Pure cutaneous lupus erythematosus in a population of African descent in French Guiana: a retrospective population-based description.

The objective of this study was to examine the characteristics of cutaneous lupus erythematosus, excluding systemic lupus erythematosus (SLE), in patients of African descent. Indeed, since the description of subacute cutaneous lupus erythematosus (SCLE), which had been included in chronic cutaneous lupus erythematosus (CCLE), there has been no description of the disease in black patients. In 2000, we performed a retrospective epidemiological study by querying multiple sources to identify all patients with lupus in French Guiana--a part of France in South America having western living conditions, free healthcare and 157,000 inhabitants, most of whom are of African origin. We found 45 patients with pure cutaneous lupus, which included CCLE (mostly discoid), SCLE and bullous lupus. The disease characteristics of these patients exhibited few differences compared with those of the Caucasian patients cited in the literature. However, the age of onset for our patients of African descent was younger than that of Caucasian patients. In contrast to the race-related differences reported for SLE, we found no major differences in terms of demographic, clinical and biological presentation between this cohort of pure cutaneous lupus erythematosus patients of African origin and Caucasian patients with similar forms of lupus.

Lytic bone lesions in lupus-associated myelofibrosis.

Myelofibrosis is associated with numerous causes other than hematologic malignant neoplasms, including autoimmune diseases. We describe a 44-year-old woman who suffered bone lytic lesions with extramedullary haematopoiesis in the setting of myelofibrosis associated with systemic lupus erythematosus.

[Diabetic muscular necrosis: three Afrocaribbean cases]. / Nécrose musculaire du diabétique : trois cas Afro-caribéens.

INTRODUCTION: Diabetic muscular infarction is an exceptional complication of diabetes mellitus. Patients with macro- and microvascular manifestations are mostly concerned. Muscular pain with swelling of the thigh is the main manifestation, sometimes associated with fever. OBSERVATIONS: We report here five cases of diabetic muscular infarction in three Afro-carribean patients with atypical clinical features. The surgical biopsy procedure allowed the diagnosis and ruled out pyomyositis, a more common bacterial disease on tropical condition. CONCLUSION: This report of three cases of this exceptional disease in a small population seems more related to the high prevalence of diabetes mellitus in the French West Indies than to their ethnic origin.

Isolated conjunctival ulcerations as the first sign of Behçet's disease.

PURPOSE: To report an unusual incident of isolated conjunctival ulcerations which preceded all other signs of Behçet's disease. METHODS: Description of a 34-year-old woman of West Indian origin with an unremarkable medical history presenting with bilateral bulbar conjunctival ulcerations. RESULTS: The authors noted the presence of a diffuse bilateral conjunctival hyperemia which was more pronounced at the temporal bulbar conjunctiva of the right eye and the nasal conjunctiva of the left eye, each having one ulceration. The rest of the ophthalmologic examination was normal. One month later, the appearance of buccogenital aphthosis led to the diagnosis of Behçet's disease. CONCLUSIONS: This observation is unusual, as the conjunctival ulcerations are isolated and precede the onset of all other symptoms of Behçet's disease. Conjunctival ulcerations are rarely seen with Behçet's disease, but are characteristic enough to be included among the diagnostic criteria.

Refractory multicentric reticulohistiocytosis treated by infliximab: two cases.

We report the effect of infliximab, a monoclonal anti-TNFalpha antibody, in two patients with refractory cutaneous and articular multicentric reticulohistiocytosis (MRH). One 37-year-old woman and one 53-year-old woman with polyarthritis, facial rash and nodular lesions on the hands related to MRH were refractory to multiple agents: cariolysine, corticosteroids, hydroxychloroquine and cytotoxic agents. Infliximab at 3 mg/kg which was then increased to 5 mg/kg in combination with methotrexate or azathioprine was effective on cutaneous manifestations of the disease but not on polyarthritis. A switch to etanercept did not improve polyarthritis in the second patient. Some data suggest that TNFalpha is involved in MRH, but based on our cases anti-TNFalpha therapy needs further evaluation in patients with refractory MRH.

Familial lupus erythematosus. Clinical and immunologic features of 125 multiplex families.

Evidence for a genetic susceptibility to systemic lupus erythematosus (SLE) in humans is based on the high concordance rate observed in identical twins and on the relatively high incidence of familial cases. Although recent genetic studies have lead to significant advances in the identification of new susceptibility genes in SLE, no large clinico-pathologic study of familial SLE has been reported to date. In the present study, we describe the main clinical and immunologic features of 125 lupus multiplex families including at least 2 cases of SLE and/or discoid lupus erythematosus (DLE), recruited through a French national survey starting in July 1997. Medical records of all affected members were reviewed by the same investigator, all available family members were interviewed using the same standardized procedure, and blood was drawn for autoantibodies typing. Clinical and immunologic features of 90 probands from multiplex SLE families were compared with those of 100 sporadic SLE patients sharing the same French Caucasian origin. The 125 lupus multiplex families included 282 affected members (2.3 patients per family); of the 125 families, 96 were of French Caucasian origin. One hundred multiplex families included 2 affected relatives, while 25 included 3 or more affected individuals. The relationship between affected members was sibs (45%), parent-offspring (31%), and second-degree (24%). An autosomal dominant mode of inheritance was strongly suggested in 1 extended pedigree with 6 clinically affected members, and a recessive pattern was suspected in 5 other families. No obvious mode of inheritance could be suspected in most of the remainder. Among French Caucasians, sex ratio, mean age at onset, and clinical and biologic SLE-related manifestations were not significantly different in multiplex compared with sporadic SLE cases. The analysis of these 125 multiplex families suggests a genetic heterogeneity that should be considered for ongoing genomic screening.

Six cases of cervical ligamentum flavum calcification in Blacks in the French West Indies.

OBJECTIVE: Calcification of the ligamentum flavum at the cervical spine is an uncommon condition reported mainly in Japanese patients. We describe the clinical manifestations, imaging study findings, and outcomes in six cases seen in the French West Indies. MATERIAL AND METHODS: We retrospectively reviewed the medical charts of six patients admitted to an orthopedics department for spinal cord compression shown upon computed tomography to be caused by calcification of the ligamentu flavum. There were five women and one man, mean age at admission was 71.7 years (range, 64-79 years) and all six patients were Black. RESULTS: Five patients had cervical myelopathy and one was asymptomatic. All five symptomatic patients had cervical spinal stenosis, explaining the rapid symptom onset (within six and a half months) and severe motor loss. Computed tomography reconstruction in the sagittal plane ruled out ossification of the ligamentum flavum. Magnetic resonance imaging of the neck failed to demonstrate the calcifications but was useful in evaluating the severity of the spinal cord compression. One patient had articular chondrocalcinosis in both knees and another had calcifications in the basal ganglia. Surgical decompression by the posterior route was performed in two patients and was effective in both, whereas two of the three symptomatic patients who did not have surgery experienced worsening neurological loss. Analysis of the operative specimens from the two surgically treated patients showed a mixture of calcium pyrophosphate dihydrate crystals and apatite microcrystals. CONCLUSION: Calcification of the ligamentum flavum is probably underrecognized in blacks. This condition causes severe neurological loss. Imaging studies provide the diagnosis. The pathogenesis remains unclear.

[Treatment of corticodependent systemic lupus erythematosus with low-dose methotrexate]. / Traitement du lupus érythémateux systémique corticodépendant par le méthotrexate à faibles doses.

The authors report an open prospective study in a group of 16 patients who presented systemic lupus erythematosus with cutaneous and articular symptoms, and who required treatment with a minimum dose of 15 mg per day of prednisone. Methotrexate was given at a dose of 7.5 mg IM per week. Efficacy was demonstrated at the third month with a statistical analysis of four evolution parameters. Improvement was observed in 13 patients out of 16 and permitted the reduction of the amount of prednisone required. Secondary relapse occurred in four cases in spite of an increase in the dose of methotrexate (10 mg per week). Minor side effects were observed in four cases, and methotrexate was discontinued in only two cases. This study suggests that treatment by low doses of methotrexate is beneficial to patients with articular and cutaneous manifestations of corticodependent systemic lupus erythematosus and this therapy could be corticosteroid-sparing.

[Acute disseminated encephalomyelitis preceding cutaneous lupus]. / Encéphalomyélite aiguë disséminée précédant un lupus cutané.

STUDY DESIGN: A case of acute disseminated encephalomyelitis (ADEM) preceding the features of cutaneous lupus is reported. OBJECTIVES: To suggest that ADEM and cutaneous lupus are pathophysiologically related. SUMMARY OF THE BACKGROUND DATA: Neurological complications of systemic lupus erythematosus are common. However, demyelinative central nervous system manifestations are rare, and restricted to neuromyelitis optica (NMO). NMO is thought to be a partial form of ADEM. METHODS: A clinical, neuroradiological and immunological study was performed in this case. RESULTS: ADEM was diagnosed in a young black female. Three years later, she developed a cutaneous lupus without hypocomplementemia. CONCLUSION: A relationship between ADEM and cutaneous lupus is suggested. The normal serum complement level in our patient might be a predisposing factor for the development of ADEM.

[Sarcoidosis and leukemia/T-cell lymphoma associated with HTLV-1 virus infection in adults (apropos of a case)]. / Sarcoïdose et leucémie/lymphome T de l'adulte associées au virus HTLV-I (à propos d'une observation).

The HTLV-1 virus causes a disturbance of the immune system, the evaluation of which is often difficult. We report a case of sarcoidosis in a 49 year old woman of Martinique as evidenced by bilateral hilar adenopathy, hypercalcaemia, uveitis and granulomatous lesions on histological examination. Serological was positive for HTLV-1 antibodies. Three years later she developed an adult T-cell leukemia/lymphoma. The relationships between the HTLV-1 retroviral infection and different pathologies observed are discussed.

[Systemic lupus erythematosus in Martinique: an epidemiologic study]. / Lupus systémique en Martinique: enquête épidémiologique.

PURPOSE: To review epidemiological and clinical aspects of systemic lupus erythematosus (SLE) in Martinique, French West Indies. METHODS: Cases of SLE were identified by attending physicians. Patients who presented with at least four of the criteria defined by the American College of Rheumatology were included. Determination of incidence and prevalence included the new cases arising during the 1990-1999 period and 1999 population census results. Probability of survival was based on the use of the Kaplan-Meier estimator. RESULTS: Two hundred and eighty-six patients were studied, including 265 females (92.7%). The average annual incidence was 4.7 cases per 100,000 inhabitants (95% confidence interval [CI]: 2.5-6.9). The prevalence for 1999 was 64.2 cases per 100,000 inhabitants (CI: 56.2-72.2). The mean age at onset was 30 years. Eleven percent of all patients had at least one parent with SLE. Renal disease was present in 139 patients (48.6%), and neurological disorders were diagnosed in 70 patients (24.5%). Patients tested positive for the following antibodies: anti-Sm (37.1%), anti-RNP (58.7%), anti-SSA (47.2%). Mean survival time was: 96.4% (CI: 94.1-98.7) at 5 years, 91.8% (CI: 87.9-95.7) at 10 years. Survival was significantly reduced in patients with end-stage renal disease (n = 40, chi 2 = 6.96, P < 0.01). CONCLUSION: The high incidence of SLE in Martinique and the immunological characteristics of patients were found to be similar to those described in other populations of African descent. The frequency of renal disease and survival rates were identical to those reported in Caucasians.

[Blount's disease in the Antilles. Apropos of 26 cases]. / La maladie de Blount aux Antilles. A propos de 26 observations.

Blount's disease is associated with agenesis of the medial tibial plateau leading to tibia vara. It appears to be more frequent in Scandinavian countries and in black populations. Twenty-six cases had been seen in Martinique (Antilles) of whom twenty were children. A classification into six stages was described. Stage IV is a critical one. Before it, corrective osteotomy will often lead to complete healing. After stage IV, lateral epiphysiodesis must be added to avoid recurrence of deformity. The technique of osteotomy was variable according to the age of the child and the obliquity of the joint line. In young children, a subtraction closing wedge osteotomy is suitable. In older children a "V" shaped osteotomy is recommended to lessen the amount of shortening. At the end of the growth period, a medial opening wedge osteotomy is advisable. In adults (six cases) whose deformity can reach as much as 50 degrees with considerable ligamentous laxity, reefing of the ligament must be added to the osteotomy. The problem of the opposite knee is discussed. Even when the disease is not bilateral, it can tend to develop towards arthrosis.

[Capillary hemangioma of the spinal cord. A new case]. / Hémangiome capillaire médullaire. Un nouveau cas.

A 49-year-old man presented with a 8-month history of gait and sphincter disturbances. Examination revealed a spastic paraplegia predominant on the left limb, associated with decreased tactile sensitivity below T10. MRI showed a right posterolateral intramedullary lesion with an isosignal on T1-weighted images and a mild hypersignal on T2-weighted images, with an intense contrast enhancement; the spinal cord presented with an hypersignal on T2-weighted images. At operation, a vascular tumor presented at the spinal cord surface, was well demarcated from the parenchyma and was easily removed. Microscopic examination showed a capillary hemangioma. Three months later the patient exhibited a motor improvement with a right spasticity, deficits in proprioception, without sphincter abnormalities. A new MRI showed the disappearance of the hemangioma and of the preoperative spinal cord hypersignal. Capillary hemangiomas are unusual on nerves or roots. Cases found in the spinal cord are extremely rare: to our knowledge, 5 cases have been reported. On MRI, they are easily distinguished from cavernous hemangiomas, but microscopic examination is necessary to distinguish them from hemangioreticulomas. The postoperative prognosis is generally good. However, a report of a multiple location case, on roots and spinal cord, suggests the need for long follow-up.

[Hypercalcemia of T-cell leukemia in adults]. / Les hypercalcémies des leucémies à lymphocytes T de l'adulte.

A retrospective study of 26 adults with acute T-cell leukemia showed that 14 patients (54%) had hypercalcemia at some point of the disease. Hypercalcemia was found at presentation in nine patients and revealed the disease in one. Eight patients had hypercalcemia at the time of death. Serum phosphorus and parathyroid hormone levels were normal. All patients with hypercalcemia tested positive for the HTLV-1 by Elisa and Western blot. Six patients had focalized or diffuse lytic roentgenographic bone lesions. Hypercalcemia in acute T-cell leukemia may involve production of interleukin-1-alpha and parathyroid hormone-related protein by HTLV-1-infected cells.

Is African descent an independent risk factor of peripartum cardiomyopathy?

Risk factors for peripartum cardiomyopathy (PPCM) are controversial. PPCM seems to be more prevalent in women of African descent, the highest observed incidence is in Haiti (1 per 300 live births). Our retrospective study conducted in Martinique showed an incidence of 1 per 5500 live births. This incidence is significantly lower than in Haiti. Women from Martinique and Haiti do not differ for most classical risk factors: African descent, age, pregnancy-associated hypertension, multiple pregnancy and pre-eclampsia. However, the parity rate and the socioeconomic level are different. Thus, African descent could be confounded by high parity rate and socioeconomic status.

Characteristics and outcome of connective tissue diseases in patients with sickle-cell disease: report of 30 cases.

OBJECTIVES: To analyze the main characteristics of adults with sickle cell disease (SCD) and concurrent connective tissue disease (CTD). METHODS: A retrospective investigational study was performed. CTD was diagnosed according to standard international criteria. Severity of SCD was assessed by a clinical severity score. RESULTS: Thirty patients, 23 women (76%) and 7 men, with hemoglobin S/S (n = 25) or S/C (n = 5) SCD were included. The subtypes of CTD were rheumatoid arthritis (RA) (n = 15), definite systemic lupus erythematosus or "incomplete lupus" requiring treatment (n = 13), primary Sjögren's syndrome with central nervous system involvement (n = 1), and systemic sclerosis (n = 1). Twenty-five of the 30 patients (83%) received steroid treatment, and 15 (50%) received at least 1 immunosuppressive agent (methotrexate in 14 cases) to control CTD. Four RA patients were given antitumor necrosis factor (TNF)alpha and 1 was treated with rituximab without SCD exacerbation. After a median follow-up of 4.5 years [range: 6 months to 30 years] from CTD diagnosis, 11 of the 25 (44%) patients receiving steroids had at least 1 episode of severe infection (mostly due to Staphylococcus aureus or Escherichia coli). SCD exacerbated in 13 of the 30 (43%) patients after CTD onset; 12 of these patients were receiving prednisone and/or methotrexate. Six patients (20%) had died from sepsis (n = 2), stroke (n = 2), or acute chest syndrome (n = 2). CONCLUSIONS: CTD-related clinical manifestations and outcome were not particularly severe in patients with SCD. However, those with active CTD and undergoing steroid +/- methotrexate treatment had more serious SCD-related manifestations, a higher rate of severe infections, and an overall patient mortality rate of 20%. Thus, the management of patients with CTD and underlying SCD should consider the risk/benefit ratio of each treatment and steroid-sparing strategies should be implemented.

Isolated conjunctival ulcerations as the first sign of Behets disease.

PURPOSE: To report an unusual incident of isolated conjunctival ulcerations which preceded all other signs of Behets disease. METHODS: Description of a 34-year-old woman of West Indian origin with an unremarkable medical history presenting with bilateral bulbar conjunctival ulcerations. RESULTS: The authors noted the presence of a diffuse bilateral conjunctival hyperemia which was more pronounced at the temporal bulbar conjunctiva of the right eye and the nasal conjunctiva of the left eye, each having one ulceration. The rest of the ophthalmologic examination was normal. One month later, the appearance of buccogenital aphthosis led to the diagnosis of Behets disease. CONCLUSIONS: This observation is unusual, as the conjunctival ulcerations are isolated and precede the onset of all other symptoms of Behets disease. Conjunctival ulcerations are rarely seen with Behets disease, but are characteristic enough to be included among the diagnostic criteria.