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Clinico-Pathological Analysis of Cutaneous Vasculitis

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Clinico-Pathological Analysis of Cutaneous Vasculitis Journal of Rawalpindi Medical College (JRMC); 2015;19(1):26-28 Original Article Clinico-Pathological Analysis of Cutaneous Vasculitis Mariam Nadeem Rana, Afaf Tahir Baig, Shahid Jamal, Nasser Rashid Dar, Department of Histopathology ,Army Medical College, Rawalpindi, Pakistan Abstract localized, self limited process or may spread to involve multiple organs.1 Vasculitis predominantly involving Background: To assess the clinical presentation small vessels include cutaneous small vessel vasculitis, and histological pattern of cutaneous vasculitis cryoglobulinemic vasculitis, urticarial vasculitis, Methods: In this descriptive study patients with a Henoch-Schonlein purpura and vasculitis associated clinical diagnosis of cutaneous vasculitis were with malignancy. The medium sized vessels are included. Gross examination of the specimens was mainly involved by polyarteritis nodosa, microscopic carried out and recorded. The biopsies were polyangiitis, Wegener’s granulomatosis, Churg- processed in an automatic tissue processor . The Strauss Syndrome and drug induced, neither may be paraffin embedding was done in the embedding found in both small and medium sized vessels. 2 station . The sections were cut (3-5µm) and stained Superficial lesions of cutaneous vasculitis commonly for haematoxylin and eosin . After the slides were manifest as infiltrated erythema or palpable purpura prepared, they were observed under the microscope. whereas lesions affecting deeper regions appear as All cases regardless of age and gender were included nodular erythema, livedoracemosa, deep ulcers or in our study. digital gangrene. A careful clinical history is taken Results: A total of 37 cases (20 males and 17 and close examination is required to rule out any females) with age range of 8-80 years were studied. treatable etiology such as systemic disease, drugs, The majority of the patients presented with ulcer of malignancy or infection. 3 the skin, followed by purpura, nodules, rash and The diagnosis of cutaneous vasculitis cannot be plaques. On histopathology, the patients made solely on the basis of clinical presentation. predominantly had leukocytoclastic vasculitis (27%), When there is a suspicion of vasulitis a biopsy is followed by cryoglobulinemic vasculitis (10.8%), required. This is followed by histopathological panniculitis with vasculitis, and lymphocytic examination which yields information such as the vasculitis. A few cases of Churg Strauss Syndrome, distribution of vasculitis along with the type of vessel Capillaritis and drug induced vasculitis were also (small/medium) affected.3 Further investigations seen. include immunofluorescence to detect immune Conclusion: Cutaneous vasculitis predominantly complexes and serological investigations to detect presents as ulceration and leukocytoclastic vasculitis antineutrophil cytoplasmic antibodies (ANCA). 1 is the main histological type. Biopsy definitely helps Depending on the extent of disease, treatment can the exact characterization of vasculitis for further range from general measures such as leg elevation to management of the cases. use of anti-B-cell antibody rituximab. 4 Key Words: Capillaritis, Granulomatous vasculitis, Leukocytoclastic vasculitis Patients and Methods This study was a descriptive cross sectional survey Introduction carried out in the Department of Histopathology, Vasculitis can present in many ways and can be Army Medical College, Rawalpindi from January 2013 challenging for the treating physician. Always the to April 2015. Skin biopsy specimens in formalin diagnosis cannot be made solely on the basis of clinical accompanied by their requisition forms bearing the presentation and biopsy correlation is very important. clinical details and provisional diagnosis were Vasculitis is defined as inflammation and necrosis of received mainly from Military Hospital and Combined the blood vessel walls which may occur independently Military Hospital as well as some of the civil hospitals or be a consequence of certain primary disorders. A of the surrounding area. Gross examination of the more specific condition, cutaneous vasculitis specimens was carried out and recorded. The biopsies preferably involves small and medium sized vessels of were processed in an automatic tissue processor . The the dermis and subcutaneous tissue. It may be a paraffin embedding was done in the embedding 26 Journal of Rawalpindi Medical College (JRMC); 2015;19(1):26-28 station . The sections were cut (3-5µm) and stained for Figure 1: Photomicrograph showing Leucocytoclastic haematoxylin and eosin . After the slides were vasculitis (H&E x100), Inset showing same in high power prepared, they were observed under the microscope. view (H&E x400) All cases regardless of age and gender were included Table 2: Types of cutaneous vasculitidies on in our study. histopathology (n=37) Histopathological Diagnosis Frequency(n=37) Percentage Results Leukocytoclastic vasculitis 10 27 A total of 37 cases of cutaneous vasculitis were reported during the study period. Only 3 (8.1%) of the Nonspecific vasculitis 7 18.9 patients were less than 20 years of age. The highest Cryoglobulinemic vasculitis 4 10.8 frequency of cutaneous vasculitis was among patients Panniculitis with vasculitis 3 8.1 between the ages of 21 and 40 years (37.8%), followed Lymphocytic vasculitis 3 8.1 by the 41 to 60 years age group with 13 (35.1%) patients. Seven (19%) patients were over 60 years of Urticarial vasculitis 2 5.4 age. Males were more frequently affected(54%). Ulcer Polyarteritis Nodosa 2 5.4 was found to be the most common clinical Endarteritis 1 2.7 presentation (29.7%) (Table 1).On histopathological Erythema indurate 1 2.7 examination, Majority of the cases, 10 (27%) had Granulomatous leukocytoclastic vasculitis (Figure 1). This was inflammation followed by non specific vasculitis, 7 (18.9%) cases, with vasculitis 1 2.7 cryoglobulinemic vasculitis, 4 (10.8%) cases, Churg Strauss Syndrome 1 2.7 panniculitis with vasculitis and lymphocytic vasculitis Capillaritis 1 2.7 in 3 (8.1%) patients each. Furthermore, urticarial vasculitis and polyarteritis nodosa were diagnosed in Drug induced vasculitis 1 2.7 2 (5.4%) patients (Table 1). Discussion Table 1: Cutaneous Vasculitis- Gross Lesion Cutaneous vasculitis is inflammation of the blood Gross lesion No(%) vessels leading to vessel wall destruction along with Ulcer 11 (29.7) hemorrhage and ischemia. Its incidence ranges from 15.4 to 29.7 cases per million per year.3 However, as Purpura 8 (21.6) this condition is fairly uncommon in our setup, it Nodules 6 (16.2) sometimes manages to elude dermatologists. In this Rash 6(16.2) study, 37 cases of cutaneous vasculitis were reported Plaques 5 (13.5) over a period of more than 2 years. Similar results were shown in a study in Spain.5 Another study in Germany identified 642 primary systemic vasculitidies over 5 years. 6 Adults between the age groups of 41 to 60 years (37.8%) were the most commonly affected age group. On the other hand, Blanco et. al. reported Henoch Schonlein Purpura, a type of cutaneous vasculitis to be more common in children with an average age of about 7 years than in adults whereas a study in Europe showed that Wegener’s Granulomatosis, microscopic polyangiitis and Churg Strauss Syndrome are more common in the older, 65 to 70 year old age group. 7,8 Males were more commonly affected by cutaneous vasculitis in the present study. Similarly, Trapani et. reported a male to female ratio of 1.8:1. 9 In a study by Lane et al. 61.1% of the patients were male. 10 Ulcers were seen to be the most common clinical presentation in the present study. However, the 27 Journal of Rawalpindi Medical College (JRMC); 2015;19(1):26-28 disease more commonly presents as palpable purpura 2. Fiorentino DF. Cutaneous vasculitis. J Am Acad Dermatol, 2003;48(3):311–40. or infiltrated erythema, indicating involvement of the 3. Chen K-R and Carlson JA. Clinical Approach to Cutaneous dermal small vessel vasculitis.3 Leukocytoclastic Vasculitis. Am J Clin Dermatol, 2008;9(2):71– vasculitis (27%) was undoubtedly the most frequent 92. finding. A study in Minnesota showed its incidence to 4. Carlson JA, Cavaliere LF, Grant-Kels JM. Cutaneous 11 vasculitis: diagnosis and management. Clin Dermatol, be 4.5 per 100,000 person years. It manifests as 2006;24(5):414–29. palpable purpura and is diagnosed on histopathology 5. Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V. based on the findings of neutrophilic infiltrate and Cutaneous Vasculitis in Children and Adults: Associated nuclear debris around vessels, endothelial swelling Diseases and Etiologic Factors in 303 Patients. Medicine, 1998;77(6):403–18. 12 and RBC extravasation. Cryoglobulinemic vasculitis 6. Reinhold-Keller E, Herlyn K, Wagner-Bastmeyer R, Gross is characterized by presence of immunoglobulins that WL. Stable incidence of primary systemic vasculitides over precipitate below 37ºC and re-dissolve on re-warming. five years: results from the German vasculitis register. 13 Arthritis Rheum, 2005;53(1):93–99. In Taiwan, 114 cases of the condition were 7. Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, diagnosed over a period of 10 years and almost the García-Fuentes. Henoch-Schönlein purpura in adulthood same was observed in the present study. 14 and childhood. Two different expressions of the same Panniculitis, that is, tender subcutaneous nodules were syndrome. Arthritis Rheum, 1998;40(5):859– 64. seen to be associated with vasculitis (8.1%) was seen to 8. Lane SE, Watts R, Scott DGI. Epidemiology of systemic be present. 15 A study in Barcelona isolated 91 cases of vasculitis. Curr Rheumatol Rep, 2005;7(4):270–75. panniculitis with vasculitis.16 Lymphocytic vasculitis 9. Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini was also identified in 8.1% of the patients. About the F. Henoch Schonlein Purpura in Childhood: Epidemiological and Clinical Analysis of 150 Cases Over a 5-year Period and same frequency (9%) was reported in Albany Medical Review of Literature. Semin Arthritis Rheum, Center. 1 Urticarial vasculitis (5.4%) affects 5-10% 2005;35(3):143–53. patients affected by chronic urticaria.15 Its rare nature 10.
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