[Atorvastatin-induced drug reaction with eosinophilia and systemic symptoms (DRESS)]. / Syndrome d'hypersensibilité médicamenteuse induit par l'atorvastatine.

BACKGROUND: Atorvastatin is a widely-used therapeutic statin given for hypercholesterolaemia and for prevention of cardiovascular events. We report herein the occurrence of a drug reaction with eosinophilia and systemic symptoms (DRESS) secondary to intake of this drug. CASE REPORT: A 58-year-old woman presented with a febrile skin rash and facial oedema, appearing 6weeks after the start of atorvastatin for dyslipidaemia. The clinical features associated disseminated polymorphic lesions, oral mucosa involvement and systemic symptoms (fever, abdominal pain, diarrhoea, polyarthralgia and adenomegaly). Blood tests showed hypereosinophilia up to 11,540/mm(3), inflammatory syndrome and anicteric cholestasis without cytolysis. Serological tests for hepatitis B and C, HIV, EBV, HHV-6, HHV-8, CMV and human Parvovirus B-19 were negative. Cutaneous histology was unspecific. A diagnosis of DRESS secondary to atorvastatin was suspected. The clinical outcome was favourable after atorvastatin discontinuation. DISCUSSION: To our knowledge, this is the first description of atorvastatin inducing DRESS, a severe life-threatening drug eruption. Atorvastatin has previously been implicated in various cutaneous adverse events. Because of their potentially serious side effects, prescription of statins must be carefully evaluated.

[Clinical and genetic study in 22 patients with basal cell nevus syndrome]. / Etude clinique et recherche de mutations germinales du gène PTCH 1 dans le syndrome des hamartomes basocellulaires.

BACKGROUND: Nevoid basal cell carcinoma syndrome is an autosomal dominant disorder characterized by developmental abnormalities and cancer predisposition. The PTCH 1 gene, the human homolog of the Drosophila segment polarity gene patched, has been shown to be involved in the development of nevoid basal cell carcinoma syndrome. PTCH 1 is mapped to chromosome 9q22.3. The aim of the present study was to report on clinical and genetic characteristics in patients followed for nevoid basal cell carcinoma syndrome and to compare them to the data in the literature. PATIENTS AND METHODS: Screening for PTCH 1 mutations was done in 22 patients followed between 1981 and 2003 for clinical suspicion of nevoid basal cell carcinoma syndrome. Clinical and radiological data were reviewed retrospectively from records. Genetic analysis was performed using blood samples after patient informed consent was obtained. When possible, DNA was also analyzed from the parents of patients in whom PTCH 1 mutations were found. RESULTS: All patients had developed basal cell carcinomas: 45% palmar and plantar pitting, 62% jaw cysts and 66% calcification of falx cerebri. Medulloblastomas and meningiomas were the most common associated tumors. PTCH 1 mutations were identified in 13 patients: 6 familial cases, 3 sporadic cases and for 4 patients, it was not possible to conclude. Nine different new germ-line mutations were identified. DISCUSSION: Genetic analysis allows molecular confirmation of diagnosis in about half of all patients. Early diagnosis is essential for detection of clinical and radiological manifestations in young patients and for provision of advice concerning protection of the skin from the sunlight.

Awareness, knowledge and attitudes towards sun protection among skin cancer-treated patients in France.

BACKGROUND: The incidence of skin cancer has risen over the past years, owing to increased exposure to ultraviolet radiation. Sun protection measures include avoiding exposure to the sun, using covering clothing and applying sunscreen. We evaluated the knowledge and compliance with advices about sun protection in a population of patients who had presented skin tumour(s). SUBJECTS AND METHODS: A 30 question self-reporting questionnaire evaluating sun protective behaviour was distributed to 217 consecutive skin cancer-treated patients and completed by 198 of them. RESULTS: 72% of the responders had presented a melanoma, and 26% of them had presented only non-melanoma skin cancer. The present survey shows that patients who have had a skin cancer were aware of the cancer related risk of sunlight since 98% of the responders knew that ultraviolet radiations can include skin cancer. These patients did also take sun-protective measures because 73% of them had worn covering clothes when in the sun and 59% of them avoided outdoor activities during the midday hours. CONCLUSION: These results suggest that, after diagnosis of a skin cancer, patients limited their sun exposure; and wear protective clothing. However, sun-protection measures did not seem to be completely adequate. An evaluation of the various barriers to sun safety might be a key to understanding the sub-optimal sun protection.