DOI: http://dx.doi.org/10.18203/issn.2455-4529.IntJResDermatol20183169

An insight into the etiopathogenesis, clinical patterns, treatment outcome and repercussions of cutaneous small-vessel vasculitis

Rajkumar Kannan, Muthusubramanian Chandrasekar, Sridhar Venu, Jayakalyani Vijayananth

Abstract


Background: Cutaneous small-vessel vasculitis (CSVV) is a group of disorder which is characterised by involvement of capillaries, arterioles and venules. CSVV can be idiopathic or primary, or secondary to infection, drugs or as a part and parcel of underlying systemic disease. The aim of our study is to find out the etiological factors, treatment options and their outcome in CSVV.

Methods: We analysed 75 cases of CSVV out of patients who attended Dermatology OPD, in a tertiary care-centre from April 2017 to March 2018.The study design was descriptive study. A detailed history taking, thorough clinical examination and appropriate relevant investigations including biopsy were done for all the patients fulfilling the inclusion criteria and exclusion criteria.

Results: A sample size of 75 patients (53 women and 22 men) were included in the study. Their mean age was found to be 25 years (range 18-40). The following etiological factors were made out in our study: Benign isolated (40) patients, (53%), infective etiology (14) patients, (19%), vasculitis in background of ANA/dsDNA/ANCA positivity (12) patients, (16%), drug induced (9), patients (12%). The main clinical manifestations of CSVV in our study were found to be the following viz, palpable purpura in all 75 patients (100%), fever & malaise in 30 patients, (40%), ulcers in 30 patients (40%) arthritis/arthralgia in 15 patients, (20%). After a median follow up of 6 months, complete recovery was observed in all patients, although relapses occurred in 8 patients (11%).

Conclusions: CSVV is usually associated with other vasculitis and connective tissue disorders and patients turning ANCA positive somewhere in the course of the disease is of ominous sign and hence, it becomes mandatory to keep these patients on a long term vigil. 


Keywords


Cutaneous small vessel vasculitis, Anti-nuclear antibody, Leucocytoclastic vasculitis, Fibrinoid necrosis, Vasculitic ulcers, Anti-neutrophil cytoplasmic antibody positive vasculitis

Full Text:

PDF

References


Griffiths C, Barker J, Bleiker, Chalmers R. Rook’s Textbook of Dermatology: 9th ed. London, UK: Wiley Blackwell; 2016: 102(1-8).

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. A rthritis Rheum. 2013;65(1):1–11.

Calabrese LH, Michel BA, Bloch DA, Arend WP, Edworthy SM, Fauci AS, et al. The American College of Rheumatology 1990 criteria for the classification of hypersensitivity vasculitis. Arthritis Rheum. 1990;33(8):1108 –13.

Garcia‐Porrua C, Gonzalez‐Gay MA. Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch‐Schonlein purpura in adults. Semin Arthritis Rheum. 1999;28(6):404-12.

Watts RA, Jolliffe VA, Grattan CE, Elliott J, Lockwood M, Scott DG. Cutaneous vasculitis in a defi ned population – clinical and epidemiological associations. J Rheumatol. 1998;25(5):920-4.

Martinez‐Taboada VM, Blanco R, Garcia‐Fuentes M, Rodriguez‐Valverde V. Clinical features and outcome of 95 patients with hypersensitivity vasculitis. Am J Med. 1997;102( 2):186-91.

Boom BW, Mommaas AM, Vermeer BJ. Presence and interpretation of vascular immune deposits in human skin: the value of direct immune-fluorescence. J Dermatol Sci. 1992;3(1):26-34.

Garcia‐Porrua C, Gonzalez‐Gay MA. Bacterial infection presenting as cutaneous vasculitis in adults. Clin Exp Rheumatol. 1999;17(4):471-3.

Cribier B, Couilliet D, Meyer P, Grosshans E. The severity of histopathological changes of leukocytoclastic vasculitis is not predictive of extracutaneous involvement. Am J Dermatopathol. 1999;21(6):532-6.

Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). A nn Rheum Dis. 2009;68(12):1827-32.

Podjasek JO, Wetter DA, Pittelkow MR, Wada DA. Cutaneous small vessel vasculitis associated with solid organ malignancies: the Mayo Clinic experience, 1996 to 2009. J Am Acad Dermatol. 2012;62(2):55-65.

Blanco R, Martı´nez-Taboada VM, Rodrıguez-Valverde V, Garcı´a-Fuentes M. Cutaneous vasculitis in children and adults: associated diseases and etiologic factors in 303 patients. Medicine. 1998;77:403-18.

Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37:187-92.

Loricera J, Calvo-Rı´o V, Ortiz-Sanjua´n F, González-López MA, Fernández-Llaca H, Rueda-Gotor J, et al. The spectrum of paraneoplastic cutaneous vasculitis in a defined population: incidence and clinical features. Medicine. 2013;92:331-43.

Loricera J, Calvo-Rı´o V, Mata C, Ortiz-Sanjuán F, González-López MA, et al. Urticarial vasculitis in northern Spain: clinical study of 21 cases. Medicine. 2014;93:53-60.

Soter NA, Austen KF. Cutaneous necrotizing angiitis. In: Samter M, editor. Immunological diseases. Boston:Little, Brown; 1978: 993.

Ekenstam E, Callen JP. Cutaneous leukocytoclastic vasculitis: clinical and laboratory features of 82 patientsseen in private practice. Arch Dermatol 1984;120:484-90.

Lotti T, Comacchi C, Ghersetich I. Cutaneous necrotizing vasculitis. Int J Dermatol. 1996;35:457-74.

1. Griffiths C, Barker J, Bleiker, Chalmers R. Rook’s Textbook of Dermatology. 8th ed. London, UK: Wiley Blackwell; 2010:50:13.

Comacchi C, Ghersetich I, Lotti T. Vasculite necrotizzante cutanea. G Ital Dermatol Venereol 1998;133:23-49.

Ryan TJ. Cutaneous vasculitis. In: Rook A, Wilkinson DS, Ebling FJG, editors. Textbook of dermatology. Oxford: Blackwell Scientific Publications; 1992: 1893-1861.