Chronic granulomatous disease: late onset of skin lesions only, in two siblings.
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Biomedical subjects
Publications and source records attributed to J F Stalder.
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A methodic study of 50 cases, had to the discussion of a real clinical well established entity. The lesions start from an initial site, located on the inferior limbs in 2/3rd of the cases; then within a variable period of time extend larger and larger by proliferating in numerous patches. These lesions present as polymorphic, erythemato-squamous or oozing. They often evolve towards recurrence. The disease is more frequently encountered among 50-year-old men. Several factors contribute to the onset of the lesions: chronic alcoholism--lack of sanitary conditions--microbial background--stasis--overweight. A disturbed system of cutaneous protection and a lack of immune defences versus an antigen, probably a bacteria, are at the origin of the pathogenesis of these disorders. Considering the treatment, we wish to underline the benefit of local coal tar applications.
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Observations of two patients illustrating the problem of Shulman syndroma. The first had presented an oedema of the four limbs, without injury of the trunk, but with myalgias, histological fasciitis, eosinophilia and hypergammaglobulinemia. In spite of corticotherapy, evolution toward a generalized morphea. The second has presented the same oedema of the four limbs, with myalgias, but also Raynaud syndroma, histological fasciitis and eosinophilia. Evolution toward acrosclerosis in spite of corticotherapy. For the authors, Shulman's syndroma is apprehended as a particular clinical form of scleroderma.
The most early outset, the usually bilateral type, the eventual association with abnormalities of extremities distinguish Milroy-Meige-Nonne' disease and congenital not familial lymphedema. Two cases illustrate this problem. The simplicity of realization, the topographic informations and the possibility of repetition are arguments for isotope lymphangiography with colloidal sulfur of rhenium.
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The authors report three personal cases of phagedenic pyoderma associated with hemopathy. Studying twenty-five other cases, described in the reviews, the question can be debated on three levels: 1) The clinical characteristics of hemopathic pyoderma remain non-specific. However blisters, pustules, and even vegetating lesions occur very often; 2) the etiology of hemopathy is subject to change; acute leukaemia or myeloproliferation syndrome. During the polyglobulars associated with pyoderma, anemia and myelofibrosis appear quite constantly; 3) finally on the pathogenic level, the recent works tend towards the hypothesis of a damage in the functioning of the polymorphonuclears hence displaying the increase in the inflammation during phagedenic pyoderma.
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In children, fibromatoses are fairly uncommon tumours assuming a variety of forms, whilst cutaneous fibromas are rarely found. Recognisable histologically by varied degrees of proliferation of fibroblasts and collagenous fibres, they present a very varied clinical and evolutive picture. A description is given of a case corresponding to "progressive ossifying myositis" and a criticism made of this designation which includes a condition which is not myositis at all but rather an ossifying fibromatosis.
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INTRODUCTION: The assessment of the educational management of 40 moderate to severe atopic patients was performed in the atopic dermatitis school created in Nantes (France) from November 2000 to May 2001. SUBJECTS AND METHODS: Patients with moderate to severe atopic dermatitis (SCORAD >20) were selected according to the criteria of Hanifin and Rajka and included in an open pilot study including a specific educational program. Atopic training included consultations, demonstrations, interviews with both dermatologist and nurses and workshops organized in three subgroups according to the age of the patients. SCORAD index, educational diagnosis and at least one educational objective were established during the first consultation. Evaluation criteria were defined in accordance with the educational objectives. Specific educational tools were used for each objective. RESULTS: Forty patients (mean age: 9 years) were followed during 6 months. At the beginning of the study the mean SCORAD was of 50.5. After a 6 months follow-up, the mean SCORAD was of 22 and improvement of the SCORAD was noted in 97 p. 100 of cases. Educational objectives were reached in 70.6 p. 100 of cases. One patient lost contact. DISCUSSION: The educational approach of atopic dermatitis includes global assessment of the disease after identification of risk factors, disrupted quality of life, skin-damaging behavior, family stress. Educational diagnosis focusing on local treatment is a major step; topical treatment is either misunderstood or conducted erroneously or non-existent. Therapeutic education plays a key role in the compliance and efficacy of treatment. Pain management, demonstration and explanation of skin care by health care professionals, information on the disease in age-specific workshops provided good results in more than 70 p. 100 of cases. Severe atopic dermatitis in children can lead to major domestic conflicts when the parents are in charge of the treatment. Giving responsibilities to the children, by teaching them how to treat themselves, relieves the parents from their involvement and finally reduces the conflicts. CONCLUSION: The difficulties encountered in treating refractory atopic dermatitis may be secondary to the severity of the disease, to poor compliance or to the extent of the psychological impact. Therapeutic education of the patient, as performed in the atopic dermatitis school, represents a major breakthrough in the care of such patients.
Orf is an infectious ulcerative stomatitis of sheep and goats. The responsible pathogen, parapoxvirus, may be transmitted to humans. Orf lesions are often atypical in immunocompromised individuals. The present report describes two very large exophytic lesions in a 31-year-old transplant patient receiving oral tacrolimus, mycophenolate mofetil and prednisone. Early surgical excision was successful, with no relapse after 14 months.
From 1979 to 1988, 8 cases of balanitis xerotica obliterans were histologically demonstrated in children at Nantes University Hospital. In 6 of these 8 patients, whose average age at the time of consultation was 9.4 years, the phimosis was obviously acquired or recurrent, and the prepuce was sclerous, thick, and produced a tight distal phimosis. One child coming to consultation for relapsing urinary infections at once presented with a long, tight stenosis of the anterior ureter, associated with a dilatation of the upstream ureter and a filiform stream of urine. All the children were circumcised, and the histological study of the specimen demonstrated characteristic lesions in the superficial derma. During the same operation, meatostomy was performed in two children presenting with meatal stenosis. Six of 8 children were followed up with an average time lapse of 6 months: one of them presented with an involvement of the glans penis and a moderate meatal stenosis, which disappeared spontaneously. No improvement occurred for the child presenting with an extensive stenosis of the anterior ureter, in spite of the local instillation of androgens then of corticoids, but his left kidney was dilated on sonograms. Balanitis xerotica obliterans in children is often not recognized and regarded as a common phimosis; it can be suspected because of the clinical features of the phimosis. The condition must be confirmed by the histological study of a circumcision specimen, then followed up regularly to prevent any risk of involvement of the glans, of the ureteral meatus or of the anterior ureter.
Study of the course of 23 patients, with late cutaneous porphyria, after 3 to 8 years. So, we remark: the clear improvement of the majority of the patients, from whom a quarter appear entirely cured, the more satisfactory affects of bleeding and alkali-therapy, the absence of reference with the course of chronic alcoholism the absence of visceral complication of porphyria.
Discussion of a case relating to a patient affected by a typical tuberous sclerosis, and a lymphangiomyomatosis syndrome, proliferation of unstriated muscles of lymphatic system. These symptoms induce pulmonary injuries, ascites and renal angiomyolipomas. The closeness of these localizations with the visceral manifestations of tuberous sclerosis leads to debate connections among the two diseases.