Background. The introduction of foreign material into the skin can lead to sarcoidal reactions. Such a reaction is reported, consecutive to injections of botulic toxin A (Botox ). Case report. A 57-year-old woman, n...Background. The introduction of foreign material into the skin can lead to sarcoidal reactions. Such a reaction is reported, consecutive to injections of botulic toxin A (Botox ). Case report. A 57-year-old woman, noticed the occurrence of frontal and glabellar nodules, 3 weeks after the injection of botulic toxin A (Botox ), for the correction of wrinkles. Histopathological examination revealed a sarcoidal granuloma. Clinical and biological investigations were negative, ruling out the hypothesis of systemic sarcoidosis. The lesion could be reproduced experimentally by an intradermal injection of botulic toxin A on the volar aspect of the forearm. Corticosteroids per os associated with intralesional injections of triamcinolone acetonide were followed by a complete regression of the nodules. Discussion. The occurrence of sarcoidal granulomas at the sites of injection of botulic toxin A (Botox ) has not been reported-so far in the literature. Systemic sarcoidosis has been ruled out. The sarcoidal reaction has been reproduced experimentally by the intradermal injection of botulic toxin A, but not by saline. This leads to think that the sarcoidal reaction was provoked by antigenic stimulation, comparable to the Kveim reaction, and did not correspond to “ scar sarcoidosis ” .展开更多
Background. Cutaneous calciphylaxis, seen most often in dialysis patients, is characterised by skin necrosis, and is a disabling and life-threatening disease. Despite intensive topical treatment, recourse to parathyro...Background. Cutaneous calciphylaxis, seen most often in dialysis patients, is characterised by skin necrosis, and is a disabling and life-threatening disease. Despite intensive topical treatment, recourse to parathyroidectomy is often necessary. We report the case of a female patient with skin necrosis due to calciphylaxis: pain and necrotic lesions were controlled by grafting of cultured autologous keratinocytes (Epibase ). Case report. A 75-year-old woman with a 5-year history of dialysis-dependent chronic renal failure secondary to nephroangiosclerosis presented a very painful necrotic ulceration on her left leg. In spite of an autologous patch grafts, the lesions rapidly deteriorated. Laboratory data showed high levels of calcium, phosphate and parathyroid hormone and imaging suggested parathyroidal adenoma. Although cutaneous biopsywas not performed, the diagnosis of cutaneous necrosis due to cal-ciphylaxis with tertiary hyperparathyroidism was established. Treatment consisted of a low-calcium and low-phosphorus diet with autologous keratinocytes grafts (Epibase ). After the third application of keratinocytes, the pain disappeared, necrosis ceased and wound healing began. Subtotal parathyroidectomy was performed two months after the start of grafts. At three months, the patient was cured. Discussion. Calciphylaxis is an obstructive vascular disease secondary to calcification of the arterioles leading to ischemic tissue necrosis. Prompt diagnosis is essential since this disease is disabling and life-threatening due to sepsis and ischemic complications. In our case, autologous keratinocyte grafts allowed pain relief to be achieved within large expanses of cutaneous necrosis after correction of calcium and phosphorus levels, thereby allowing parathyroidectomy to be performed under optimal conditions.展开更多
Introduction. Fluindione (Previscan ) is an oral anticoagulant belonging to the vitamin K antagonist class and is very widely used in France. While bleeding is a common complication, severe immunoallergic reactions ...Introduction. Fluindione (Previscan ) is an oral anticoagulant belonging to the vitamin K antagonist class and is very widely used in France. While bleeding is a common complication, severe immunoallergic reactions are less frequent. The authors report a case of drug-induced hypersensitivity syndrome. Case report. A 75 year-old woman was hospitalized for diffuse erythematous papular rash associated with facial oedema. These symptoms appeared 3 weeks after the beginning of treatment with fluindione, allopurinol and perindopril. Laboratory tests showed hyperleukocytosis, mixed hepatitis and moderate renal failure, with the entire picture being evocative of drug-induced hypersensitivity reaction. The eruption was associated with eosinophilia, hepatic cytolysis with cholestasis, and acute renale failure. While allopurinol and perindopril were stopped definitively, fluindione was only suspended temporarily following overdosage. On reintroduction, rapid recurrence of clinical and biologic signs was observed with increased severity. The skin rash resolved completely on withdrawal of the drug. Patch tests performed later were positive for fluindione and negative for allopurinol and perindopril. Discussion. These manifestations were consistent with the diagnosis of drug-induced hypersensitivity syndrome due to fluindione. Very few cases have been described with fluindione despite widespread prescription of the treatment is in France. While there may be no skin involvement, immunoallergic signs such as fever, hepatitis and acute tubular interstitial nephritis have been described with fluindione and these may be related to this syndrome (DRESS-Drug Reaction with Eosinophilia and Systemic Symptoms). Skin patch testing, which is easily performed, can be extremely helpful in determining a causal relationship with medication.展开更多
Background. Pseudoxanthoma elasticum (PXE) is normally associated with mutations in the ABCC6 gene. A PXE phenotype without mutations in ABCC6 has been described in Greek and Italian patients presenting with beta thal...Background. Pseudoxanthoma elasticum (PXE) is normally associated with mutations in the ABCC6 gene. A PXE phenotype without mutations in ABCC6 has been described in Greek and Italian patients presenting with beta thalassemia. We attempted to determine the incidence of beta thalassemia in a cohort of French patients with PXE. Patients and methods. Fifty patients with PXE were included in the study. Laboratory examinations comprised hemoglobin electrophoresis, ABCC6 gene study and in some studies: mutation analysis, beta-globin gene. Results. No cases of beta thalassemia were diagnosed in this cohort of French patients with PXE. However, 20%of the latter exhibited a significant but isolated (i.e. without microcytic anemia) increase of hemoglobin A2 (HbA2). Statistical comparisons showed no difference in terms of geographical origin or severity of PXE between patients with high levels of HbA2 and those with normal levels of HbA2 other than the extent of cutaneous involvement. Study of the beta-globin gene displayed mutations only in the two patients with the highest recorded levels of HbA2. ABCC6 +beta-globin digenism was ruled out of the pathogenesis of PXE. Discussion. The PXE phenotype seen in some patients with beta thalassemia appears to be associated with epigenetic modification of ABCC6 transcription and depends specifically on the beta globin locus. Isolated increase in HbA2 is probably a laboratory marker for PXE. Here again, a functional epigenetic reaction between ABCC6 and the beta-globin locus was suspected. However, these reciprocal interactions are clearly unequal since the change in ABCC6 transcription occurring during the course of beta thalassaemia is responsible for a phenotype while increased HbA2 during the course of PXE has no clinical consequences.展开更多
文摘Background. The introduction of foreign material into the skin can lead to sarcoidal reactions. Such a reaction is reported, consecutive to injections of botulic toxin A (Botox ). Case report. A 57-year-old woman, noticed the occurrence of frontal and glabellar nodules, 3 weeks after the injection of botulic toxin A (Botox ), for the correction of wrinkles. Histopathological examination revealed a sarcoidal granuloma. Clinical and biological investigations were negative, ruling out the hypothesis of systemic sarcoidosis. The lesion could be reproduced experimentally by an intradermal injection of botulic toxin A on the volar aspect of the forearm. Corticosteroids per os associated with intralesional injections of triamcinolone acetonide were followed by a complete regression of the nodules. Discussion. The occurrence of sarcoidal granulomas at the sites of injection of botulic toxin A (Botox ) has not been reported-so far in the literature. Systemic sarcoidosis has been ruled out. The sarcoidal reaction has been reproduced experimentally by the intradermal injection of botulic toxin A, but not by saline. This leads to think that the sarcoidal reaction was provoked by antigenic stimulation, comparable to the Kveim reaction, and did not correspond to “ scar sarcoidosis ” .
文摘Background. Cutaneous calciphylaxis, seen most often in dialysis patients, is characterised by skin necrosis, and is a disabling and life-threatening disease. Despite intensive topical treatment, recourse to parathyroidectomy is often necessary. We report the case of a female patient with skin necrosis due to calciphylaxis: pain and necrotic lesions were controlled by grafting of cultured autologous keratinocytes (Epibase ). Case report. A 75-year-old woman with a 5-year history of dialysis-dependent chronic renal failure secondary to nephroangiosclerosis presented a very painful necrotic ulceration on her left leg. In spite of an autologous patch grafts, the lesions rapidly deteriorated. Laboratory data showed high levels of calcium, phosphate and parathyroid hormone and imaging suggested parathyroidal adenoma. Although cutaneous biopsywas not performed, the diagnosis of cutaneous necrosis due to cal-ciphylaxis with tertiary hyperparathyroidism was established. Treatment consisted of a low-calcium and low-phosphorus diet with autologous keratinocytes grafts (Epibase ). After the third application of keratinocytes, the pain disappeared, necrosis ceased and wound healing began. Subtotal parathyroidectomy was performed two months after the start of grafts. At three months, the patient was cured. Discussion. Calciphylaxis is an obstructive vascular disease secondary to calcification of the arterioles leading to ischemic tissue necrosis. Prompt diagnosis is essential since this disease is disabling and life-threatening due to sepsis and ischemic complications. In our case, autologous keratinocyte grafts allowed pain relief to be achieved within large expanses of cutaneous necrosis after correction of calcium and phosphorus levels, thereby allowing parathyroidectomy to be performed under optimal conditions.
文摘Introduction. Fluindione (Previscan ) is an oral anticoagulant belonging to the vitamin K antagonist class and is very widely used in France. While bleeding is a common complication, severe immunoallergic reactions are less frequent. The authors report a case of drug-induced hypersensitivity syndrome. Case report. A 75 year-old woman was hospitalized for diffuse erythematous papular rash associated with facial oedema. These symptoms appeared 3 weeks after the beginning of treatment with fluindione, allopurinol and perindopril. Laboratory tests showed hyperleukocytosis, mixed hepatitis and moderate renal failure, with the entire picture being evocative of drug-induced hypersensitivity reaction. The eruption was associated with eosinophilia, hepatic cytolysis with cholestasis, and acute renale failure. While allopurinol and perindopril were stopped definitively, fluindione was only suspended temporarily following overdosage. On reintroduction, rapid recurrence of clinical and biologic signs was observed with increased severity. The skin rash resolved completely on withdrawal of the drug. Patch tests performed later were positive for fluindione and negative for allopurinol and perindopril. Discussion. These manifestations were consistent with the diagnosis of drug-induced hypersensitivity syndrome due to fluindione. Very few cases have been described with fluindione despite widespread prescription of the treatment is in France. While there may be no skin involvement, immunoallergic signs such as fever, hepatitis and acute tubular interstitial nephritis have been described with fluindione and these may be related to this syndrome (DRESS-Drug Reaction with Eosinophilia and Systemic Symptoms). Skin patch testing, which is easily performed, can be extremely helpful in determining a causal relationship with medication.
文摘Background. Pseudoxanthoma elasticum (PXE) is normally associated with mutations in the ABCC6 gene. A PXE phenotype without mutations in ABCC6 has been described in Greek and Italian patients presenting with beta thalassemia. We attempted to determine the incidence of beta thalassemia in a cohort of French patients with PXE. Patients and methods. Fifty patients with PXE were included in the study. Laboratory examinations comprised hemoglobin electrophoresis, ABCC6 gene study and in some studies: mutation analysis, beta-globin gene. Results. No cases of beta thalassemia were diagnosed in this cohort of French patients with PXE. However, 20%of the latter exhibited a significant but isolated (i.e. without microcytic anemia) increase of hemoglobin A2 (HbA2). Statistical comparisons showed no difference in terms of geographical origin or severity of PXE between patients with high levels of HbA2 and those with normal levels of HbA2 other than the extent of cutaneous involvement. Study of the beta-globin gene displayed mutations only in the two patients with the highest recorded levels of HbA2. ABCC6 +beta-globin digenism was ruled out of the pathogenesis of PXE. Discussion. The PXE phenotype seen in some patients with beta thalassemia appears to be associated with epigenetic modification of ABCC6 transcription and depends specifically on the beta globin locus. Isolated increase in HbA2 is probably a laboratory marker for PXE. Here again, a functional epigenetic reaction between ABCC6 and the beta-globin locus was suspected. However, these reciprocal interactions are clearly unequal since the change in ABCC6 transcription occurring during the course of beta thalassaemia is responsible for a phenotype while increased HbA2 during the course of PXE has no clinical consequences.
