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SWEET SYNDROME: CLINICAL REPORT
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Oral Communications / European Journal of Internal Medicine 19S (2008), S1–S59
She was readmitted 8 weeks later with another fall and mild confusion. She had a normocytic anaemia (Hb 9.6g/dL) and her corrected Calcium was 3.60mmol/L. In the view of normal PTH and normocytic normochromic anaemia, a bone marrow biopsy was performed, which showed some reactive changes but with no evidence of myeloma or lymphoma. Unexpectedly the bone marrow trephine biopsy showed multiple noncaseating granulomas, occupying about 80 percent of marrow. Neither acid fast bacilli nor fungi were detected. The angiotensin converting enzyme level at that point were found to be elevated 144U/L (normal, 0—60U/L). A diagnosis of Bone marrow sarcoidosis was made and she was started on prednisolone at 40mg/d. Serum calcium normalised in 6 days and her haemoglobin rose from 9.6mg/dl to 11.9mg/dl. Serum ACE level repeated in 4 weeks showed a fall to within the normal range with sustained normocalcemia. Discussion: This is an unusual presentation of hypercalcemia. The most common cause for an incidental finding of hypercalcemia in adults is Hyperparathyroidism. In her case, although initial ultrasound showed a parathyroid nodule, the PTH levels were normal. The finding of a breast nodule was another red herring. Bone scan however showed no hot spots and serum alkaline phosphatase was normal. These findings along with normal PTHrp levels excluded humoral hypercalcemia of malignancy as the cause. Normocytic anemia prompted a bone marrow biopsy; the findings of which, along with raised ACE levels confirmed the diagnosis. CT scan of the chest did not show any evidence of pulmonary involvement making this a case of isolated bone marrow sarcoidosis. Although bone marrow involvement is not uncommon in sarcoidosis it is exceedingly rare to have isolated bone marrow disease. Only about 10% of cases of sarcoidosis present with hypercalcemia and this again can be only transient, depending on disease activity. It is due to excess of 1, 25- (OH)2-D3 production from the alveolar macrophages and the sarcoid granuloma. After institution of prednisolone, there was prompt normalization of her calcium and ACE levels. Therefore sarcoidosis should be considered and investigated as a cause of hypercalcemia when the preliminary investigations are non-contributory.
TH-69 HAEMOPHILUS AORTITIS SECONDARY TO AORTIC VALVE ENDOCARDITIS S. Saraf, A.P. Worrall, A.M. Veitch. New Cross Hospital, Wolverhampton, United Kingdom An 86 yr old gentleman was admitted with confusion and fever. His past medical history included ischaemic heart disease, cerebrovascular disease, renal impairment, dementia and an infra-renal abdominal aortic aneurysm of 5.3 centimetres diameter, measured by ultrasound 2 years previously. This was treated conservatively, given his co-morbidity. On admission, he was pyrexial, hypotensive and tachycardic. Blood tests revealed a neutrophilia of 39.7×109 /l (normal range 2.5-7.5), serum C-reactive protein levels of 148 mg/l (1–6), alkaline phosphatase level of 283 IU/l (38–126). The initial diagnosis was possible cholangitis, and he was treated with Co-amoxiclav. An abdominal ultrasound revealed echogenic sludge within a distended gallbladder, but with no biliary dilatation. Further imaging was arranged to investigate other sources of sepsis. Computerised tomographic (CT) scanning of the abdomen revealed an 8 cm infrarenal aortic aneurysm with a tiny pocket of air in the anterior wall which is a unique radiographic finding of an infected aneurysm. An echocardiogram demonstrated a 3mm diameter vegetation on the left coronary cusp of the aortic valve. Haemophilus influenzae was isolated from blood-cultures and the diagnosis revised to Haemophilus aortitis secondary to aortic valve endocarditis. This was treated with Co-amoxiclav and Gentamycin. He died as a result of his sepsis despite appropriate anti-microbial therapy.Following the advent of antibiotics, secondary aortitis has become a rare complication of native valve endocarditis, and we are aware of only one reported case of haemophilus aortitis in the recent literature.
TH-70 SWEET SYNDROME: CLINICAL REPORT M. Teodoro, E. André, N Seabra. Centro Hospitalar Cova Beira-hospital do Fundão Introduction/Background: Acute febrile neutrophilic dermatose is an uncommon skin disease characterized by fever, neutrophilia, painful red papules,
nodules or plaques and an infiltrate consisting predominantly of mature neutrophils diffusely distributed in the upper dermis. Extra-cutaneous manifestations may be present. Based on clinical setting Sweet Syndrome (SS) is classified as idiopathic, malignancy-associated or drug induced. The idiopathic form usually affects women in the fourth and fifth decades. Case description: a 80-year-old man was admitted to our hospital with a 3 weeks history of high fever (39°C), malaise and anorexia. Besides fever, physical exam was irrelevant. Laboratory data showed: anemia, neutrophilia, monocytosis and high ESR. Diagnostic workup included cultures, serologic tests, imagiologic exams and temporal biopsy all negative. A small mitral regurgitation was found. One week after admission several painful nodules in the palmar face of both hands, reminding Osler nodules were found along with a new heart murmur that revealed to be due to a severe mitral regurgitation. In spite of no vegetations were seen, the patient started antibiotic therapy without clinical improvement. Skin biopsy showed a neutrophilic infiltration of the dermis. He then started corticotherapy with rapid clinical and laboratory improvement. 3 and 6 weeks the valvular function was markedly improved. Exams to rule out malignancy were negative. Conclusion: Cases of cardiac valvular regurgitation due to acute valvitis are described in SS; this possibility can‘t be rule out in this patient, given the significant improvement in valvular function with corticoids; as the possibility of malignancy remains, we keep a close fallow-up specially in older patients
TH-71 ADDISON‘S DISEASE: A CLINICAL CASE M. Teodoro, J.L. Temprano, E. André, N Seabra. Centro Hospitalar Cova da Beira- Medicina Fundão Introduction/Background: Addison‘s disease is an uncommon endocrine disorder due to primary adrenal failure. Autoimmune destruction of the adrenal cortex accounts for more than 80% of the reported cases. Presentation is often insidious and non specific therefore the diagnosis at an early stage is easily missed. Case description: A 23-year-old female, with a 4 months history of progressive weakness, poor appetite, depressed mood and sleep disturbances was diagnosed as having a major depression by a psychiatry. She started oral antidepressants without clinical improvement. So she was admitted to our hospital to perform intravenous tricyclic therapy. Physical examination revealed increase skin pigmentation, hypotension and orthostasis. Routine laboratory studies showed a low serum sodium (115 mmol/L) strengthen the index of suspicion of adrenal insufficiency. Serum cortisol level was also very low (2,05 mg/dL); ACTH was 451 pg/mL. The ACTH stimulation test confirmed the diagnosis. Abdominal CT and MRI were performed with normal results. Mantoux was negative. Only 21-hidroxilase autoantibody was positive in autoantibodies screening for type II poliglandular autoimmune syndrome. The patient started corticoid therapy with rapid clinic improvement. Discussion/Conclusion: With this case we would like to emphasize the diagnostic challenge offer by a rare condition whose unspecific presentation mixes the diagnoses, making it possible only in situations where the death risk is considerably high like in acute adrenal crises. This is highly probably when the first symptoms are psychiatric given the prevalence of depression nowadays.
TH-72 CELIAC DISEASE ASSOCIATED WITH SJÖGREN’S SYNDROME AND SYSTEMIC LUPUS ERYTEMATOUS. A CASE REPORT O. Ben Aballah, F. Ben Fredj, B. Mrad, I. Ben Mansour, S. Toumi, H. Mhiri, C Laouani Kechrid. Department of Internal Medecine, Sahloul Hospital, Sousse, Tunisia Introduction: Celiac disease is an autoimmune disease due to a hyper sensibility to gluten. It may occur with other autoimmune diseases. However the association with systemic lupus erythematosus (SLE) and Sjögren’s syndrome is infrequent. We report a new observation. Case report: We report the case of a 31 years old woman who had a celiac disease since 7 years old with a poor compliance for a gluten free diet, referred to our consultation for xerostomia, xerophtalmia and arthralgia. A diagnosis of Sjögren’s syndrome was made supported by an abnormal schirmer’s test, a labial minor salivary gland biopsy (Chisholm III) and anti SSA, anti SSB antibodies presence. She presented a recent photo sensibility and mouth ulceration. We found, in the biology, an anemia, leucopenia and lymphopenia, proteinuria, low complement and anti DNA antibodies.
Oral Communications / European Journal of Internal Medicine 19S (2008), S1–S59
She was readmitted 8 weeks later with another fall and mild confusion. She had a normocytic anaemia (Hb 9.6g/dL) and her corrected Calcium was 3.60mmol/L. In the view of normal PTH and normocytic normochromic anaemia, a bone marrow biopsy was performed, which showed some reactive changes but with no evidence of myeloma or lymphoma. Unexpectedly the bone marrow trephine biopsy showed multiple noncaseating granulomas, occupying about 80 percent of marrow. Neither acid fast bacilli nor fungi were detected. The angiotensin converting enzyme level at that point were found to be elevated 144U/L (normal, 0—60U/L). A diagnosis of Bone marrow sarcoidosis was made and she was started on prednisolone at 40mg/d. Serum calcium normalised in 6 days and her haemoglobin rose from 9.6mg/dl to 11.9mg/dl. Serum ACE level repeated in 4 weeks showed a fall to within the normal range with sustained normocalcemia. Discussion: This is an unusual presentation of hypercalcemia. The most common cause for an incidental finding of hypercalcemia in adults is Hyperparathyroidism. In her case, although initial ultrasound showed a parathyroid nodule, the PTH levels were normal. The finding of a breast nodule was another red herring. Bone scan however showed no hot spots and serum alkaline phosphatase was normal. These findings along with normal PTHrp levels excluded humoral hypercalcemia of malignancy as the cause. Normocytic anemia prompted a bone marrow biopsy; the findings of which, along with raised ACE levels confirmed the diagnosis. CT scan of the chest did not show any evidence of pulmonary involvement making this a case of isolated bone marrow sarcoidosis. Although bone marrow involvement is not uncommon in sarcoidosis it is exceedingly rare to have isolated bone marrow disease. Only about 10% of cases of sarcoidosis present with hypercalcemia and this again can be only transient, depending on disease activity. It is due to excess of 1, 25- (OH)2-D3 production from the alveolar macrophages and the sarcoid granuloma. After institution of prednisolone, there was prompt normalization of her calcium and ACE levels. Therefore sarcoidosis should be considered and investigated as a cause of hypercalcemia when the preliminary investigations are non-contributory.
TH-69 HAEMOPHILUS AORTITIS SECONDARY TO AORTIC VALVE ENDOCARDITIS S. Saraf, A.P. Worrall, A.M. Veitch. New Cross Hospital, Wolverhampton, United Kingdom An 86 yr old gentleman was admitted with confusion and fever. His past medical history included ischaemic heart disease, cerebrovascular disease, renal impairment, dementia and an infra-renal abdominal aortic aneurysm of 5.3 centimetres diameter, measured by ultrasound 2 years previously. This was treated conservatively, given his co-morbidity. On admission, he was pyrexial, hypotensive and tachycardic. Blood tests revealed a neutrophilia of 39.7×109 /l (normal range 2.5-7.5), serum C-reactive protein levels of 148 mg/l (1–6), alkaline phosphatase level of 283 IU/l (38–126). The initial diagnosis was possible cholangitis, and he was treated with Co-amoxiclav. An abdominal ultrasound revealed echogenic sludge within a distended gallbladder, but with no biliary dilatation. Further imaging was arranged to investigate other sources of sepsis. Computerised tomographic (CT) scanning of the abdomen revealed an 8 cm infrarenal aortic aneurysm with a tiny pocket of air in the anterior wall which is a unique radiographic finding of an infected aneurysm. An echocardiogram demonstrated a 3mm diameter vegetation on the left coronary cusp of the aortic valve. Haemophilus influenzae was isolated from blood-cultures and the diagnosis revised to Haemophilus aortitis secondary to aortic valve endocarditis. This was treated with Co-amoxiclav and Gentamycin. He died as a result of his sepsis despite appropriate anti-microbial therapy.Following the advent of antibiotics, secondary aortitis has become a rare complication of native valve endocarditis, and we are aware of only one reported case of haemophilus aortitis in the recent literature.
TH-70 SWEET SYNDROME: CLINICAL REPORT M. Teodoro, E. André, N Seabra. Centro Hospitalar Cova Beira-hospital do Fundão Introduction/Background: Acute febrile neutrophilic dermatose is an uncommon skin disease characterized by fever, neutrophilia, painful red papules,
nodules or plaques and an infiltrate consisting predominantly of mature neutrophils diffusely distributed in the upper dermis. Extra-cutaneous manifestations may be present. Based on clinical setting Sweet Syndrome (SS) is classified as idiopathic, malignancy-associated or drug induced. The idiopathic form usually affects women in the fourth and fifth decades. Case description: a 80-year-old man was admitted to our hospital with a 3 weeks history of high fever (39°C), malaise and anorexia. Besides fever, physical exam was irrelevant. Laboratory data showed: anemia, neutrophilia, monocytosis and high ESR. Diagnostic workup included cultures, serologic tests, imagiologic exams and temporal biopsy all negative. A small mitral regurgitation was found. One week after admission several painful nodules in the palmar face of both hands, reminding Osler nodules were found along with a new heart murmur that revealed to be due to a severe mitral regurgitation. In spite of no vegetations were seen, the patient started antibiotic therapy without clinical improvement. Skin biopsy showed a neutrophilic infiltration of the dermis. He then started corticotherapy with rapid clinical and laboratory improvement. 3 and 6 weeks the valvular function was markedly improved. Exams to rule out malignancy were negative. Conclusion: Cases of cardiac valvular regurgitation due to acute valvitis are described in SS; this possibility can‘t be rule out in this patient, given the significant improvement in valvular function with corticoids; as the possibility of malignancy remains, we keep a close fallow-up specially in older patients
TH-71 ADDISON‘S DISEASE: A CLINICAL CASE M. Teodoro, J.L. Temprano, E. André, N Seabra. Centro Hospitalar Cova da Beira- Medicina Fundão Introduction/Background: Addison‘s disease is an uncommon endocrine disorder due to primary adrenal failure. Autoimmune destruction of the adrenal cortex accounts for more than 80% of the reported cases. Presentation is often insidious and non specific therefore the diagnosis at an early stage is easily missed. Case description: A 23-year-old female, with a 4 months history of progressive weakness, poor appetite, depressed mood and sleep disturbances was diagnosed as having a major depression by a psychiatry. She started oral antidepressants without clinical improvement. So she was admitted to our hospital to perform intravenous tricyclic therapy. Physical examination revealed increase skin pigmentation, hypotension and orthostasis. Routine laboratory studies showed a low serum sodium (115 mmol/L) strengthen the index of suspicion of adrenal insufficiency. Serum cortisol level was also very low (2,05 mg/dL); ACTH was 451 pg/mL. The ACTH stimulation test confirmed the diagnosis. Abdominal CT and MRI were performed with normal results. Mantoux was negative. Only 21-hidroxilase autoantibody was positive in autoantibodies screening for type II poliglandular autoimmune syndrome. The patient started corticoid therapy with rapid clinic improvement. Discussion/Conclusion: With this case we would like to emphasize the diagnostic challenge offer by a rare condition whose unspecific presentation mixes the diagnoses, making it possible only in situations where the death risk is considerably high like in acute adrenal crises. This is highly probably when the first symptoms are psychiatric given the prevalence of depression nowadays.
TH-72 CELIAC DISEASE ASSOCIATED WITH SJÖGREN’S SYNDROME AND SYSTEMIC LUPUS ERYTEMATOUS. A CASE REPORT O. Ben Aballah, F. Ben Fredj, B. Mrad, I. Ben Mansour, S. Toumi, H. Mhiri, C Laouani Kechrid. Department of Internal Medecine, Sahloul Hospital, Sousse, Tunisia Introduction: Celiac disease is an autoimmune disease due to a hyper sensibility to gluten. It may occur with other autoimmune diseases. However the association with systemic lupus erythematosus (SLE) and Sjögren’s syndrome is infrequent. We report a new observation. Case report: We report the case of a 31 years old woman who had a celiac disease since 7 years old with a poor compliance for a gluten free diet, referred to our consultation for xerostomia, xerophtalmia and arthralgia. A diagnosis of Sjögren’s syndrome was made supported by an abnormal schirmer’s test, a labial minor salivary gland biopsy (Chisholm III) and anti SSA, anti SSB antibodies presence. She presented a recent photo sensibility and mouth ulceration. We found, in the biology, an anemia, leucopenia and lymphopenia, proteinuria, low complement and anti DNA antibodies.