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Background: The term "auto-inflammatory diseases" encompasses a group of disorders characterised by febrile episodes of sudden onset associated with joint, abdominal, lymph node and cutaneous signs, each presenting a genetic and/or laboratory specificity allowing their identification. Polyclonal elevation of serum IgD is highly suggestive of hyper-IgD syndrome, a disease with autosomal recessive transmission that usually begins before the age of one year., Case Report: We report the case of a 46-year-old woman presenting a disease since the age of 30 years clinically very similar to hyper-IgD syndrome except for ocular and pulmonary involvement. However, tests revealed neither mevalonate kinase gene mutation nor elevation of urinary mevalonic acid. The near-monthly attacks were controlled with mycophenolate mofetil., Discussion: Three cases with identical clinical and laboratory profiles have been reported in the literature and diagnosed as late-onset hyper-IgD syndrome. Our case is the only one involving indirect screening for mutation of the mevalonate kinase gene, which proved negative. This finding rules out the diagnosis of classic hyper-IgD syndrome in our case and raises the possibility of auto-inflammatory disease with reactional hyper-IgD of a different cause, either acquired or secondary to an as yet unidentified mutation.

Introduction: Impetigo herpetiformis is a rare dermatitis that occurs during pregnancy and may be life threatening for both mother and child. In this case report, we present an Ondine curse involving the baby, and the good response to isotretinoine., Case Report: A first pregnancy, 26 year-old woman developed at 8 months a widespread skin lesion involving the medial side of the thighs, abdomen and intertriginous areas, with a severe systemic toxic condition and fever. Diagnosis of impetigo herpetiformis was made and corticosteroids, methotrexate and cyclosporine were unsuccessful. Isotretinoine rapidly improved the patient with good control of the disease. The full term baby had an Ondine curse., Discussion: Our case is typical of impetigo herpetiformis. Maternal and infant complication may be life threatening and we report a real Ondine curse the etiology of which remains unknown. Moreover, this observation is unusual because the lesions did not clear despite delivery and good treatment. In our opinion, the great improvement with isotretinoine would suggest it could be used as first line treatment.

Background: The recent outbreak of an epidemic of West-Nile fever in New-York revealed this virus' responsibility in some cases of fatal encephalitis. However the clinical manifestations of the infection may be mild as in the case reported herein., Case-Report: A 41-year-old woman without previous medical history presented an acute febrile rash after a trip to Senegal. The course was spontaneously favorable after 5 days, with a biphasic fever at day 3. Reverse passive hemagglutination test confirmed the diagnosis of acute West-Nile fever., Discussion: West-Nile virus, a mosquito-borne flavivirus is usually responsible for a flue-like disease presenting a maculopapular rash in half of the cases. The biphasic evolution of fever is suggestive of the diagnosis. The diagnosis relies on serological tests but cross-reactivities with heterologous flaviviruses may render the interpretation difficult. A rapid recovery is usual but fatal meningoencephalitis can occur especially in the elderly. Treatment is supportive and prevention is limitation of human exposure. West-Nile virus is widely distributed (Africa, Asia, Middle East), but is also endemic in european areas and responsible for seasonal infections.