These articles have been accepted for publication in the British Journal of Dermatology and are currently being edited and typeset. Readers should note that articles published below have been fully refereed, but have not been through the copy-editing and proof correction process. Wiley-Blackwell and the British Association of Dermatologists cannot be held responsible for errors or consequences arising from the use of information contained in these articles; nor do the views and opinions expressed necessarily reflect those of Wiley-Blackwell or the British Association of Dermatologists This article is protected by copyright. All rights reserved. Accepted Date : 14-Feb-2014 Article type : Correspondence Title: Concurrent Cutaneous Polyarteritis Nodosa and Arteritis Temporalis in a 68Year-Old Female Running head: Cutaneous Polyarteritis Nodosa associated with Arteritis Temporalis Authors: L.K. Andersen1, T. Steiniche2, I.T. Hansen3, M.S. Deleuran1 Author Affiliations: 1
Department of Dermato-Venereology, Aarhus University Hospital, Aarhus C,
Denmark 2
Institute of Pathology, Aarhus University Hospital, Aarhus C, Denmark.
3
Department of Rheumatology, Aarhus University Hospital, Aarhus C, Denmark
Corresponding Author: Louise Kronborg Andersen, Department of Dermato-Venereology, Aarhus University Hospital, P.P. Ørumsgade 11, 8000 Aarhus C, Denmark. E-mail: [email protected] Funding: None Conflict of interest: None Editor, Cutaneous polyarteritis nodosa (CPN) is a rare vasculitis affecting the smalland medium-sized arteries in the reticular dermis and subcutaneous tissue. CPN should be distinguished from systemic polyarteritis nodosa (PAN), which is an aggressive systemic vasculitis with internal organ involvement. Extracutaneous findings of CPN include fever, myalgia, arthralgia, and neuropathy.1 In contrast,
This article is protected by copyright. All rights reserved. Temporal Arteritis (TA) or Giant-cell arteritis (GCA) is a vasculitis that involves the medium- and large arteries, and may develop along with polymyalgia rheumatica.2 Overlapping vasculitic syndrome of PAN and TA has been reported in literature. 3-8 To our knowledge CPN along with TA has only been described in one previous case report.9 We report a 68-year-old female who presented with livedo reticularis with necrotic centres in a starburst pattern on the back, chest, and upper arms of 3 weeks duration. (Fig. 1) Extracutaneous findings included fever, myalgia and weakness in the thighs. She had no other neurological symptoms or symptoms from the gastrointestinal or genitourinary tract. She also denied having any cardiopulmonary symptoms. Her medical history included longterm treatment for depression. A punch biopsy was performed on the upper arm and she was started on prednisolone (50 mg daily). Laboratory tests revealed normal hemoglobin, slightly elevated C-reactive protein (CRP), erythrocyte sedimentation rate (ERS), and leukocytosis. Urin analysis, s-creatinine, glomerular filtration rate, and creatine kinase were normal. Hepatitis A, B, and C serology were negative. Immunoglobin levels, cryoglobulins, anti-nuclear antibody (ANA), anti-neutrophil cystoplasmic antibody (ANCA) and rheumatoid factor were normal. Echocardiography as well as bone marrow aspirate was normal. A CT- scan of chest, abdomen and pelvis showed no sign of malignancy. A 18Fluorodeoxyglucose positron emission tomography (FDG-PET) scan revealed a rectal adenoma, which was surgically removed. The histology of the punch biopsy showed vasculitis in the small- and medium-sized arteries in subcutaneous tissue (Fig. 2 a and b), which are typical findings of PAN. An abdominal angiography showed no involvement of the abdominal vessels. A revised final diagnosis of CPN was made. The patient improved on prednisolone treatment, which was tapered off over the next months. The cutaneous ulcers healed and the other symptoms disappeared. She had no further relapse. Eight months later, she presented with low back pain along with nausea and weight loss. She had fever, moderate leucocytosis, elevated CRP, and high ERS. Physical examination of the abdomen revealed right upper quadrant tenderness with hepatomegaly. She was admitted and started on IV antibiotics but did not improve. A CT scan of the chest, abdomen and pelvis showed slightly dilatation of the ductus choledochus. Magnetic resonance cholangiopancreatography only revealed several liver cysts. After two days she complained over tenderness of the scalp. Physical
This article is protected by copyright. All rights reserved. examination revealed infiltration of the temples on both sides. She had no other symptoms of TA. A right temporal artery biopsy was performed showing inflammatory cell infiltration in relation to the internal elastic lamina with a few multinucleate giant cells and some lymphocytes – typical histological findings of TA (Fig. 2 c and d). She responded on oral prednisone 50 mg daily with complete resolution of her symptoms. At 10 months follow-up she had no symptoms or signs of skin disease or internal disorders. Classification of vasculitic disorders is based on clinical, biochemical, and pathologic findings.10 Overall, the incidence of vasculitis is low, especially those affecting the small vessels. Overlapping features of distinct types of vasculitis are reported in the literature and are predominantly described in young adults (mean age 25).9 It is known that PAN may also involve the temporal arteries.3-8 Concurrent CPN and TA has only been described once before in a 42-year-old male by Leavitt and Fauci.9 Currently, CPN and TA are regarded as distinct clinical and histological entities. CPN and TA are mainly distinguished by their vessel involvement and histopathology. CPN affects the small-and medium-sized arteries in the reticular dermis and subcutaneous tissue whereas TA involves the medium- and large arteries. The histopathology of CPN shows fibrinoid necrosis with thickening and infiltration of the vessel walls. Present are also neutrophils, eosinophils and lymphocytes. In contrast, TA shows infiltrates of T lymphocytes and macrophages with multinucleated giant cells. Occasional plasma cells, neutrophils, and eosinophils occur. In addition, the clinical features of CPN and TA differ. CPN predominantly includes skin lesions on legs, but lesions on arms, torso and neck have also reported. Extracutaneous findings of CPN include fever, myalgia, arthralgia, and neuropathy. On the other hand patients with TA present with headaches, scalp tenderness and infiltration of the temples. Although CPN and TA are different disease entities, both conditions are described to burn out within months to years.1,2 We report a 68-year-old female who had characteristic clinical and histological findings of 2 well-defined types of vasculitides, but with an 8 months interval. Even though overlap exists among well-defined types of vasculitides in one single patient, it is uncommon.
This article is protected by copyright. All rights reserved. Reference: 1 2 3 4 5 6 7 8
9 10
Morgan AJ, Schwartz RA. Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol 2010; 49: 750-6. Kale N, Eggenberger E. Diagnosis and management of giant cell arteritis: a review. Curr Opin Ophthalmol 2010; 21: 417-22. Haugeberg G, Bie R, Johnsen V. Vasculitic changes in the temporal artery in polyarteritis nodosa. Scand J Rheumatol 1997; 26: 383-5. Jawad AS, Hamour AA, Scott DG. Giant cell (temporal) arteritis following polyarteritis nodosa. Br J Rheumatol 1989; 28: 268-70. O'Neill WM, Jr., Hammar SP, Bloomer A. Giant cell arteritis with visceral angiitis. Arch Intern Med 1976; 136: 1157-60. Frayha RA, Abu-Haidar F. Polyarteritis nodosa masquerading as temporal arteritis. J Rheumatol 1979; 6: 76-9. Highton J, Anderson KR. Concurrent polyarteritis nodosa and temporal arteritis. N Z Med J 1984; 97: 766-7. Walz LeBlanc BA, Keystone EC, Feltis JT et al. Polyarteritis nodosa clinically masquerading as temporal arteritis with lymphadenopathy. J Rheumatol 1994; 21: 949-52. Leavitt RY, Fauci AS. Polyangiitis overlap syndrome. Classification and prospective clinical experience. Am J Med 1986; 81: 79-85. Jennette JC, Falk RJ, Bacon PA et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65: 1-11.
Figure legends:
Figure 1: Clinical presentation of livedo reticularis with necrotic centres in a starburst pattern on the chest and upper arms (a) and back (b). Figure 2: Punch biopsy from the upper arm with vasculitis affecting small- and medium sized arteries in the upper subcutis (a). The affected artery is heavily infiltrated by neutrophils and some eosinophils and lymphocytes. Fibrin extravasation is seen in the vessel wall (b). Serial sections from the temporal artery (c).Inflammatory cell infiltration is present in relation to the internal elastic lamina with a few multinucleate giant cells and some lymphocytes (d).
This article is protected by copyright. All rights reserved.
Department of Dermato-Venereology, Aarhus University Hospital, Aarhus C,
Denmark 2
Institute of Pathology, Aarhus University Hospital, Aarhus C, Denmark.
3
Department of Rheumatology, Aarhus University Hospital, Aarhus C, Denmark
Corresponding Author: Louise Kronborg Andersen, Department of Dermato-Venereology, Aarhus University Hospital, P.P. Ørumsgade 11, 8000 Aarhus C, Denmark. E-mail: [email protected] Funding: None Conflict of interest: None Editor, Cutaneous polyarteritis nodosa (CPN) is a rare vasculitis affecting the smalland medium-sized arteries in the reticular dermis and subcutaneous tissue. CPN should be distinguished from systemic polyarteritis nodosa (PAN), which is an aggressive systemic vasculitis with internal organ involvement. Extracutaneous findings of CPN include fever, myalgia, arthralgia, and neuropathy.1 In contrast,
This article is protected by copyright. All rights reserved. Temporal Arteritis (TA) or Giant-cell arteritis (GCA) is a vasculitis that involves the medium- and large arteries, and may develop along with polymyalgia rheumatica.2 Overlapping vasculitic syndrome of PAN and TA has been reported in literature. 3-8 To our knowledge CPN along with TA has only been described in one previous case report.9 We report a 68-year-old female who presented with livedo reticularis with necrotic centres in a starburst pattern on the back, chest, and upper arms of 3 weeks duration. (Fig. 1) Extracutaneous findings included fever, myalgia and weakness in the thighs. She had no other neurological symptoms or symptoms from the gastrointestinal or genitourinary tract. She also denied having any cardiopulmonary symptoms. Her medical history included longterm treatment for depression. A punch biopsy was performed on the upper arm and she was started on prednisolone (50 mg daily). Laboratory tests revealed normal hemoglobin, slightly elevated C-reactive protein (CRP), erythrocyte sedimentation rate (ERS), and leukocytosis. Urin analysis, s-creatinine, glomerular filtration rate, and creatine kinase were normal. Hepatitis A, B, and C serology were negative. Immunoglobin levels, cryoglobulins, anti-nuclear antibody (ANA), anti-neutrophil cystoplasmic antibody (ANCA) and rheumatoid factor were normal. Echocardiography as well as bone marrow aspirate was normal. A CT- scan of chest, abdomen and pelvis showed no sign of malignancy. A 18Fluorodeoxyglucose positron emission tomography (FDG-PET) scan revealed a rectal adenoma, which was surgically removed. The histology of the punch biopsy showed vasculitis in the small- and medium-sized arteries in subcutaneous tissue (Fig. 2 a and b), which are typical findings of PAN. An abdominal angiography showed no involvement of the abdominal vessels. A revised final diagnosis of CPN was made. The patient improved on prednisolone treatment, which was tapered off over the next months. The cutaneous ulcers healed and the other symptoms disappeared. She had no further relapse. Eight months later, she presented with low back pain along with nausea and weight loss. She had fever, moderate leucocytosis, elevated CRP, and high ERS. Physical examination of the abdomen revealed right upper quadrant tenderness with hepatomegaly. She was admitted and started on IV antibiotics but did not improve. A CT scan of the chest, abdomen and pelvis showed slightly dilatation of the ductus choledochus. Magnetic resonance cholangiopancreatography only revealed several liver cysts. After two days she complained over tenderness of the scalp. Physical
This article is protected by copyright. All rights reserved. examination revealed infiltration of the temples on both sides. She had no other symptoms of TA. A right temporal artery biopsy was performed showing inflammatory cell infiltration in relation to the internal elastic lamina with a few multinucleate giant cells and some lymphocytes – typical histological findings of TA (Fig. 2 c and d). She responded on oral prednisone 50 mg daily with complete resolution of her symptoms. At 10 months follow-up she had no symptoms or signs of skin disease or internal disorders. Classification of vasculitic disorders is based on clinical, biochemical, and pathologic findings.10 Overall, the incidence of vasculitis is low, especially those affecting the small vessels. Overlapping features of distinct types of vasculitis are reported in the literature and are predominantly described in young adults (mean age 25).9 It is known that PAN may also involve the temporal arteries.3-8 Concurrent CPN and TA has only been described once before in a 42-year-old male by Leavitt and Fauci.9 Currently, CPN and TA are regarded as distinct clinical and histological entities. CPN and TA are mainly distinguished by their vessel involvement and histopathology. CPN affects the small-and medium-sized arteries in the reticular dermis and subcutaneous tissue whereas TA involves the medium- and large arteries. The histopathology of CPN shows fibrinoid necrosis with thickening and infiltration of the vessel walls. Present are also neutrophils, eosinophils and lymphocytes. In contrast, TA shows infiltrates of T lymphocytes and macrophages with multinucleated giant cells. Occasional plasma cells, neutrophils, and eosinophils occur. In addition, the clinical features of CPN and TA differ. CPN predominantly includes skin lesions on legs, but lesions on arms, torso and neck have also reported. Extracutaneous findings of CPN include fever, myalgia, arthralgia, and neuropathy. On the other hand patients with TA present with headaches, scalp tenderness and infiltration of the temples. Although CPN and TA are different disease entities, both conditions are described to burn out within months to years.1,2 We report a 68-year-old female who had characteristic clinical and histological findings of 2 well-defined types of vasculitides, but with an 8 months interval. Even though overlap exists among well-defined types of vasculitides in one single patient, it is uncommon.
This article is protected by copyright. All rights reserved. Reference: 1 2 3 4 5 6 7 8
9 10
Morgan AJ, Schwartz RA. Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol 2010; 49: 750-6. Kale N, Eggenberger E. Diagnosis and management of giant cell arteritis: a review. Curr Opin Ophthalmol 2010; 21: 417-22. Haugeberg G, Bie R, Johnsen V. Vasculitic changes in the temporal artery in polyarteritis nodosa. Scand J Rheumatol 1997; 26: 383-5. Jawad AS, Hamour AA, Scott DG. Giant cell (temporal) arteritis following polyarteritis nodosa. Br J Rheumatol 1989; 28: 268-70. O'Neill WM, Jr., Hammar SP, Bloomer A. Giant cell arteritis with visceral angiitis. Arch Intern Med 1976; 136: 1157-60. Frayha RA, Abu-Haidar F. Polyarteritis nodosa masquerading as temporal arteritis. J Rheumatol 1979; 6: 76-9. Highton J, Anderson KR. Concurrent polyarteritis nodosa and temporal arteritis. N Z Med J 1984; 97: 766-7. Walz LeBlanc BA, Keystone EC, Feltis JT et al. Polyarteritis nodosa clinically masquerading as temporal arteritis with lymphadenopathy. J Rheumatol 1994; 21: 949-52. Leavitt RY, Fauci AS. Polyangiitis overlap syndrome. Classification and prospective clinical experience. Am J Med 1986; 81: 79-85. Jennette JC, Falk RJ, Bacon PA et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65: 1-11.
Figure legends:
Figure 1: Clinical presentation of livedo reticularis with necrotic centres in a starburst pattern on the chest and upper arms (a) and back (b). Figure 2: Punch biopsy from the upper arm with vasculitis affecting small- and medium sized arteries in the upper subcutis (a). The affected artery is heavily infiltrated by neutrophils and some eosinophils and lymphocytes. Fibrin extravasation is seen in the vessel wall (b). Serial sections from the temporal artery (c).Inflammatory cell infiltration is present in relation to the internal elastic lamina with a few multinucleate giant cells and some lymphocytes (d).
This article is protected by copyright. All rights reserved.
