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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.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.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.

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.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.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.

We report a case of neuritic borderline tuberculoid leprosy with lingering insidious growth disclosed by a brutal reversal reaction. Inflammatory polyarthralgia and sensory and motor loss in the median and ulnar territories, without skin lesions, suggested vasculitis. A few weeks later, inflammatory skin lesions developed leading to the diagnosis of biopsy proven leprosy. Leprosy should be considered as a possible diagnosis in patients with multineuritis, especially when associated with rheumatic or cutaneous manifestations. Early skin biopsy in neuritic leprosy is discussed.

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.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.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.

African tick-bite fever is caused by R. africae. All cases reported to date in France have occurred among patients who came from southern Africa, the endemic zone. We report the first case, to our knowledge, of a patient infected in France.A 69-year-old white man who had never left France developed fever and 3 "black spots" on the legs with lymphangitis and enlarged nodes. The clinically suspected diagnosis was confirmed by positive serology reactions to R. africae. As cross immunity with R. conorii can occur, the diagnosis was further confirmed by western blot for R. africae and by the persistence of the reaction after adsorption of the R. conorii serum.African tick-bite fever was identified as a clinical entity different from Mediterranean spotted fever by Kelly in 1992 who demonstrated the causal role of R. africae. The typical clinical presentation associates fever, several black spots, lymphangitis and multiple node enlargement. The serological diagnosis is difficult owing to cross immunity with R. conorii. Western blot and polymerase chain reaction are required for definitive diagnosis. In our case, the infection was probably due to a R. africae imported into France in the luggage of the patient's daughter who had spent 3 months in Zimbabwe.

Deep or infiltrating lipoma is an often misdiagnosed clinical entity. We report two typical cases.Two women, aged 92 and 62 years, were seen for a tumefaction on an upper limb with progressively increasing volume. The clinical presentation suggested deep lipoma, confirmed by magnetic resonance imaging and histology which eliminated liposarcoma, the main differential diagnosis. Surgical excision was successful. There has been no recurrence.These two observations recall the clinical, diagnostic and therapeutic features of infiltrating lipoma.

The pathophysiology of mycosis fungoides remains uncertain but HTLV I or a similar virus could be involved. We observed a couple who developed mycosis fungoides suggesting the infectious hypothesis might indeed be valid.A 70-year-old man who had often travelled in foreign countries developed parapsoriasis en plaques, lymphomatoid papulosis and mycosis fungoides successively over a thirty year period. Several years after the first manifestation of mycosis fungoides, his wife also developed a single plaque of mycosis fungoides. The diagnosis was confirmed on pathology slides and immunohistochemistry tests as well as on the basis of T-receptor gene rearrangement in both patients. Search for HTLV I was negative using serology tests and PCR on circulating lymphocytes.The epidemiological situation in our observation (several trips in foreign countries and the delayed development of mycosis fungoides in the wife) favours the hypothesis of an infectious mechanism. Search for HTLV I was unsuccessful with classical virology methods. Certain recent work suggests a virus similar but different from the HTLV I virus could be involved in the pathogenesis of mycosis fungoides.

Cutaneous larva migrans is rarely contracted in temperate countries.When his house became flooded, he had to stand for a long period of time with mud up to the thigh. Some days later, he developed multiple erythematous, serpiginous pruritic tracts moving 1-2 cm per day over preexisting lesions of the right leg. Local and systemic treatment with thiabendazole led to rapid and definitive cure.Cutaneous larva migrans results from the migration of hookworm larvae in the dead-end human host. It is mainly an imported disease and native cases in Europe as reported here are rare. This case demonstrates that the conditions leading to the development of cutaneous larva migrans are rarely found simultaneously in temperate zones.

Forty refugees (23 from 5 lao families and 17 from 3 cambodian families) previously and routinely treated against intestinal parasites, underwent stools and serum controls: 77 p. 100 had still intestinal parasites but 61 p. 100 had a normal blood cells count without hypereosinophilia. The parasites so detected were: Clonorchis sinensis, Heterophyes heterophyes, Trichuris trichiura, Necator americanus, Strongyloides stercoralis, Giardia intestinals and Entamoeba coli.