P0166 PYODERMA GANGRENOSUM A DIFFERENTIAL DIAGNOSIS TO CONSIDER – CASE REPORT

Abstracts from 8th Congress of the European Federation of Internal Medicine / European Journal of Internal Medicine 20S (2009), S1–S283

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CO-EXISTENCE OF GOUGEROT SJÖGREN SYNDROME AND BEHÇET DISEASE: REPORT OF THREE CASES

LYMPH NODE AND PULMONARY SARCOIDOSIS IN A PORTUGUESE WOMAN WITH SILICONE BREAST PROSTHESES

Hamzaoui Amira, Harzallah Olfa, Klii Rim, Attig Chiraz, Mahjoub Silvia. Departement of Internal Medecine- Monastir- Tunisia

Mónica Caldeira, Filipe Perneta, Susana Chaves, Luís Freitas, Rubina Miranda, Rita Rodrigues, António Caldeira. Hospital Central Do Funchal

Introduction: Behçet’s disease (BD) is a multisystemic inflammatory vasculitic disorder with a chronic course for which the etiology is unknown. It is rarely associated with Gougerot Sjögren’s syndrome (SGS) which accompanies most autoimmune diseases. We report three cases of this association. Cases: There were 3 women with the mean age of 33, 66 years. The diagnosis of the BD was made according to the International Group Study on BD and of the SGS according to the European criteria. The diagnosis of BD precedes the SGS in the 3 cases. The BD was cutaneo-mucosal in 2 cases and associated to vascular involvement in the other case. The prominent features in the SGS were sicca syndrome. One patient had bilateral parotidis. Ther was no pulmonary nor neurological involvement. Conclusion: The SGS is more associated with connective disorder; however his association with vasculitis, such as BD, is possible but rare suggesting a chance association of 2 independent pathologies. Searching for sicca syndrome (Oral and ocular) is recommended in patient with BD.

P0166 PYODERMA GANGRENOSUM A DIFFERENTIAL DIAGNOSIS TO CONSIDER – CASE REPORT

João Freitas, João Gaspar, Margarida Jardim, Ana Silva, Dina Santos, Marta Pereira, Rita Vieira, Luz Brazão. Hospital Central Funchal Pyoderma gangrenosum (PG) is an uncommon ulcerative cutaneous condition of uncertain etiology. Systemic illnesses are seen in 50% of patients with PG and may occur prior to, concurrently or following the diagnosis. PG occurs in about 1 person per 100,000 people each year, with no racial predilection, slightly more predominant in women specially in the fourth and fifth decades of life. There are 2 primary variants of PG: a classic ulcerative form, usually observed on the legs, and a more superficial variant known as atypical PG that tends to occur on the hands. The case report presented involves a 74-year old woman, natural from Madeira that appeared in our Emergency Department with a three week appearance of small, red papule and pustule blisters that rapidly evolved into a vast loss of granulating cutaneous substance surrounded by an erythematous skin in the anterior region of the left leg associated with excursing pain. On admission the patient reported a brown recluse similar to an insect bite. Apart from that she also referred treatment with several antibiotics and topical oinks with no resolution. Her physical examination revealed a deep ulceration with a violaceus border that overhang the ulcer bed, surrounded by an erythematous skin in the anterior region of the left leg. The laboratorial evaluation disclosed: normocromic-normocytic anemia (Hb 10,9 g/dl; Htc 31,1%; VGM 81,6 fl), leukocytosis and neutrofilia (GB 39 500 103u/L; neutrofils 87%), PCR 485,2 mg/dl, CA 19.9- 41,7; negative serologic exams for hepatitis, HIV, Chlamydia and Treponema. Two microbiological swabs revealed pollution of the lesion by Pseudomonas aeruginosa, Staphilococcus aureus Methicillin resistant, Enterococcus faecalis and Streptococcus pyogenes for which the patient initiated proper antibiotics. An ecotomografy of the abdomen disclosed: “gallbladder lithiasis with moderate dilatation of intrahepatic ducts”. The thoraco-abdominal CT scan showed “two well defined round lesions located in the upper part of the right lung and lower part of the left lung – metastasis?” Other tests performed during hospitalization did not reveal any underlying systemic pathology. Oral administration of steroids prednisolone (1mg/kg/day) was accompanied by topical tacrolimus treatment after a high dose pulse IV methylprednisolone. After an ongoing recovery the patient was discharged and instructed to follow oral steroid therapy in scalar doses for a further two weeks (an initial 60 mg dose of prednisolone, progressively decreasing) accompanied by pain killers. The authors enhance two aspects: on one hand that a complete history should be taken with special focus on certain organs such as the heart, the central nervous system, kidney, the GI tract, the eyes, the liver, the spleen, bones, and lymph nodes; and on the other hand that the prognosis of PG is generally good, however, recurrences may occur and residual scarring is common.

Introduction: Sarcoidosis (SARC) is a multisystem granulomatous disease of uncertain aetiology. Patients present with bilateral hilar lymphadenopathy, pulmonary infiltration, ocular and skin lesions. Liver, spleen, lymph nodes, salivary glands, heart, nervous system, muscles, bone and other organs may be involved. Although the majority of sarcoid granulomas are non-caseating, necrosis has been documented (5-40%). Non-caseating granulomas are not specific for the diagnosis of SARC. They can occur in other conditions: infection, extrinsic allergic alveolitis, berylliosis, neoplasms, and adverse reactions (drugs, foreign materials). Silicone elastomer is one of the three common forms of silicone. It has been used in medical implants. Side-effects include local cutaneous inflammation and siliconomas of the breast plus regional lymphadenopathy (silicone granulomas). Remission of SARC following removal of silicone gel breast implants has been reported. Aims: Review the features of SARC and its association with silicone. Methods: Patient medical file review. Results: The authors report a clinical case of a 53 years old female patient, with a past medical history of right breast cancer submitted to surgery (T2N0M0) in 2001, followed by chemotherapy and radiotherapy. In remission since then, in 2003 she was submitted to breast reconstruction with silicone prosthesis. In 2007 she had an anterior uveitis. In 2008 she referred constitutional symptoms with loss of 5 kg in 3 months (7% of her body weight), extreme fatigue, tiredness and a left cervical node. Cervical Computed Tomography (CT) Scan: multiple cervical non-necrotizing lymphadenopathies. Node needle aspirate: non-necrotizing granulomatous lymphadenitis. Biopsy showed: caseating granulomatous lymphadenitis. Chest X-Ray was normal. She started anti-tuberculosis treatment empirically but stopped five months later due to poor response. At this time we couldn’t exclude breast cancer spread. Tumour markers were normal. Thoracic-and abdominal CT scan showed multiple lymphadenopathies with some micronodular images in the upper lobe of the right lung. Positron Emission Tomography Scan: multiple cervical, intrathoracic and intra-abdominal metastatic lymphadenopathies. Blood tests: elevated levels of ACE (90.3 U/L) in several measurements. A Bronchoalveolar Lavage (BAL) was made: CD4/CD8 ratio >3.5. Pulmonary function tests were normal. Gram’s and Ziehl-Neelsen stain, sputum culture and BAL for fungus and mycobacteria were negative. At this time we admitted a definite diagnosis of SARC and she was managed conservatively. Conclusions: The authors made this report to call our attention to this puzzling case. Earlier reports that silicone is biologically inert have recently been challenged, and the question has arisen as to whether silicone can cause systemic effects. As yet, this question has no answer.

P0168 OUT OF THE FRYING PAN INTO THE FIRE: MYOPATHY AS A CONSEQUENCE OF AN ALTERNATIVE PAIN THERAPY

Mine Durusu Tanrıöver, Mehmet Emin Kuyumcu, Gulay Sain Guven, Arzu Topeli. Hacettepe University Faculty of Medicine, Department of Internal Medicine Background: Myopathy may be due to several different entities. Complementary and alternative medicine (CAM) therapies should also be considered in the work-up. Aim: To define a potential side effect of a CAM modality and to review the differential diagnosis of myopathy. Method: A case of myopathy due to infrared heat and massage application is described. Result: A 66 year old male was seen in August 2008 because of swelling of the upper extremities and night sweating. Neck and bilateral arm movements were painful and limited, and he had a chronic lumbar pain. There was massive swelling, which was hard in consistency and non-pitting. The extremity movements were limited because of severe pain. When he had been evaluated in a rural hospital, serum ALT, AST and LDH levels were elevated. Imaging studies were normal. He was on 100 mg acetyl salicylic acid. The family history was positive for head and neck cancer. The laboratory tests are summarized in Table 1. There was no thrombus in the upper extremity venous system on Doppler ultrasonography, but hyperechogenicity compatible with edema of the skin and subcutaneous tissues was present. The neck, thorax and abdomen CT were normal.