BMJ Case Rep: first published as 10.1136/bcr-2019-232183 on 2 February 2020. Downloaded from Unusual association of diseases/symptoms

Case report Mesenteric panniculitis in a patient with new onset dermatomyositis Pankaj Bansal,1 Emily L Gilbert ‍ ‍ ,2 Rodrigo Otavio Lami Pereira,3 Andrew R Virata4

1Rheumatology, Mayo Clinic SUMMARY osteoarthritis of knees and hips, Bell’s palsy, gener- Health System, Eau Claire, Mesenteric panniculitis (MP), part of the spectrum alised anxiety disorder, irritable bowel syndrome Wisconsin, USA of sclerosing mesenteritis, is an often asymptomatic and osteoporosis with T12 compression fracture. 2Internal Medicine, Mayo disorder that is characterised by chronic inflammation Four months prior to rheumatologic evaluation, she Clinic Hospital Jacksonville, of abdominal mesentery. We present a case of an Jacksonville, Florida, USA noted a pruritic, erythematous rash on her face and 3Anesthesiology and 83-­year-old­ woman who presented with proximal upper torso in a photodistribution (figure 1). She Perioperative Medicine - FLA, muscle weakness and erythematous, photosensitive was evaluated by a dermatologist and underwent Mayo Clinic, Jacksonville, rash of the face and upper torso and was subsequently skin biopsy showing vacuolar interface dermatitis Florida, USA diagnosed with dermatomyositis based on skin with increased dermal mucin, for which, she was 4 Pathology, Mayo Clinic Health biopsy, electromyography and muscle biopsy. She had prescribed topical corticosteroids (figures 2–4). System, Eau Claire, Wisconsin, radiographic evidence of panniculitis on CT scan of the A few weeks later, she started noticing proximal USA abdomen and pelvis for malignancy surveillance, which muscle weakness in both the upper and lower improved on serial CT scan 3 months after beginning extremities. Specifically, she had difficulty getting Correspondence to treatment for her underlying dermatomyositis with Dr Pankaj Bansal; up from a chair and used her hands to support prednisone and mycophenolate mofetil. Our case bansal.​ ​pankaj@mayo.​ ​edu herself and complete the movement. Review of highlights that MP can be associated with underlying systems was specifically negative for shortness of Accepted 14 January 2020 autoimmune disease. Connective tissue disease could be considered in the differential of MP when other breath, cough, chest pain, dysphagia, odynophagia,

etiologies such as surgery, trauma and malignancy are Raynaud’s, joint pain or swelling, fevers, night by copyright. ruled out. sweats, weight loss, abdominal pain, vomiting, or any new gastrointestinal complaints. Medical history was negative for any malignancies. Physical examination was remarkable for violaceous, edem- Background http://casereports.bmj.com/ atous periorbital erythema with minimal overlying Mesenteric panniculitis (MP) is a rare disorder scaling. On her chest, a telangiectatic eruption was first recognised nearly a century ago involving seen in a photodistribution. Joint examination chronic fibrosing inflammation of the abdominal demonstrated Heberden's and Bouchard nodes mesentery.1 2 MP is part of a spectrum of conditions based on histological findings including mesenteric in the hands without tenderness or synovitis and lipodystrophy and retractile mesenteritis; collec- no synovitis in any other joint. Muscle exam- tively these are grouped under the umbrella term ination showed symmetric weakness (4/5) in the sclerosing mesenteritis (SM).3–7 Though many hip and thigh flexor and extensor muscles. Distal patients are asymptomatic, chronic inflammation of lower extremity and upper extremity strength was the mesentery can lead to non-­specific symptoms preserved. She had difficulty getting out of a chair on March 30, 2020 at Weill Cornell Medical Library. Protected such as nausea/vomiting, abdominal pain, fever without support. and weight loss. While the aetiology of mesen- teric panniculitis is yet to be elucidated, it has been associated with surgery, trauma, cancer and less frequently with autoimmune diseases including Sjögren’s syndrome, systemic lupus erythematosus, ankylosing spondylitis, rheumatoid arthritis and IgG4 disease.1 4 6 8–13 However, MP has not been associated with inflammatory myopathies to the best of our knowledge. We report a case of MP incidentally discovered in a patient with new onset © BMJ Publishing Group dermatomyositis (DM). Limited 2020. No commercial re-use­ . See rights and permissions. Published by BMJ. Case presentation An- 83-­year old­ woman was referred to the rheuma- To cite: Bansal P, Gilbert EL, Pereira ROL, et al. BMJ Case tology department for a constellation of symptoms Rep 2020;13:e232183. including skin rash and muscle weakness. Medical Figure 1 83-year­ -­old Caucasian woman with a doi:10.1136/bcr-2019- history was significant for obesity, hyperlipidemia, pruritic erythematous rash on her upper torso in a 232183 hypertension, asthma, obstructive sleep apnea, photodistribution.

Bansal P, et al. BMJ Case Rep 2020;13:e232183. doi:10.1136/bcr-2019-232183 1 BMJ Case Rep: first published as 10.1136/bcr-2019-232183 on 2 February 2020. Downloaded from Unusual association of diseases/symptoms

Figure 2 4 mm punch biopsy of the chest. H&E stain demonstrates superficial dermal lymphohistiocytic inflammation.

Figure 4 4 mm punch biopsy of the chest. Low power (4×) colloidal Investigations iron stain demonstrates a prominent increase in dermal mucin. Initial investigations revealed elevation in creatine kinase at 998 U/L along with aldolase at 16 U/L. Serological work-­up was positive for ANA 4.4 U, anti-­Ds-­DNA 81.7 IU/mL, and weakly scan of the chest, abdomen and pelvis. Both the CT chest scan positive MI-2. Rest of the myomarker panel, and serological and pelvic ultrasound were unremarkable. A suspicious lesion work-­up including extractable nuclear antibodies panel was on mammography was biopsied and was negative for breast negative. C3 and C4 complement levels were normal, as were cancer. Colonoscopy showed multiple non-­malignant polyps the basic chemistries, thyroid and parathyroid hormone levels scattered throughout the large bowel. Leukaemia and lymphoma and serum protein electrophoresis. Electromyography and nerve work-­up were negative. CT scan of the abdomen/pelvis revealed by copyright. conduction study (EMG/NCS) showed low amplitude motor significant mesenteric stranding and thickening suggestive of responses and mixed motor unit potentials on needle examina- mesenteric panniculitis (figure 8). No evidence of underlying tion, suggestive of inflammatory myopathy. Right deltoid muscle malignancy was found.

biopsy showed active inflammatory myopathy with necrotic and http://casereports.bmj.com/ regenerating fibres in addition to perivascular inflammation and Outcome and follow-up invasion of vessel wall by mononuclear inflammatory cells indi- The patient was initiated on prednisone 40 mg daily and myco- cating a vasculitic component (figures 5–7). Overall, the patient’s phenolate mofetil 3 g daily with goal to taper the prednisone clinical presentation including physical examination, laboratory off to maintain steroid free treatment regimen for long-term­ findings, EMG/NCS and skin and muscle biopsies confirmed the management of the dermatomyositis. She started showing clin- diagnosis of dermatomyositis. Cancer screening was pursued ical improvement within 1 month, and prednisone taper was including pelvic ultrasound, colonoscopy, mammogram, serum initiated. After 3 months, she had improved dramatically with protein electrophoresis, leukaemia/lymphoma panel, and CT on March 30, 2020 at Weill Cornell Medical Library. Protected

Figure 5 Right deltoid muscle biopsy demonstrating active Figure 3 4 mm punch biopsy of the chest. Intermediate power inflammatory myopathy with a asculiticv component. Low power (2×) (10×) H&E stain demonstrates vacuolar interface inflammation with H&E stain demonstrates perivascular inflammatory exudate in the keratinocyte necrosis and thickening of the basement membrane. perimysium.

2 Bansal P, et al. BMJ Case Rep 2020;13:e232183. doi:10.1136/bcr-2019-232183 BMJ Case Rep: first published as 10.1136/bcr-2019-232183 on 2 February 2020. Downloaded from Unusual association of diseases/symptoms

Figure 6 Right deltoid muscle biopsy demonstrating active inflammatory myopathy with a asculiticv component. ATPase stain demonstrates type 2 fibre atrophy. Figure 8 Axial CT image of the abdomen with oral and intravenous contrast demonstrates moderate fat stranding within the abdomen at the level of (A) mid abdomen, (B) aortic bifurcation and (C) lower normal muscle examination and normal muscle enzymes. She was abdomen. continued on mycophenolate mofetil 3 g daily, and we continued tapering prednisone from 10 mg daily dose. Repeat CT scan of as the ‘misty mesentery’ is extensive, and other etiologies must the abdomen and pelvis was performed which showed signifi- 16 cant improvement in the mid abdominal mesenteric fat stranding be excluded before MP is considered by the radiologist. Non-­ and panniculitis (figure 9). Nodularity and adenopathy of the Hodgkin’s lymphoma is one mimic; others include mesenteric mesenteric fat had improved significantly as well. She had close oedema, focal inflammation (appendicitis or diverticulitis for follow-­up in the rheumatology clinic and internal medicine clinic example), other neoplasms and haemorrhage. It is worthy to by copyright. during this time, and no clinical, laboratory or imaging evidence note that many of these diagnoses present acutely and symptom- of malignancy was found on any of her follow-up­ visits as well. atically. In our patient, MP was incidentally discovered on CT scan of the abdomen/pelvis for malignancy surveillance given the underlying diagnosis of DM. This is not uncommon as one study Discussion http://casereports.bmj.com/ has indicated that only 8% of patients with CT findings of SM MP is a chronic inflammatory disorder with a reported inci- experienced symptoms related to the disorder.2 The spectrum of dence of 0.16%–3.4%.2 14 Radiographically, MP is defined by SM has been described as occurring during the course of auto- increased density of mesenteric fat tissue. Mass effect on loops immune disease. In a single case of limited systemic sclerosis, of small bowel may occur usually without disturbance of mesen- teric vascular structures. Other characteristic radiographic find- ings include ‘fat ring’ sign, tumorous pseudocapsule and soft tissue nodules.15 The differential diagnosis for alterations in mesenteric fat density on CT scan coined by Mindelzun et al on March 30, 2020 at Weill Cornell Medical Library. Protected

Figure 9 Corresponding images on follow-­up CT abdomen 3 months Figure 7 Right deltoid muscle biopsy demonstrating active after therapy showing significant reduction in mesenteric fat stranding inflammatory myopathy with a asculiticv component. H&E stain within the abdomen at the level of (A) mid abdomen, (B) aortic demonstrates perifascicular atrophy. bifurcation and (C) lower abdomen.

Bansal P, et al. BMJ Case Rep 2020;13:e232183. doi:10.1136/bcr-2019-232183 3 BMJ Case Rep: first published as 10.1136/bcr-2019-232183 on 2 February 2020. Downloaded from Unusual association of diseases/symptoms

MP was the presenting symptom.17 Our patient presented with This occurred despite persistence of SM findings on repeat CT many of the classic signs and symptoms of DM namely proximal imaging. Similar persistence of radiographic evidence of MP has muscle weakness of both the upper and lower extremities and been reported even 6 years after initial diagnosis.17 characteristic rash on her face and upper torso. However, she never experienced abdominal pain which is a typical symptom Learning points in patients with SM. The timeline of both these diagnoses in our case as well as radiographic improvement seen in the MP ►► Mesenteric panniculitis is a chronic inflammatory disorder. with treatment targeted at the DM suggest a direct association It is part of the spectrum known as sclerosing mesenteritis. between these two conditions and to our knowledge, no cases Many patients are asymptomatic or have non-­specific of MP have been reported in patients with DM. However, we symptoms such as fever, nausea, vomiting, abdominal pain acknowledge the limitations of a single case and further studies and weight loss. are needed to clarify direct association between MP and autoim- ►► For patients who are symptomatic, there are multiple mune diseases such as DM. treatment options including steroids, immunosuppressive There are multiple etiologies for MP including trauma, medications and hormonal therapies. There is no consensus surgery and chronic infection. In addition, MP has traditionally on a preferred regimen. been associated with malignancy, and retrospective studies have ►► Mesenteric panniculitis can have several underlying etiologies recommended that malignancy be ruled out in the case of inci- including trauma, surgery, infection, malignancy and dentally discovered MP.18 No evidence of solid organ or haema- connective tissue/autoimmune diseases as highlighted by our tologic malignancy was found in our patient’s initial screening case. It has likely been overdiagnosed in recent years based examinations as well as follow-up­ examinations. Our patient on imaging studies. received close and regular follow-up­ in the rheumatology and internal medicine clinics and no clinical, laboratory or imaging evidence of malignancy was found on any of her follow-up­ visits. Twitter Rodrigo Otavio Lami Pereira @LamiPereira A New Zealand study found that the most common sites of malig- Contributors PB: Direct patient care of the case involved, collection of data, final nancy associated with MP were the large bowel, lymph system review and approval of submission. ELG and ROLP: Collection of data, literature 19 review and writing the article. ARV: Patient care, pathology review and collection of and urogenital tract. As these are readily visualised on CT scan, data. they concluded that more in depth investigation beyond initial Funding The authors have not declared a specific grant for this research from any CT in asymptomatic patients is typically low yield. The potential funding agency in the public, commercial or not-­for-profit­ sectors. paraneoplastic phenomenon of MP has also been called in to Competing interests None declared. question by a retrospective study from New Zealand in which by copyright. rate of regression of MP was not different between those with Patient consent for publication Obtained. and without malignancy.20 Overall, these studies suggest that MP Provenance and peer review Not commissioned; externally peer reviewed. is perhaps often associated with malignancy due to the number ORCID iD of CT scans cancer patients undergo for staging. Case reports Emily L Gilbert http://orcid.​ ​org/0000-​ ​0003-2108-​ ​8315 http://casereports.bmj.com/ have also indicated an association between SM and connective tissue diseases. Sjögren’s syndrome, SLE, vasculitis, ankylosing spondylitis and limited systemic sclerosis have all been associated References with MP in case reports. A retrospective and prospective study at 1 Jura VS. Mesenterite E sclerosante. Policlinico 1924;31:575–81. Mayo Clinic identified 92 cases of sclerosing mesenteritis from 2 Daskalogiannaki M, Voloudaki A, Prassopoulos P, et al. Ct evaluation of 9 mesenteric panniculitis: prevalence and associated diseases. AJR Am J Roentgenol 1982 to 2005. From this cohort, nine patients had an associated 2000;174:427–31. rheumatologic condition with retroperitoneal fibrosis the most 3 Emory TS, Monihan JM, Carr NJ, et al. Sclerosing mesenteritis, mesenteric panniculitis common in four patients, followed by rheumatoid arthritis in and mesenteric lipodystrophy: a single entity? Am J Surg Pathol 1997;21:392–8. two patients. 4 Kipfer RE, Moertel CG, Dahlin DC. Mesenteric lipodystrophy. Ann Intern Med 1974;80:582–8. Whether or not SM requires treatment is a matter of debate. 5 Durst AL, Freund H, Rosenmann E, et al. Mesenteric panniculitis: review of the on March 30, 2020 at Weill Cornell Medical Library. Protected In the 92 patients at Mayo Clinic with SM, approximately 50% leterature and presentation of cases. Surgery 1977;81:203–11. of cases received some form of treatment.9 For symptomatic 6 Ogden WW, Bradburn DM, Rives JD. Mesenteric panniculitis: review of 27 cases. Ann patients, multiple treatment modalities have been explored Surg 1965;161:864–75. including surgery and medical therapy with steroids, immune 7 Kelly JK, Hwang W-S­ . Idiopathic retractile (sclerosing) mesenteritis and its differential diagnosis. Am J Surg Pathol 1989;13:513–21. modulators, anti-­inflammatories, hormonal and antifibrotic 8 Sharma P, Yadav S, Needham CM, et al. Sclerosing mesenteritis: a systematic review of 8 9 21 agents. Our patient received therapy targeted at her DM 192 cases. Clin J Gastroenterol 2017;10:103–11. with both steroids and mycophenolate mofetil with improve- 9 Akram S, Pardi DS, Schaffner JA, et al. Sclerosing mesenteritis: clinical features, ment in her symptoms of DM. Repeat CT scan of the abdomen treatment, and outcome in ninety-two­ patients. Clin Gastroenterol Hepatol 2007;5:589–96. and pelvis, performed after 3 months of treatment with pred- 10 Vlachos K, Archontovasilis F, Falidas E, et al. Sclerosing mesenteritis: diverse clinical nisone and mycophenolate mofetil, demonstrated improvement presentations and dissimilar treatment options. A case series and review of the in radiographic findings of panniculitis as well. Treatment algo- literature. Int Arch Med 2011;4:17. rithms for SM have been proposed based on whether or not the 11 Makdsi F, Brit M. A case of sclerosing mesenteritis with rheumatoid arthritis. South patient is symptomatic.9 Serial CT scans for follow-up­ are not Med J 2010;103:96–7. 12 Goyal A, Saul D, Annunziata G, et al. A self destructing reaction to a self destructive necessarily recommended as this treatment algorithm focuses on habit: levamisole tainted cocaine causing autoimmune vasculitis. New York Medical severity of symptoms guiding treatment. Serial CT scans may, Journal 2014;8:1. however, be pursued due to the association of SM with malig- 13 Sugihara T, Koike R, Nosaka Y, et al. A case of subcutaneous and mesenteric acute nancy. Case reports of rheumatoid arthritis patients with abdom- panniculitis with Sjoegren’s syndrome. Jpn.​ ​J.clin.​ ​Immun 2002;25:277–84. 14 Coulier B. Mesenteric panniculitis Part 2: prevalence and natural course: MDCT inal pain and radiographic evidence of SM reported improvement prospective study. JBR-BTR­ 2011;94:241–6. in abdominal symptoms following escalated doses of predni- 15 van Breda Vriesman AC, Schuttevaer HM, Coerkamp EG, et al. Mesenteric panniculitis: sone in conjunction with administration of methotrexate.10 11 US and CT features. Eur Radiol 2004;14:2242–8.

4 Bansal P, et al. BMJ Case Rep 2020;13:e232183. doi:10.1136/bcr-2019-232183 BMJ Case Rep: first published as 10.1136/bcr-2019-232183 on 2 February 2020. Downloaded from Unusual association of diseases/symptoms

16 Mindelzun RE, Jeffrey RB, Lane MJ, et al. The misty mesentery on CT: differential 19 Cross AJ, McCormick JJ, Griffin N, et al. Malignancy and mesenteric panniculitis. diagnosis. AJR Am J Roentgenol 1996;167:61–5. Colorectal Dis 2016;18:372–7. 17 Arroyo-Ávila M, Vilá LM. Limited systemic sclerosis initially presenting with mesenteric 20 Buchwald P, Diesing L, Dixon L, et al. Cohort study of mesenteric panniculitis and its panniculitis. BMJ Case Rep 2014;17. relationship to malignancy. Br J Surg 2016;103:1727–30. 18 Scheer F, Spunar P, Wiggermann P, et al. Mesenteric panniculitis (MP) in CT – a 21 Klasen J, Güller U, Muff B, et al. Treatment options for spontaneous and postoperative predictor of malignancy? Fortschr Röntgenstr 2016;188:926–32. sclerosing mesenteritis. World J Gastrointest Surg 2016;8:761–5.

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