Rheumatol Int (2011) 31:525–527 DOI 10.1007/s00296-009-1168-0
C A S E RE P O RT
Coexistent pyoderma gangrenosum and tibialis anterior myositis as presenting manifestations of Crohn’s disease: case report and review of the literature Oran Goldshmid · Zamir Dovorish · Tania Zehavi · Alon Eisen · Yaron Bar-Dayan · Howard Amital
Received: 29 July 2009 / Accepted: 20 September 2009 / Published online: 22 October 2009 © Springer-Verlag 2009
Abstract Crohn’s disease (CD) is a well deWned inXammatory bowel disease. Patients primarily present with abdominal pain and diarrhea, however, extra-intestinal manifestations due to musculoskeletal and cutaneous involvement are seen in a varying range of patients. In this communication we describe a young woman who presented with a severe inXammatory illness that consequently developed into pyoderma gangrenosum, anterior tibialis myositis and arthritis that were all antecedent to the intestinal involvement. The patient favorably responded to inXiximab with concomitant azathioprine therapy.
shown that 6% of EIM develop prior to the onset of IBD; the most common being arthritis (26%) and aphthous stomatitis (21%) [3]. In the following communication, we describe an unusual case of a patient with a severe, systemic form of inXammatory EIM of Crohn’s disease. The EIM preceded the clinical gastrointestinal signs and symptoms and were more prominent.
Keywords Crohn’s Disease · Myositis · Pyoderma gangrenosum · Arthritis · Erythema nodosum
A 24-year-old, white Jewish woman was admitted 5 days following the appearance of an erythematous swelling covering her right foot and shin. The initial suspected diagnosis was cellulitis, which was treated unsuccessfully with oral antibiotics. The patient’s medical history was uneventful. Except for oral contraceptives, she was not on any chronic medications. She had intentionally lost 6 kg over the past 2 months and denied abnormal bowel habits. Her temperature was 37.1°C and heart rate was 120 beats/min. There was a notable tender erythema nodosum lesion over the distal right shin and pitting edema over her lower right extremity accompanied by right ankle arthritis. Preliminary lab results showed a normal white blood cell count of 6,430 cells/mm3, a hemoglobin concentration of 12.2 gram/dl and a platelet count of 152,000 cells/mm3. She was sent for a leg sonography which excluded deep vein thrombosis but noted signiWcant subcutaneous edema. During the following 3 days her temperature increased to 38.3°C. The tenderness over her right foot and shin become excruciating and she found it increasingly diYcult to walk. She became extremely fatigued with a parallel and
CD and Ulcerative Colitis (UC) are chronic inXammatory bowel diseases (IBD) that often involve extra-intestinal organs. The most common organs involved are the skin, eyes, joints, biliary tract, and lungs [1]. Reported frequencies of extra-intestinal manifestations (EIM) range between 6 and 47%. Furthermore, it has been reported that the development of one EIM raises the odds developing another [1, 2]. A study of EIM in pediatric IBD patients has
O. Goldshmid · Z. Dovorish · A. Eisen · Y. Bar-Dayan · H. Amital (&) Department of Medicine ‘D’, Meir Medical Center, Kfar-Saba, Israel. AYliated to the Sackler Faculty of Medicine, Tel-Aviv University, Tshernichovsky 59, 44281 Kfar-Saba, Israel e-mail:
[email protected];
[email protected] T. Zehavi Department of Pathology, Meir Medical Center, Kfar-Saba, Israel. AYliated to the Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel
Patient description
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gradual decrease of her hemoglobin concentration to 8.5 gram/dl, requiring blood transfusions. A pyoderma gangrenosum (PG) lesion gradually developed on the mid-shin of her left leg (Fig. 1). Additional blood tests revealed a sedimentation rate of 110 mm/h, a C-reactive protein concentration of 43 mg/dL (0–0.3) and mildly elevated liver function tests. A MRI of the right leg displayed myositis of the right tibialis anterior muscle with bilateral fasciitis of the shin and thigh (Fig. 2). A concomitant rise of creatine phosphokinase to 330 units (N < 180) was recorded. Antibodies to a variety of infectious agents were negative as
Fig. 3 Severe acute and chronic inXammation within the colonic mucosa. Severe cryptitis is clearly seen (E-H 400£)
Fig. 1 Pyoderma gangrenosum appearing over the left leg. The center is ulcerative and gangrenous, with an erythematus rim
well as various autoantibodies. A chest and abdominal CT scan revealed small, bilateral pleural eVusions, no enlarged lymph nodes, and a mild splenomegaly with an intact and homogenous parenchyma. Due to the systemic Wndings, she also received an endoscopy which was normal and a colonoscopy in which pancolitis was seen. Biopsy demonstrated acute and chronic inXammation and cryptitis without granulomas (Fig. 3). Crohn’s Disease with extra-intestinal manifestation was diagnosed. The patient was put on 100 mg methyl-prednisolone daily with a rapid resolution of all clinical Wndings. An attempt to gradually decrease the dose resulted in a prompt relapse; therefore intravenous inXiximab (at a dose of 5 mg/ kg) was instituted with concomitant azathioprine. With this regimen, the patient is in a stable remission.
Discussion
Fig. 2 T1 MRI with gadolinium contrast demonstrating an inXammatory right tibialis anterior muscle. The muscle is encapsulated by an inXamed fascia (gray arrow), a focus of necrosis within the muscle is clearly seen (black arrow)
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We present a case of a patient who had an abrupt systemic presentation of multiple EIM, including myositis, fasciitis, arthritis, PG and erythema nodosum as the initial appearance of Crohn’s disease. Interestingly, the coexisting clinical expressions of the intestinal disease were minimal, despite intense inXammation shown in the biopsies. Both UC and CD are systemic inXammatory diseases that often involve extra-gastrointestinal organs. In general, EIMs tend to follow the clinical course of IBD, and their resolution often parallels that of the active IBD. However, some may not correlate with IBD disease activity. Among those, more commonly reported are primary sclerosing cholangitis and ankylosing spondylitis [1, 2]. The reported frequency of EIMs varies from 6 to 47% [1, 2, 4]. The development of one EIM appears to increase the
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susceptibility of developing others [2]. Some reports state that only 0.3% of patients develop multiple extra-intestinal diseases [4, 5]. It is very possible that the true rate of coexisting EIM is under reported and misdiagnosed due to the overlapping existence between lesions such as peripheral arthritis, erythema nodosum and PG. Nevertheless, it is evident that the longer the disease duration, the more likely that one or more EIM will be observed [1, 2]. Erythema nodosum is the most common skin manifestation in IBD. It aVects between 3 and 20% of IBD patients and has a female predominance [2–4]. The appearance of erythema nodosum characteristically parallels the intestinal disease activity and often occurs in acute Xares of IBD. It is usually self-limiting and tends to improve with treatment of the underlying bowel disease [2, 3]. PG is a less common skin manifestation of IBD, it appears in about 1% of patients with IBD. About 20% of patients with PG will develop IBD. It is more common in UC than in Crohn’s disease. One report described six patients with PG that appeared after the diagnosis of IBD [5]. PG usually appears while the concomitant IBD is active, yet it could also appear years before intestinal disease. PG is more severe in IBD; the correlation between bowel disease activity and the development of PG is variable [1–5]. Without treatment PG may last for years and the ulcers may spread [2]. The treatment of PG in IBD is diYcult as the treatment of intestinal disease usually does not suYce. Recent reports have suggested using inXiximab for PG associated with IBD [6]. Myositis and myopathy in patients with IBD are rare and have only been noted in case reports, so far. Myositis appears to be associated more often with CD rather than UC [7, 8]. The most commonly reported sites of myositis are the lower and upper limbs, which at times manifest together. Ocular muscle myositis has also been reported [8]. In our case, the MRI scan revealed myositis and fasciitis of the right tibialis anterior; fasciitis was detected in adjacent muscles as well. In relation to the underlying bowel disease activity, it appears that most cases of myositis occurred during a quiescent phase. Interestingly, all patients with myositis and CD presented primarily with colonic involvement [7, 8]. In their summary, Paoluzi et al. [8] described 19 cases in the
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medical literature of IBD in which myositis appeared. The majority of patients developed myositis years after the diagnosis of IBD; half after long-standing disease of more than 10 years. Nevertheless, in a few patients myositis heralded the onset of IBD as in the case reported here [8, 9].
Conclusion We report a rare case of a patient with CD who presented with an acute systemic illness with multiple concomitant EIMs preceding her bowel symptoms, among them myositis and PG. This case demonstrates that in IBD extra-intestinal manifestations may sometimes dominate the clinical picture, turning the diagnosis into a complex challenge. ConXict of interest statement
None.
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