Absence of antiphospholipid and anti-endothelial cell antibodies in malignant atrophic papulosis: a study of 15 cases.
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Biomedical subjects
Publications and source records attributed to G Guillet.
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BACKGROUND: Dermatofibrosarcoma is a tumor relatively ignored in childhood. CASE REPORT: A 11 year-old boy was referred because he suffered from a tumor of the neck, which had progressively developed after a cervical traumatism occurring 4 years earlier. Examination showed a tumor moderately infiltrated, attached to the overlying erythematous skin. Biopsy showed ill-limited dermal and hypodermal proliferation of fusiform cells with a storiform arrangement. Two successive excisions were necessary and the patient is well 2 years later. CONCLUSION: This case confirms responsibility of previous traumatism, progressiveness of course and necessity of large excision.
INTRODUCTION: Darier's erythema annulare centrifugum (EAC) is a disease of unknown etiology. Some observations of EAC in infancy suggest a better prognosis for early EA. The observations presented herein gives different information regarding prognosis and pathogenesis. OBSERVATION: A child presents with typical lesions of EAC from early infancy up to the age of 15 years. Laboratory data are normal. Similar lesions were locally reproduced by intradermal injection by candidine but antifungal drugs were unsuccessful. Many antiinflammatory treatments failed to improve the disease. Because of the improvement during hyperthermic episodes, a treatment by subcutaneous interferon alpha (2 millions U 3 times a week) was given for six months with a dramatic success (with seric increase of TNF alpha and IL2) DISCUSSION: The long term duration of EAC suggests that EA of infancy may represent the early beginning of Darier's EAC. The efficacy of interferon suggests that cytokines are involved in the pathogenesis of EAC.
INTRODUCTION: Primary erythermalgia is a rare acrosyndrome due to paroxystic arteriolar dilatation of extremities that become red and congested with warmth and burning sensation or pain. OBSERVATION: We report herein the observation of a primary erythermalgia affecting 3 degrees in the same family (grandfather, uncle/mother, and children with early onset at 6 years): pizotifene, an antiserotoninergic drug proved to be effective in mother and 14-year-old child who had never drawn benefit from any previous treatment. DISCUSSION: Several etiologies may be responsible for erythermalgia according to the clinical type: primary erythermalgia with pediatric onset, and secondary erythermalgia as a reaction to myeloproliferation, other diseases, drug or heavy metal poisoning. Pathophysiology of primary erythermalgia is not clearly understood and the main concern is therapy: in this respect, pizotifene deserves to be considered as a valuable possibility.
A patient with an acquired pigmentation related to lichen planus pigmentosus is described. Features of acrokeratosis of Bazex were associated, related to a head and neck carcinoma. Both cutaneous conditions disappeared after treatment of the neoplasia. Diagnostic criteria of lichen planus pigmentosus are reviewed. The paraneoplastic nature of this original observation of lichen planus pigmentosus is discussed.
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Nine dual-fluorescence combinations were used to enumerate T-cell subsets in 112 human immunodeficiency virus type 1-infected patients. Two blood samples were analyzed, with a 6-month interval between the tests, in 53 of these 112 patients. The alteration in CD4 over this period of time correlated with the change in CD8 and CD8S6F1 (P < 0.02 and P < 0.01), irrespective of the disease stage. Two groups of patients were defined by the CD8S6F1 subset at the first normal levels. Changes in numbers of CD4, CD4CD45RA, and CD4CD29 were significantly higher in group B than in group A patients. The absolute count of CD8S6F1 could thus serve as an indicator of the ensuing depletion of the CD4 population, as well as the CD4 subsets.
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Ionophoresis is a well-established treatment for idiopathic hyperhidrosis. Modern apparatuses are reliable as long as the electrical equipment is used correctly. The authors present five cases of cutaneous incidents resulting in burns and secondary necrosis. Two of the cases could be explained by defective protection and the other three were apparently of the same type although non confirmation could be established. In comparison with uneventful incidences (pruritus, erythematous reactions, dysaesthesia) these spectacular burns should not exist. Machines must be regularly check and only electrodes with optimal protection should be used.
INTRODUCTION: A new case of hairy elbows is reported. This localized hypertrichosis, which appears in the first months of life, deserves to be individualized. CASE-REPORT. A 10-year old girl presented with hairy elbows and short stature (-2.5 S.D.). Hypertrichosis and unexplained intrauterine growth retardation had been noticed at birth. CONCLUSION: Ten cases of hairy elbow have been reported since Beighton's case. Unexplained short stature (unrelated to any specific dysmorphic syndrome) was observed in 7 cases. Hairy elbows could be considered a mark of short stature, but this association might be overestimated owing to the very few published cases.
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Food origin seems to have a special place in the etiology of infantile urticaria, as it forms 62% of a series of 51 observations of urticaria in children, greater than drug etiology (22%), physical urticaria (8%) and contact urticaria (8%). Distinction between true and false food allergy, which is important for prognosis and evolution, puts true food allergy (55%) in the lead before false (7%). A reason for this, the atopic concept, was seen in 67% of the food urticarias, corresponding apparently to true food allergy. If in the majority of these cases the pathway of sensitizations was unknown, in very rare observations the sensitization pathway was reported as an inhalation or even a sensitization in utero. Finally, infantile urticaria, more serious because of the vital risks that may be associated with it, deserves a special comment from the etiological viewpoint: observation of urticaria (and shock) to peach in an infant of 4 months, to wheat flour at 5 months and egg at 6 months are a demonstration that between 4 and 6 months intolerance of cows milk proteins is not the only etiology of infantile urticaria. For prognosis, early detection of true food allergy in children gives hope of better results than in adults, with higher chances of regression of the sensitization.
BACKGROUND AND DESIGN: The aim of the study was to define the natural history of sensitization in atopic dermatitis (AD) through cross-sectional (comparison of three age classes) and longitudinal study: 250 children with minor (32.5%), moderate (32.5%), and severe (35%) AD were examined for aeroallergen and food hypersensitivity (skin tests, clinical scoring after allergen elimination, and food challenges). RESULTS: The allergic screening was negative in minor AD and positive in 33% of cases of moderate AD that were concerned with aeroallergen sensitizations with only limited respiratory involvement. This suggests that nonspecific cutaneous hyperreactivity remains an almost exclusive precipitating factor in moderate or minor AD. Severe AD was characterized by a positive allergologic assessment in 100% of patients: food allergens were incriminated as flare factors in 96% of patients, with associated aeroallergen sensitization in 36%. Even at a young age (less than or equal to 2 years), the severe AD group is marked by an extreme frequency of food sensitization (93%) that persists in 73% of children younger than 7 years and 67% younger than 16 years. From the comparison of three age classes, the sequence of food and respiratory sensitization seems to be part of the natural course of AD. Prospective study in 29 children of group 1 (less than 2 years) with a 3-year follow-up confirms the data of the cross-sectional study since 27 had development of aeroallergen sensitization, with respiratory symptoms as early as age 3 years in 23 of these children. CONCLUSION: The detection of food allergy in a child presenting with AD is likely to indicate a prognosis of severe AD and should be considered by dermatologists as a potentially important predictor of further respiratory symptoms.
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