Patterns of Skin Disease in the Context of a High Prevalence HIV Population in Botswana.

The authors reviewed outpatients in a tertiary dermatology clinic in Botswana to expand knowledge on patterns of skin disease in this population with a high prevalence of human immunodeficiency virus (HIV). Approximately one-third of new and follow-up patients were HIV positive. Common dermatologic conditions included eczematous eruptions, viral and fungal infections, malignant neoplasms, vascular disorders, disorders of pigmentation, and mechanical/physical injury-related disorders. HIV has impacted patterns of dermatologic disease in Botswana, with Kaposi sarcoma being the most frequently biopsied condition. Given the shortage of dermatology specialists, resources should be allocated toward education and management of these most prevalent skin conditions.

Cutaneous microangiopathy in patients with type 2 diabetes: Impaired vascular endothelial growth factor expression and its correlation with neuropathy, retinopathy and nephropathy.

AIMS/INTRODUCTION: To examine the three-dimensional morphology and vascular endothelial growth factor (VEGF) expression of skin microvasculature in patients with type 2 diabetes in relation to neuropathy, retinopathy and nephropathy. MATERIALS AND METHODS: The present study enrolled 17 individuals with type 2 diabetes and 16 without. Skin sections were double-immunostained for type IV collagen and VEGF-A or protein gene product 9.5. Projected images from confocal microscopy served to quantify the occupancy rate of subepidermal type IV collagen-immunoreactive microvascular basement membrane area (OR-T4MBM), subepidermal VEGF-A-immunoreactive area and the VEGF/T4MBM ratio, as well as the protein gene product 9.5-immunoreactive intraepidermal nerve fiber density. Reduced intraepidermal nerve fiber density was applied for the diagnosis of neuropathy, fundic ophthalmoscopy and fluorescein angiography for retinopathy, and microalbuminuria or persistent proteinuria for nephropathy. RESULTS: A total of 12 patients with diabetes had neuropathy, 10 had retinopathy and eight had nephropathy. Regardless of the presence or absence of neuropathy, retinopathy or nephropathy, OR-T4MBM was significantly increased in patients with diabetes compared with individuals without diabetes. In contrast, VEGF/T4MBM ratio was significantly decreased in those with neuropathy and retinopathy, as well as in those with and without nephropathy, whereas a trend toward a decreased VEGF/T4MBM ratio was seen in patients without retinopathy, as compared with individuals without diabetes. CONCLUSIONS: The present study is the first report to show that cutaneous microangiopathy, as indicated by subepidermal microvascular proliferation and impaired VEGF expression, appears to occur before the development of overt clinical neuropathy, retinopathy or nephropathy in patients with type 2 diabetes.

[Vascular Cutaneous Abnormalities in Togo: a 120-Case Study]. / Anomalies vasculaires cutanées au Togo : étude de 120 cas.

The purpose of this study was to describe the epidemio-clinical profile, and treatment of vascular cutaneous abnormalities in Togo. It was a retrospective study of patients recorded in dermatology for vascular cutaneous abnormality between 1998 and 2017. During the study period, 120 (0.1%) of 88,869 patients received in dermatology have consulted for vascular cutaneous abnormalities. Their mean age was 25.3 months and the sex-ratio (M/F) 0.4. The most recurring vascular cutaneous abnormalities were vascular tumors (97 cases; 80.8%), mainly infantile hemangioma (IH) (93 cases; 77.5%). The mean time of onset of IH after birth was 2.9 months. The IH (54 cases; 50.5%) and port wine stains (8 cases, 53.3%) were predominantly localized at the cephalic region and Klippel-Trenaunay syndrome cases on lower limbs. We opted for a therapeutic abstention in the majority of the patients (50 cases of IH, 16 cases of simple vascular abnormalities, all Klippel-Trenaunay syndrome cases and telegiectasia cases). Of the 43 IH cases treated, the main drugs used were corticosteroids (23 cases of which 17 between 1998 and 2011 and 6 from 2012) followed by propranolol (11 cases from 2012). We had a favorable response in 9 of the 15 patients seen again. Cutaneous vascular abnormalities are very rare in dermatology in Togo and are dominated by IH with female predominance. In IH treatments, propranolol use, started in 2012 in Togo, is increasing when corticotherapy has declined.

L'objectif de cette étude était de décrire le profil épidémioclinique et thérapeutique des anomalies vasculaires cutanées au Togo. Il s'agit d'une étude rétrospective portant sur les dossiers des patients reçus en dermatologie pour une anomalie vasculaire cutanée entre 1998 et 2017. Durant la période d'étude, 120 (0,1 %) des 88 869 patients reçus en dermatologie ont consulté pour une anomalie vasculaire cutanée. L'âge moyen des patients était de 25,3 mois et le sex-ratio (H/F) de 0,4. Les anomalies vasculaires les plus recensées étaient les tumeurs vasculaires (97 cas ; 80,8 %), principalement les hémangiomes infantiles (HI) [93 cas ; 77,5 %]. Le délai moyen d'apparition des HI après la naissance était de 2,9 mois. Les HI (54 cas ; 50,5 %) et les angiomes plans (8 cas, 53,3 %) étaient localisés de façon prépondérante à la région céphalique et les cas de syndrome de Klippel-Trenaunay aux membres inférieurs. Nous avons opté pour une abstention thérapeutique chez la plupart des patients (50 cas d'HI, 16 cas de malformations vasculaires simples, tous les cas de syndrome de Klippel-Trenaunay et le cas de télangiectasies). Sur les 43 cas d'HI traités, les principaux médicaments utilisés étaient les corticoïdes (23 cas dont 17 entre 1998­2011 et 6 à partir de 2012) suivis du propranolol (11 cas à partir de 2012). Nous avions noté une réponse favorable chez 9 des 15 patients revus. Les anomalies vasculaires cutanées sont très rares en dermatologie au Togo et sont dominées par les HI, avec une prédominance féminine. Dans le traitement des HI, l'usage du propranolol, débuté en 2012 au Togo, est en augmentation pendant que celui de la corticothérapie baisse.

Wilms tumor screening in diffuse capillary malformation with overgrowth and macrocephaly-capillary malformation: A retrospective study.

BACKGROUND: CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies) syndrome is associated with regional bony and/or soft tissue overgrowth, capillary malformation, and an increased risk for Wilms tumor. OBJECTIVE: To evaluate the frequency of Wilms tumor in patients with 2 similar conditions: diffuse capillary malformation with overgrowth (DCMO) and macrocephaly-capillary malformation (M-CM). METHODS: Culling our Vascular Anomalies Center database, we retrospectively reviewed patients in whom DCMO and M-CM had been diagnosed and who were evaluated between 1998 and 2016 for possible development of Wilms tumor. Patients younger than 8 years of age at their last visit and not seen in more than 2 years were contacted for follow-up. RESULTS: The study comprised 89 patients: 67 with DCMO, 17 with M-CM, and 5 with an indeterminate diagnosis. No case of Wilms tumor was found in these groups. LIMITATIONS: Some patients were younger than 8 years of age at last follow-up visit and the sample size was small. CONCLUSION: Patients with DCMO do not appear to be at increased risk for Wilms tumor. Screening is probably unnecessary in DCMO unless there is associated hemihypertrophy. Although there were no cases in our cohort, there are 2 reports of M-CM associated with Wilms tumor in the literature.

[Cutaneous vascular anomalies in children]. / Les anomalies vasculaires cutanées chez l'enfant.

INTRODUCTION: Vascular anomalies, which are erroneously categorized under the term angiomas, are a highly heterogeneous group of lesions that are poorly understood and affect a mean of 5 to 10 % of children. The fortuitous discovery of propranolol's efficacy in one of these entities has made them a topical issue. OBJECTIVES: The paper's main objective is to inform family doctors of the various types of vascular anomalies, clarify their classification, and provide a common terminology. Its secondary objective is to provide a decision tree that enables primary care doctors to avoid diagnostic pitfalls, successfully detect cases, and optimize management. METHODS: Systematic review. CONCLUSIONS: According to a recent study, 71,3 % of publications use the term hemangioma erroneously, regardless of the authors' field. The key for family doctors is to use one international classification only, that of the International Society for the Study of Vascular Anomalies (ISSVA), in order to facilitate management and comprehension between the different healthcare levels. The diagnosis of vascular anomalies is clinical in 90 % of cases, so all family doctors can, whilst using a decision tree, diagnose a vascular anomaly and refer only those that are complex for specialist care. The most common vascular anomaly is infantile hemangioma in infants, which spontaneously regresses around the age of 5-7 years in 90 % of cases. Watchful waiting and regular follow-up suffice, therefore, in such settings.

[Phlebology in German departments of dermatology. An analysis on behalf of the German Society of Phlebology]. / Phlebologie an deutschen Hautkliniken. Eine Bestandsaufnahme im Auftrag der Deutschen Gesellschaft für Phlebologie.

BACKGROUND: Phlebologic diseases have become extremely common and have major socio-economic impact. However, the percentage of dermatologists working in phlebology appears to be decreasing according to the data of the German Society of Phlebology (DGP). METHODS: To investigate the reasons for this development, we--on behalf of the DGP--sent a questionnaire to 120 German Departments of Dermatology in autumn 2012. RESULTS: In 76 returned questionnaires, the number of physicians with additional fellowship training in phlebology averaged 1.5; the average number of those who fulfill the criteria for training fellows in phlebology was 0.9. In 71.1 % of the departments there was a phlebologist. A special phlebologic outpatient clinic existed in 73.7 % of the departments. Sonography with Doppler (89.5 %) and duplex (86.8 %) was used as the most frequent diagnostic tool. For therapy, compression (94.7 %), sclerotherapy (liquid 78.9 %, foam 63.2 %, catheter 18.4 %), endoluminal thermic procedures (radio wave 28.9 %, laser 17.1 %) and surgery (especially crossectomy and stripping 67.1 %, phlebectomy of tributaries 75 %) were used. The average number of treatments was very heterogenous in the different departments. CONCLUSIONS: Phlebology definitely plays an important role in dermatology. Most departments fulfill the formal criteria for the license to conduct advanced training in phlebology. A wide spectrum of phlebological diagnostic and therapeutic procedures is available.

Cutaneous manifestations in patients with POEMS syndrome.

BACKGROUND: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome is a rare multisystem paraneoplastic condition associated with plasma cell dyscrasia. METHODS: From our institution's dysproteinemia database, 107 patients met criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome between January 1, 2000, and October 1, 2009. Medical records were reviewed for documented syndrome features at diagnosis. We assessed prevalence of skin findings and associations between dermatologic and other characteristic disease findings. RESULTS: Of the 107 patients, 96 (90%) had a recognized cutaneous manifestation. Hyperpigmentation and hemangioma were most common (47%), followed by hypertrichosis (38%). Vascular skin changes--acrocyanosis (34%), Raynaud phenomenon (20%), hyperemia/erythema (20%), flushing (16%), or rubor (11%)--occurred in 62%; white nails, sclerodermoid changes, and clubbing occurred in 30%, 26%, and 6%, respectively. Mean number of skin findings per patient was 2.9 (median, 3.0; range, 0-7). Presence of cutaneous manifestation was associated with abnormal pulmonary function tests (P < 0.001); immunoglobulin G gammopathy was associated with hyperpigmentation and hypertrichosis. No other significant associations were seen. CONCLUSIONS: The high prevalence of skin findings (90%) shows the value of dermatologic evaluation in diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. Our data indicate new associations between skin findings and other disease characteristics.

[Skin vessel lesions in aluminum potroom workers].

The features of development of the skin vessels lesions in 550 aluminum production workers have been investigated. The high prevalence of these disorders have been revealed in anode-operators and cell-operators, 49, 3 and 26.0% of workers, respectively. The regularity and staging of the development of this abnormity have been established, etiology, pathogenesis and clinical significance of those remain unknown.

Frequency and phenotypes of cutaneous vascular malformations in a consecutive series of 417 patients with familial cerebral cavernous malformations.

BACKGROUND: Familial cerebral cavernous malformations (FCCM) are vascular malformations inherited as an autosomal-dominant condition. Three genes (KRIT1/CCM1, MGC4607/CCM2, PDCD10/CCM3) have been identified so far. Extra-neurological manifestations include retinal and cutaneous vascular malformations. The cutaneous vascular malformation, which had been more specifically associated with FCCM, is hyperkeratotic cutaneous capillary venous malformation (HCCVM). OBJECTIVES: To define the frequency of cutaneous vascular malformations in patients with FCCM, to precise their different phenotypes, and to study the association of each cutaneous vascular malformation subtype with the different three mutated CCM genes. METHODS: Dermatological inquiry was systematically performed in a large series of consecutive FCCM patients. Cutaneous biopsies were reviewed when available. Cutaneous vascular malformations classification was based on predominant anomalous channels, using the current International Society for the Study of Vascular Anomalies classification. Molecular screening of CCM genes was performed. Results Four hundred seventeen consecutive FCCM patients from 182 unrelated families were included. 38 patients (9%) from 25 different families had cutaneous vascular malformations. In these 38 patients, cutaneous vascular malformations were classified as follows: 13 capillary malformations (CM), 15 HCCVM, 8 venous malformations (VM) and 2 unclassified lesions. All patients (92%), but one with CM had a KRIT1/CCM1 mutation. The last patient had no detectable mutation. All of the 15 patients with HCCVM had a KRIT1/CCM1 mutation; 86.7% of cutaneous vascular malformation patients (33 of 38) had a KRIT1/CCM1 mutation. CONCLUSION: Cutaneous vascular malformations are seen in 9% of FCCM patients. Three distinct major cutaneous vascular malformations phenotypes were identified: HCCVM (39%), CM (34%) and VM (21%). CCM1 is the most frequently mutated gene in cutaneous vascular malformations-FCCM patients.

Skin manifestations of rheumatoid arthritis: a study of 215 Turkish patients.

OBJECTIVE: To investigate the frequency and clinicopathological features of skin involvement in rheumatoid arthritis (RA), to find out whether early and aggressive disease-modifying treatment is changing the spectrum towards a milder disease pattern. METHODS: Two hundred and fifteen consecutive RA patients were enrolled. Main outcome measures were the frequency of cutaneous features and their relation to disease severity and treatment modality, ultrasound (USG) examination of nodules, histopathological examination of nodules and papules. RESULTS: Mean age and disease duration were 55.3 years and 138.1 months, respectively. Sixty-six percent of the patients had erosive disease, 70% were rheumatoid factor (RF) positive and 44% had Classes III and IV functional index. Among 43 patients having nodules, 20 were diagnosed as rheumatoid nodules (RNs) and the others as synovitis, bursitis, and so on, on clinical basis and by ultrasound. In 7 of 20 RNs, diagnosis was confirmed by histopathological examination. When clinical, histopathological, and USG data were analyzed collectively, sensitivity, and specificity of USG were found as 100 and 75%, respectively. Sixteen patients had relevant papular lesions. Fourteen of these were diagnosed as palisading neutrophilic granulomatous dermatitis (PNGD) and two as rheumatoid neutrophilic dermatitis (RND) on histopathological examination. Accelerated nodulosis was seen in one, and vasculitis in two of the patients. CONCLUSIONS: We observed a milder disease pattern compared to Anglo-Saxon countries with lower RN and vasculitis frequency. This cannot be explained by early and aggressive treatment as disease onset to treatment interval was long in our patient group. PNGD resembles RN in terms of frequency and association with severe disease.

Diagnosis of vascular skin lesions in children: an audit and review.

Vascular skin lesions in children are common, but their management is complicated by the ongoing use of inappropriate and often misleading terms. Inaccurate explanations of the diagnosis and progression of these lesions may cause unnecessary distress. This study describes an audit of vascular lesions in children from one center in which the classification system described by Mulliken and Glowacki was used to establish diagnosis. Seventy patients with vascular lesions attending vascular lesion clinics, miscellaneous plastic surgery clinics and laser lists in the Royal Victoria Infirmary, Newcastle-upon-Tyne, were studied. A proforma was completed using information from the patients and their medical records. The initial diagnosis given to parents was inaccurate in 69% of patients. Furthermore, 53% of parents were given an incorrect account of what to expect with regard to progression of the lesion and the prospect of future treatment. Altogether, in 71% of instances some inappropriate terminology was applied during the course of the child's management. Plastic surgeons were the most accurate diagnosticians. This study highlights the fact that a selection of misleading and erroneous terms is still regularly used in medical practice. The recommended classifications are rarely adhered to and in consequence efficient communications between professionals and families is often lacking.

Manifestaciones mucocutáneas del lupus eritematoso sistémico en pacientes adultos chilenos / Cutaneous manifestations of lupus erythematosus in Chilean adults

El objetivo del presente estudio fue analizar la prevalencia de lesiones mucocutáneas en pacientes adultos con Lupus Eritematoso Sistémico (LES). Se incluyó a 110 pacientes. El 52,7 por ciento de los participantes presentaron manifestaciones cutáneas como presentación inicial de LES. No hubo diferencias estadísticamente significativas entre los pacientes con manifestaciones cutáneas como presentación inicial de la enfermedad y los pacientes sin manifestaciones cutáneas, en relación a la proporción mujeres/hombres, edad de presentación de los síntomas de LES, edad de diagnóstico de LES e intervalo de tiempo entre la aparición de los primeros síntomas y el diagnóstico de la enfermedad. La prevalencia de manifestaciones mucocutáneas fue de 87,2 por ciento. Los hallazgos más frecuentes fueron fotosensibilidad, eritema malar y fenómeno de Raynaud. Un 40 por ciento de los pacientes presentó lesiones específicas agudas, un 1,8 por ciento, lesiones subagudas y un 15,3 por ciento, lesiones crónicas. Un 84,5 por ciento de los pacientes mostró lesiones inespecíficas. En comparación a series extranjeras, registramos una mayor prevalencia de fotosensibilidad y livedo reticularis y una menor prevalencia de lupus cutáneo subagudo, lupus discoide, alopecia, úlceras orales, vasculitis y úlceras cutáneas crónicas.

The purpose of this study is to analyze the prevalence of mucocutaneous lesions in adults with Systemic Lupus Erythematosus (SLE). We enrolled 110 patients. Cutaneous manifestations were present as the first sign of SLE in 52.7 percent of patients. We found no statistical difference between patients with cutaneous manifestations as the initial finding of the disease and patients without cutaneous manifestations, regarding sex, age at initial symptoms of SLE, age at the diagnosis of SLE and period of time between first symptoms and the diagnosis of SLE. Prevalence of cutaneous manifestations was 87.2 percent. The most common findings were photosensitivity, malar erythema and Raynaud s phenomenon. Specific acute lesions were present in 40 percent of the cases, subacute lesions in 1.8 percent and chronic lesions in 15.3 percent. Non specific lesions were present in 84.5 percent of the cases. In the present study we found a higher prevalence of photosensitivity and livedo reticularis, and a lower prevalence of cutaneous subacute lupus, discoid lupus, alopecia, oral ulcers, vasculitis and chronic cutaneous ulcers, compared with international results.

Livedoid vasculopathy: further evidence for procoagulant pathogenesis.

OBJECTIVE: To further characterize the clinical and pathologic features, disease associations, and laboratory abnormalities of livedoid vasculopathy. DESIGN: Retrospective study of patients identified from our institutional database from January 1, 1990, to December 31, 2000. SETTING: Tertiary care institution. Patients Forty-five patients with biopsy-proved livedoid vasculopathy. MAIN OUTCOME MEASURES: Clinical presentation, histopathologic diagnosis, results of testing for coagulation abnormalities, and assessment of vascular status. RESULTS: Thirty-two patients (71.1%) were female (mean age, 45 years; age range, 10-85 years). Bilateral lower extremity disease occurred in 36 patients (80.0%), ulceration in 31 (68.9%), and atrophie blanche in 32 (71.1%). In patients tested, transcutaneous oximetry measurements were decreased in 20 (74.1%) of 27, and factor V Leiden mutation (heterozygous) was noted in 2 (22.2%) of 9, decreased activity for protein C or protein S in 2 (13.3%) of 15, prothrombin G20210A gene mutation in 1 (8.3%) of 12, and lupus anticoagulant in 5 (17.9%) of 28. Anticardiolipin antibodies were present in 8 (28.6%) of 28 patients, and elevated homocysteine levels in 3 (14.3%) of 21. Intraluminal thrombosis was observed in 44 (97.8%) of 45 skin biopsy specimens. Direct immunofluorescence disclosed multiple vascular conjugates in 31 (86.1%) of 36 biopsy specimens. CONCLUSIONS: Livedoid vasculopathy was predominantly bilateral, affected the lower extremities, and was associated with ulceration and atrophie blanche. Histologic evidence of intraluminal thrombosis was observed in almost all biopsy specimens reviewed. Laboratory testing revealed numerous heterogeneous coagulation abnormalities, providing further evidence of procoagulant mechanisms.

[Skin diseases observed in the dermatology departments of three French university teaching hospitals]. / Activité de consultation de trois services de dermatologie hospitalo-universitaires français.

BACKGROUND: We recently carried out a study concerning consultations by French dermatologists in private practice. We evaluated consultations at the dermatology departments of 3 university teaching hospitals in France. MATERIALS AND METHODS: This was a 2-month prospective study conducted in 2003 at the dermatology departments of the university teaching hospitals of Amiens, Lille and Rouen. Each consultant completed a questionnaire covering the duration of the study. The following data were recorded: consultation date, function of the consultant, study centre, type of consultation, type of disease, and whether or not the patient was hospitalised after the consultation. RESULTS: 7296 files were examined during the study. 38% of the consultations were performed by part-time hospital consultants, 29% by dermatology interns, 18% by hospital practitioners, 9% by university professors and 6% by clinical heads or assistant heads. The most commonly encountered diseases were allergies (17%), cancer (16%), arteriovenous disease (15%) and infectious disease (11%). Three types of consultation were identified: emergency consultations without an appointment, consultations by appointment for a specific problem and consultations by appointment without a specific problem. The number of resulting hospital admissions ranged from 2 to 10% of consultations, depending on the type of consultation and the role of the consultant in question. DISCUSSION: This study shows that in France, consultations at hospital dermatology departments differ greatly from those of dermatologists in private practice. The main diseases seen (cancer, arteriovenous disease, allergy, infectious dermatosis) accounted for the majority of hospitalisations in these departments. The organisation of hospital consultations is increasingly tending both towards treatment of highly specialised diseases through specifically oriented consultations and also towards the emergency treatment of certain forms of acute dermatosis.

[Ischemic stroke at the young age: the role of antiphospholipid antibodies]. / Ishemicheskii insul't v molodom vozraste: rol' antifosfolipidnykh antitel.

Output of antiphospholipid antibodies (aPL) coupled with arterial and/or venous thromboses, miscarriage and some other clinical manifestations is denoted as antiphospholipid syndrome (APS). The syndrome is primary (PAPS) in the absence of other autoimmune diseases. Arterial thromboses occur most frequently in the cerebral arteries, leading to ischemic disorders of cerebral circulation (DCC). The present work summarises the results of our own studies and of the reported data on DCC in PAPS. Their characteristic features Include the relationship with occlusion of the intracerebral or intracranial rather than of the great vessels of the head, liability for recurrences in the absence of secondary prophylaxis, frequently occurring association with primary disorder of cerebral circulation (PDCC), good restoration of the focal neurologic deficit after the first stroke, more frequent development in women. The clinical recognition of DCC which stem from aPL output is favoured by the presence in the patients of the basic non-cerebral signs of PAPS (miscarriage peripheral venous thrombosis, myocardial infarction) as well as by the presence of the additional evidence of PAPS (livedo, induration of heart valves according to the EchoCG data, epileptic syndrome, migraine-like headaches, chorea in the anamnesis, and so forth). In most cases, they precede the first DCC by several years or months. The secondary prophylaxis of DCC in PAPS includes the intake of indirect anticoagulants and. small doses of aspirin.

Polyarteritis nodosa and cutaneous polyarteritis nodosa.

Cutaneous vasculitis can present with a variety of lesions and associated symptoms. It is important for the clinician to be able to recognize vasculitis lesions and know when to pursue further laboratory studies that will determine the extent of the disease process in a patient. This review compares and contrasts polyarteritis nodosa with its cutaneous counterpart. The diagnostic workup and management of these two entities as well as the overall approach to a patient with a possible vasculitic condition are emphasized.

[Antiphospholipid syndrome in patients with systemic connective tissue diseases]. / Antifosfolipidinis sindromas sergant sisteminemis jungiamojo audinio ligomis.

The present study evaluates the incidence of antiphospholipid antibody syndrome (APS) in systemic rheumatic diseases patients with anticardiolipin antibodies. Clinical presentations of systemic rheumatic diseases in patients with or without APS are examined as well. The data from 242 consecutive patients suffering from rheumatoid arthritis (158 pts), systemic erythematoid lupus (53 pts), or systemic sclerosis (31 pts) are studied. Enzyme immunoassay test for IgG-anticardiolipin antibodies was performed for all patients. The IgG-anticardiolipin antibodies were found in 38 (15.7%) patients. There were 16 (30.2%) patients in erythematoid lupus group, 9 (29%) patients in systemic sclerosis group, and 13 (8.2%) patients in rheumatoid arthritis group. The diagnosis of secondary APS according to classificational criteria (1998) was confirmed in 14 (36.8%) from 38 seropositive patients: ten patients (62.5%) in lupus group, 2 (22.2%) patients in systemic sclerosis group, and 2 (15.4%) patients in rheumatoid arthritis group were found as APS patients. The majority of these patients were female (92.9%). These patients were younger as compared with systemic rheumatic diseases patients without APS (p=0.005). There was no significant difference found between APS patients and patients without APS in respect to neither duration of primary disease, nor disease activity, nor course of the disease. There were significantly more cases of fibrotic heart valves (p=0.028), and thrombocytopenia (p=0.002), and livedo reticularis (p=0.058) in APS group.