vasculitis is an inflammation of the blood vessel wall controversial with no generally accepted classification and it can have a wide range of clinical manifestations. system. The classification systems of the American Most reviews on vasculitides have been written College of Rheumatology (ACR) and of the Chapel from rheumatologist’s perspective.[1,2] The skin is Hill Consensus Conference (CHCC) have gained wide commonly involved in systemic vasculitic disorders, acceptance. Yet, they need to be updated, especially and cutaneous vasculitic lesions offer a window to with regard to leukocytoclastic vasculitis (LCV), the diagnosis and a ready source of accessible tissue for most common vasculitis of the skin.[3] Despite the best histopathologic examination. Many times the initial efforts at diagnosis, no identifiable cause is found in presentation of vasculitis is on the skin and it is nearly one-third patients of vasculitis. How to cite this article: Gupta S, Handa S, Kanwar AJ, Radotra BS, Minz RW. Cutaneous vasculitides: Clinico-pathological correlation. Indian J Dermatol Venereol Leprol 2009;75:356-62. Received: October, 2008. Accepted: February, 2009. Source of Support: Nil. Conß ict of Interest: None declared. 356 Indian J Dermatol Venereol Leprol | July-August 2009 | Vol 75 | Issue 4 Gupta, et al. Cutaneous vasculitides Cutaneous vasculitis presents as a mosaic of clinical Histopathological examination of a lesional skin and histological findings due to varied pathogenic biopsy specimen of the suspected vasculitic lesions mechanisms.[4,5] A definitive diagnosis of vasculitis was performed in all patients. Skin biopsy was taken requires histological confirmation in almost all cases under strict aseptic conditions, using 4 mm disposable because few vasculitic syndromes have pathognomonic skin punch. Sections were formalin fixed, paraffin clinical, radiographic and/or laboratory findings.[6] embedded and stained by hematoxylin and eosin stain However, histopathologic diagnosis alone cannot for light microscopic examination. Although specific stand by itself and must be correlated with clinical, histopathological features were dependent upon physical and laboratory findings. the type and size of blood vessel affected; however, vasculitis was broadly classified[4] as below in three Our study was an attempt to understand the main types: epidemiological spectrum of cutaneous vasculitides as seen in a dermatologic clinic and to determine the Leukocytoclastic vasculitis (LCV): Characterized by clinico-pathological correlation. swelling of endothelial cells, fibrinoid necrosis of vessel wall, neutrophilic infiltration with leukocytoclasis MMETHODSETHODS and extravasation of RBCs. Patients fulfilling two out of four criteria qualified for a diagnosis of vasculitis A cohort study was conducted on 50 consecutive patients clinically diagnosed as cutaneous vasculitis Lymphocytic vasculitis: Characterized by perivascular in dermatology outdoor clinic of our hospital, from lymphocytic cuffing and thickening of blood vessels. September 2004 to March 2006. Granulomatous vasculitis: Characterized by palisading All patients with clinical evidence of cutaneous granulomatous inflammation around blood vessels. vasculitis, irrespective of age, sex and duration of the disease were enrolled in the study. Patients presenting Direct immunofluorescence (DIF) was done in with simultaneous crops of palpable purpura, papules, 23 patients. The immunofluorescence corresponding to plaques, nodules, vesicles, bullae, pustules, ulcers immune complex deposits around dermal blood vessels and other cutaneous findings like urticaria, livedo was recorded arbitrarily as strongly positive (3+), reticularis or edema were included in the study. moderately positive (2+) and weakly positive (1+). Based on the clinical presentation, an attempt was made to classify vasculitis as per updated Gilliam’s Statistical analysis classification.[7] The patients with thrombocytopenia Each laboratory data was dichotomized according to a (<50, 000/mm3) and disorders of coagulation; and predetermined cut-off value and analyzed by unpaired patients on warfarin/heparin and patients with age of t-test. The Chi-square test was used for comparing last appeared lesion more than 48 h were excluded qualitative variables. from the study. RRESULTSESULTS A detailed history and clinical examination were carried out in all patients. History of drug exposure was A total of 50 patients clinically diagnosed as cutaneous considered significant if exposure was within 8 weeks of vasculitis in dermatology outdoor clinic were enrolled appearance of lesions. Baseline investigations - complete in the study. There were 20 male and 30 female patients hemogram, erythrocyte sedimentation rate (ESR), with age range of 5-67 years. The mean age of the serum-urea and serum-creatinine levels, liver function study group was 41.1 years for males and 35.9 years tests, chest X-ray, urine (routine and microscopic) for females. examination, stool for occult blood, antistreptolysin O (ASO) titer, Mantoux test, cryoglobulins, antinuclear Palpable purpura was the commonest cutaneous antibodies (ANA), antineutrophilic cytoplasmic presentation noticed in 43 patients (18 males and antibodies (ANCA), rheumatoid factor (RF) and 25 females). The commonest sites of purpura were markers for hepatitis B and C were done in all patients legs and ankles followed by thighs, buttocks, forearm, and specific investigations in select patients based on abdomen, back and chest [Figure 1]. The other underlying disease etiology. cutaneous lesions seen in 22 patients were in the form Indian J Dermatol Venereol Leprol | July-August 2009 | Vol 75 | Issue 4 357 Gupta, et al. Cutaneous vasculitides of plaques, ulcers, bullae, vesicles, ecthyma, gangrene Twenty five patients gave history of drug intake prior to of toes, urticarial lesions and atypical target lesions the appearance of lesions. These included analgesics [Figure 2]. The time since onset of lesions varied in nine, antibiotics in four, Ayurvedic medicines in from 1 day to 9 months. There was history of similar two, Homeopathic medicines and contrast media cutaneous vasculitic lesions in the past in 9 (18%) during angiography, amlodipine, eltroxin, asthalin, patients. These findings have been summarized
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