DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20160554

Digital gangrene a rare early manifestation of Wegener’s granulomatosis: a case report

Akash Mathur, Dinesh Gurjar, Arvind Lakesar, Ramkrishna Sai, Aradhana Singh, Hemant Malhotra

Abstract


Wegener's granulomatosis (WG) or Granulomatosis with polyangiitis (GPA) rarely presents with digital gangrene being an early manifestation. We report a case of 60-year-old woman who presented with fever and cough with occasional hemoptysis along with painful bilateral feet digital gangrene. X ray chest and HRCT thorax of the patient was suggestive of multiple nodulo-cavitatory lesions. HPE of the lung lesion showed granulomatous pathology. CT Angiography was suggestive of multiple calcified and non-calcified lesions in infra renal aorta with stenosis of right dorsalis pedis artery. Biopsy of the skin lesion revealed a leucocytoclastic vasculitis. We eventually diagnosed GPA in light of clinical symptomatology with HPE of lung mass showing granulomatous lesion along with a positive cANCA. A few months after starting combined cyclophosphamide and steroids patient had significant symptomatic improvement. Hence Wegener’s granulomatosis should always be considered as a possibility while evaluating a patient of digital gangrene specially with pulmonary and renal involvement.


Keywords


Wegener's granulomatosis, Granulomatosis with polyangiitis, Digital gangrene

Full Text:

PDF

References


Falk RJ, Gross WL, Guillevin L, Hoffman GS, Jayne DR, Jennette JC. Granulomatosis with polyangiitis (Wegener's): An alternative name for Wegener's granulomatosis. Ann. Rheum. Dis. Arthritis Rheum. 2011;63(4):863-4.

Berden A, Göçeroglu A, Jayne D, Luqmani R, Rasmussen N, Bruijn JA. Diagnosis and management of ANCA associated vasculitis. BMJ. 2012;344:e26.

Tracy CL, Papadopoulos PJ, Bye MR, Connolly H, Goldberg E, O'Brian RJ, Sharma GD, et al. (10 February 2014). Diamond, HS, ed. Granulomatosis with Polyangiitis Treatment & Management. Medscape Reference. WebMD. Retrieved 22 December 2015.

Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med. 2004;117(1):39-50.

Hoffman G, Kerr G, Leavitt X. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116:488-98.

Frances C, Du L, Piette J. Wegener’s granulomatosis: Dermatological manifestations in 75 cases with clinicopathologic correlation. Arch Dermatol. 1994;130:861-7.

Comfere NI, Macaron NC, Gibson LE. Cutaneous manifestations in Wegener’s granulomatosis: a clinicopathologic study of 17 patients and correlation to antineutrophil cytoplasmic antibody status. J Cutan Pathol. 2007;34:739-47.

Van der Woude FJ, Rasmussen N, Lobatto S. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis. Lancet. 1985;1(8426):425-9.

Bessias N, Moulakakis KG, Lioupis C, Bakogiannis K, Sfyroeras G, Kakaletri K. Wegener’s granulomatosis presenting during pregnancy with acute limb ischemia. J Vasc Surg. 2005;42:800-4.