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British Journal of Oral and Maxillofacial Surgery 53 (2015) 883–885
Short communication
Polyarteritis nodosa associated with necrosis of the tongue: a rare presentation in an adult patient Coziana Ciurtin a,∗ , Ami Shirodaria b , Jessica Manson a , Roddy MacMillan c a b c
University College London Hospitals NHS Trust, Department of Rheumatology University College London, Division of Medicine University College London - Eastman Dental Hospital, Department of Oral Medicine
Accepted 6 June 2015 Available online 25 June 2015
Abstract Polyarteritis nodosa is a rare form of vasculitis of the medium-sized vessels, which leads to the formation of microaneurysms, thrombosis, organ ischaemia, and necrosis. Involvement of the oral cavity is rare. To our knowledge, this is the first case of localised polyarteritis nodosa in an adult with ulceration of the tongue and necrosis at the onset of the disease. The patient made a full recovery after the use of strong immunosuppressants. © 2015 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Keywords: Vasculitis; Skin nodules; Tongue necrosis
Introduction Polyarteritis nodosa is a rare autoimmune disease, which affects medium and small muscular arteries, and is characterised by necrotising inflammatory lesions. It is predominantly seen in patients aged 45-65 years, has an incidence of 3 - 4.5 cases/100 000 population/year and usually affects skin, joints, peripheral nerves, the gut, and the kidneys.1 Patients typically present with systemic symptoms such as fever, weakness, arthralgia, muscle pain, and those that suggest involvement of the organs (high blood pressure, renal insufficiency, sensory abnormalities, abdominal pain, or nodules on the skin).2 Involvement of the oral cavity is exceptionally rare. We know of only a few cases in children that
∗ Corresponding author at: Department of Rheumatology, University College London, 3rd Floor Central, 250 Euston Road, London, NW1 2PG. Tel.: +44(0)34497035; fax: +44(0)34479278. E-mail address: [email protected] (C. Ciurtin).
involved necrosis of the tongue at the onset of disease.3–5 We describe the unusual case of an adult diagnosed with limited polyarteritis nodosa associated with necrosis of the tongue and a nodular skin rash, which responded well to strong immunosuppressants.
Case report In 2011, a 29-year-old woman presented with recent symptoms of a low-grade fever, generalised weakness of the muscles, loss of appetite, a burning sensation in her tongue, and subcutaneous nodules on her upper thighs. A few days after the onset of symptoms, she developed severe pain and swelling of her tongue, which became ulcerated. She lived in London and was a trainee general practitioner. At the age of 10, she had had cutaneous post-streptococcal vasculitis, which had been successfully treated for 2 years with oral steroids, and she had been in remission for 18 years. There was no evidence of chronic illness or the use of illicit drugs.
http://dx.doi.org/10.1016/j.bjoms.2015.06.009 0266-4356/© 2015 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
884
C. Ciurtin et al. / British Journal of Oral and Maxillofacial Surgery 53 (2015) 883–885
Fig. 1. Asymmetrical enlargement of the tongue with irregular, necrotic ulcerations and associated pseudomembranes.
Fig. 3. Histological examination of a biopsy specimen of the tongue showed chronic inflammatory cell infiltrate in the wall of a thick-walled blood vessel (black arrow) and partial obliteration of the vascular lumen (open arrow) (haematoxylin and eosin, original magnification x 10).
given intravenously at a dose of 15 mg/kg/month was started because she did not respond to steroids, and this continued for 6 months. She was then given a maintenance dose of methotrexate (15 mg/week), together with reducing doses of oral steroids. The tongue healed after about 9 months.
Discussion
Fig. 2. Histological examination of a deep skin biopsy showed panniculitis and prominent fibrinoid necrosis of small and mediumsized blood vessels (black arrow) (haematoxylin and eosin, original magnification x 10).
She reported no recent travels, infections, or insect bites. Her blood pressure and urinalysis were normal. Her tongue was enlarged asymmetrically, with widespread, irregular, necrotic ulcerations, and was mostly covered with pseudomembranous tissue (Fig. 1). She also had a rash similar to erythema nodosum on her thighs, and enlarged cervical lymph nodes. There was a normal salivary pool in her submandibular salivary glands. Laboratory tests showed mild anaemia with normal red cell indices, high inflammatory markers (C-reactive protein (CRP) 2120 mg/L and erythrocyte sedimentation rate (ESR) 103 mm in the first hour) with acute phase response (low albumin, increased alpha-1 globulins), and no other abnormalities. Biopsy examination of the lesions on the skin showed neutrophilic panniculitis with a lymphocytic and histiocytic inflammatory infiltrate, and prominent fibrinoid necrosis of the walls of small to medium-sized vessels (Fig. 2). Deep biopsy examination of the tongue, which showed necrotising vasculitis of the medium muscular arteries, led to a diagnosis of polyarteritis nodosa (Fig. 3). Additionally, biopsy examination of a salivary gland showed only inflammation that was non-specific. Cyclophosphamide
Classic polyarteritis nodosa is characterised by necrotising inflammation of muscular arterioles and medium-sized arteries that spares the capillaries.6 Clinical examination highlights areas where biopsy by deep incision can gain the most information for diagnosis.7 In our case, the main differential diagnosis included Sjögren syndrome, sarcoid, amyloidosis, acromegaly, and infective granulomatous diseases, but the additional investigations ruled out associated disease. Involvement of the salivary gland is not a feature of polyarteritis nodosa, but the association between the two conditions has been discussed by other authors.8 Isolated necrosis of the tongue in polyarteritis nodosa is exceptional; to our knowledge, there has been only one other case report of necrotising vasculitis associated with extensive necrosis of the tongue and soft and hard palates in an adult.9 As there are no guidelines for the management of aggressive localised polyarteritis nodosa, it can be challenging to treat. Current recommendations include the use of strong immunosuppressants when critical organs are involved and for other life-threatening complications. In a recent prospective study, patients with localised disease were treated with steroids alone, which resulted in a good long-term outcome.10 Our patient’s tongue healed without sequelae, but she needed treatment with cyclophosphamide to achieve this. There was no evidence of a relapse 18 months after the diagnosis. Necrosis of the tongue is a rare manifestation of polyarteritis nodosa, which needs prompt histological diagnosis and treatment to ensure a good long-term prognosis.
C. Ciurtin et al. / British Journal of Oral and Maxillofacial Surgery 53 (2015) 883–885
Conflict of Interest We have no conflicts of interest.
Ethics statement/confirmation of patient permission We obtained the patient’s permission.
References 1. Pettigrew HD, Teuber SS, Gershwin ME. Polyarteritis nodosa. Compr Ther 2007;33:144–9. 2. Stone JH. Polyarteritis nodosa. JAMA 2002;288:1632–9. 3. Buonuomo PS, El Hachem M, Callea F, et al. Necrosis of the tongue as first symptom of Polyarteritis Nodosa (PAN): unusual presentation of a rare disease in children. Rheumatol Int 2013;33:1071–3.
885
4. Mondal R, Sarkar S, Khan KA, et al. Necrotic tongue ulcer: an unusual presentation of childhood polyarteritis nodosa. Indian J Pediatr 2013;80:974–5. 5. Ekman-Joelsson BM, Kjellman B, Hattevig G. Tongue necrosis due to vasculitis. Acta Paediatr 1995;84:1333–6. 6. Diamantopoulos AP, Pettersen P. Polyarteritis nodosa. J Rheumatol 2013;40:87–8. 7. Furukawa F. Cutaneous polyarteritis nodosa: an update. Ann Vasc Dis 2012;5:282–8. 8. Yukawa S, Tahara K, Yukawa N, et al. Dramatic regression of mesenteric abnormalities demonstrated on angiography following prednisolone and cyclophosphamide combination therapy in a patient with polyarteritis nodosa associated with Sjögren’s syndrome. Mod Rheumatol 2008;18:416–21. 9. Phillips TJ, Sader C, Bullock M, et al. Necrotizing vasculitis resulting in necrosis of the entire oral tongue. J Otolaryngol Head Neck Surg 2012;41:E38–40. 10. Samson M, Puéchal X, Devilliers H, et al. Long-term follow-up of a randomized trial on 118 patients with polyarteritis nodosa or microscopic polyangiitis without poor-prognosis factors. Autoimmun Rev 2014;13:197–205.
British Journal of Oral and Maxillofacial Surgery 53 (2015) 883–885
Short communication
Polyarteritis nodosa associated with necrosis of the tongue: a rare presentation in an adult patient Coziana Ciurtin a,∗ , Ami Shirodaria b , Jessica Manson a , Roddy MacMillan c a b c
University College London Hospitals NHS Trust, Department of Rheumatology University College London, Division of Medicine University College London - Eastman Dental Hospital, Department of Oral Medicine
Accepted 6 June 2015 Available online 25 June 2015
Abstract Polyarteritis nodosa is a rare form of vasculitis of the medium-sized vessels, which leads to the formation of microaneurysms, thrombosis, organ ischaemia, and necrosis. Involvement of the oral cavity is rare. To our knowledge, this is the first case of localised polyarteritis nodosa in an adult with ulceration of the tongue and necrosis at the onset of the disease. The patient made a full recovery after the use of strong immunosuppressants. © 2015 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Keywords: Vasculitis; Skin nodules; Tongue necrosis
Introduction Polyarteritis nodosa is a rare autoimmune disease, which affects medium and small muscular arteries, and is characterised by necrotising inflammatory lesions. It is predominantly seen in patients aged 45-65 years, has an incidence of 3 - 4.5 cases/100 000 population/year and usually affects skin, joints, peripheral nerves, the gut, and the kidneys.1 Patients typically present with systemic symptoms such as fever, weakness, arthralgia, muscle pain, and those that suggest involvement of the organs (high blood pressure, renal insufficiency, sensory abnormalities, abdominal pain, or nodules on the skin).2 Involvement of the oral cavity is exceptionally rare. We know of only a few cases in children that
∗ Corresponding author at: Department of Rheumatology, University College London, 3rd Floor Central, 250 Euston Road, London, NW1 2PG. Tel.: +44(0)34497035; fax: +44(0)34479278. E-mail address: [email protected] (C. Ciurtin).
involved necrosis of the tongue at the onset of disease.3–5 We describe the unusual case of an adult diagnosed with limited polyarteritis nodosa associated with necrosis of the tongue and a nodular skin rash, which responded well to strong immunosuppressants.
Case report In 2011, a 29-year-old woman presented with recent symptoms of a low-grade fever, generalised weakness of the muscles, loss of appetite, a burning sensation in her tongue, and subcutaneous nodules on her upper thighs. A few days after the onset of symptoms, she developed severe pain and swelling of her tongue, which became ulcerated. She lived in London and was a trainee general practitioner. At the age of 10, she had had cutaneous post-streptococcal vasculitis, which had been successfully treated for 2 years with oral steroids, and she had been in remission for 18 years. There was no evidence of chronic illness or the use of illicit drugs.
http://dx.doi.org/10.1016/j.bjoms.2015.06.009 0266-4356/© 2015 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
884
C. Ciurtin et al. / British Journal of Oral and Maxillofacial Surgery 53 (2015) 883–885
Fig. 1. Asymmetrical enlargement of the tongue with irregular, necrotic ulcerations and associated pseudomembranes.
Fig. 3. Histological examination of a biopsy specimen of the tongue showed chronic inflammatory cell infiltrate in the wall of a thick-walled blood vessel (black arrow) and partial obliteration of the vascular lumen (open arrow) (haematoxylin and eosin, original magnification x 10).
given intravenously at a dose of 15 mg/kg/month was started because she did not respond to steroids, and this continued for 6 months. She was then given a maintenance dose of methotrexate (15 mg/week), together with reducing doses of oral steroids. The tongue healed after about 9 months.
Discussion
Fig. 2. Histological examination of a deep skin biopsy showed panniculitis and prominent fibrinoid necrosis of small and mediumsized blood vessels (black arrow) (haematoxylin and eosin, original magnification x 10).
She reported no recent travels, infections, or insect bites. Her blood pressure and urinalysis were normal. Her tongue was enlarged asymmetrically, with widespread, irregular, necrotic ulcerations, and was mostly covered with pseudomembranous tissue (Fig. 1). She also had a rash similar to erythema nodosum on her thighs, and enlarged cervical lymph nodes. There was a normal salivary pool in her submandibular salivary glands. Laboratory tests showed mild anaemia with normal red cell indices, high inflammatory markers (C-reactive protein (CRP) 2120 mg/L and erythrocyte sedimentation rate (ESR) 103 mm in the first hour) with acute phase response (low albumin, increased alpha-1 globulins), and no other abnormalities. Biopsy examination of the lesions on the skin showed neutrophilic panniculitis with a lymphocytic and histiocytic inflammatory infiltrate, and prominent fibrinoid necrosis of the walls of small to medium-sized vessels (Fig. 2). Deep biopsy examination of the tongue, which showed necrotising vasculitis of the medium muscular arteries, led to a diagnosis of polyarteritis nodosa (Fig. 3). Additionally, biopsy examination of a salivary gland showed only inflammation that was non-specific. Cyclophosphamide
Classic polyarteritis nodosa is characterised by necrotising inflammation of muscular arterioles and medium-sized arteries that spares the capillaries.6 Clinical examination highlights areas where biopsy by deep incision can gain the most information for diagnosis.7 In our case, the main differential diagnosis included Sjögren syndrome, sarcoid, amyloidosis, acromegaly, and infective granulomatous diseases, but the additional investigations ruled out associated disease. Involvement of the salivary gland is not a feature of polyarteritis nodosa, but the association between the two conditions has been discussed by other authors.8 Isolated necrosis of the tongue in polyarteritis nodosa is exceptional; to our knowledge, there has been only one other case report of necrotising vasculitis associated with extensive necrosis of the tongue and soft and hard palates in an adult.9 As there are no guidelines for the management of aggressive localised polyarteritis nodosa, it can be challenging to treat. Current recommendations include the use of strong immunosuppressants when critical organs are involved and for other life-threatening complications. In a recent prospective study, patients with localised disease were treated with steroids alone, which resulted in a good long-term outcome.10 Our patient’s tongue healed without sequelae, but she needed treatment with cyclophosphamide to achieve this. There was no evidence of a relapse 18 months after the diagnosis. Necrosis of the tongue is a rare manifestation of polyarteritis nodosa, which needs prompt histological diagnosis and treatment to ensure a good long-term prognosis.
C. Ciurtin et al. / British Journal of Oral and Maxillofacial Surgery 53 (2015) 883–885
Conflict of Interest We have no conflicts of interest.
Ethics statement/confirmation of patient permission We obtained the patient’s permission.
References 1. Pettigrew HD, Teuber SS, Gershwin ME. Polyarteritis nodosa. Compr Ther 2007;33:144–9. 2. Stone JH. Polyarteritis nodosa. JAMA 2002;288:1632–9. 3. Buonuomo PS, El Hachem M, Callea F, et al. Necrosis of the tongue as first symptom of Polyarteritis Nodosa (PAN): unusual presentation of a rare disease in children. Rheumatol Int 2013;33:1071–3.
885
4. Mondal R, Sarkar S, Khan KA, et al. Necrotic tongue ulcer: an unusual presentation of childhood polyarteritis nodosa. Indian J Pediatr 2013;80:974–5. 5. Ekman-Joelsson BM, Kjellman B, Hattevig G. Tongue necrosis due to vasculitis. Acta Paediatr 1995;84:1333–6. 6. Diamantopoulos AP, Pettersen P. Polyarteritis nodosa. J Rheumatol 2013;40:87–8. 7. Furukawa F. Cutaneous polyarteritis nodosa: an update. Ann Vasc Dis 2012;5:282–8. 8. Yukawa S, Tahara K, Yukawa N, et al. Dramatic regression of mesenteric abnormalities demonstrated on angiography following prednisolone and cyclophosphamide combination therapy in a patient with polyarteritis nodosa associated with Sjögren’s syndrome. Mod Rheumatol 2008;18:416–21. 9. Phillips TJ, Sader C, Bullock M, et al. Necrotizing vasculitis resulting in necrosis of the entire oral tongue. J Otolaryngol Head Neck Surg 2012;41:E38–40. 10. Samson M, Puéchal X, Devilliers H, et al. Long-term follow-up of a randomized trial on 118 patients with polyarteritis nodosa or microscopic polyangiitis without poor-prognosis factors. Autoimmun Rev 2014;13:197–205.
