Search

Resume Introduction Nous decrivons le cas d’un nourrisson qui presentait un nodule cutane congenital unique d’histiocytose langerhansienne. Rapportee sous des appellations variees, cette lesion benigne est rare et sa prise en charge n’est pas consensuelle. Observation Un nourrisson de 28 jours presentait un nodule congenital unique brunâtre centimetrique du flanc gauche. La biopsie du nodule montrait une proliferation dermique d’histiocytes langerhansiens. L’evolution etait spontanement favorable, avec une rapide diminution de l’infiltration ; a 16 semaines, seule persistait une pigmentation sequellaire. Discussion Trente-huit lesions de ce type ont ete rapportees dans la litterature sous de multiples appellations. Il s’agissait de nodules solitaires brunâtres centimetriques, souvent ulceres, ubiquitaires, quasiment toujours congenitaux. L’aspect histologique etait une proliferation dermique d’histiocytes langerhansiens. L’analyse en microscopie electronique montrait le plus souvent des granules de Birbeck et des corps denses intracytoplasmiques. Les examens biologiques et radiologiques ne trouvaient jamais d’atteinte systemique. Les lesions non excisees chirurgicalement ont toutes regresse dans un delai moyen de huit semaines. L’histiocytome congenital semble devoir etre distingue des autres formes d’histiocytose car il ne necessite pas le meme type de prise en charge. L’auto-involution systematique de la lesion et l’absence d’atteinte systemique plaident en faveur d’une surveillance clinique simple, en reservant l’exerese chirurgicale et le bilan d’extension aux formes persistant a six mois.

Resume Introduction Les infections cutanees a Mycobacterium intracellulare sont rares et presque exclusivement decrites chez l’immunodeprime. Nous rapportons le cas d’une bursite chez un adulte immunocompetent. Observation Un homme de 80 ans, ayant pour principal antecedent une tuberculose pulmonaire, avait une tumefaction du coude depuis six mois, avec epanchement, correspondant a une bursite retro-olecrânienne. Cette lesion evoluait vers l’abcedation et l’ulceration en regard. Une ponction du liquide articulaire trouvait la presence de M. intracellulare. L’examen histologique mettait en evidence des granulomes epithelioides et gigantocellulaires. La mise en culture revelait la presence de M. intracellulare. La recherche d’une immunodepression etait negative. Une biantibiotherapie par clarithromycine et rifabutine etait efficace avec une cicatrisation partielle a trois mois, mais avec des effets secondaires hepatiques et oculaires et une rechute a l’arret de la rifabutine. L’association ethambutol–clarithromycine permettait d’obtenir ensuite une guerison en dix semaines. Discussion Des osteomyelites, des arthrites, ainsi que des infections cutanees a M. intracellulare ont ete decrites chez les immunodeprimes ; seules de rares observations ont rapporte la survenue d’infection chez des sujets immunocompetents. Ces observations soulevent la question d’une sensibilite individuelle aux infections a mycobacteries. Des defauts genetiques de l’axe interleukine-12–interferon gamma implique dans la reponse immunitaire aux infections a mycobacteries ont ete mis en evidence. Mycobacterium avium-intracellulare sont des mycobacteries resistantes aux antituberculeux classiques. Par analogie avec le traitement recommande pour les infections pulmonaires ou disseminees, il convient d’utiliser des molecules qui ont prouve leur efficacite in vitro et in vivo, en tenant compte de l’antibiogramme et des effets indesirables, notamment pour l’association clarithromycine et rifabutine : intolerance hepatique, uveite. Conclusion Cette observation montre l’interet, surtout si l’epanchement est post-traumatique, de la recherche systematique de mycobacteries par mise en culture du liquide articulaire et cela meme si le sujet n’est pas immunodeprime.

Relapsing polychondritis (RP) is a rare disorder characterized by recurrent inflammatory episodes involving various cartilages. The clinical course of RP is variable, and dermatologic manifestations are uncommon. We report a 73-year-old patient who presented with a neutrophilic dermatosis (Sweet's syndrome) as the initial manifestation of RP. There was no evidence for a myelodysplastic syndrome, as it has been previously reported with RP, but the patient was followed-up for an indolent and untreated chronic lymphocytic leukaemia. Complete remission was obtained with oral corticosteroids. This report highlights the clinical spectrum of the RP.

A few cases of sarcoidosis have been reported in the course of chronic hepatitis C, with or without alpha-interferon treatment. We report a 54-year-old woman who presented a typical sarcoidosis in this context. The sarcoidosis involved skin, lungs and eyes. There are data in the literature supporting a pathogenic link between alpha-interferon and sarcoidosis: alpha-interferon possessing a non-specific immunologic inductor. The treatment of the sarcoidosis in this context is difficult and has to be adjusted to both the severity of the sarcoidosis and the viral hepatitis. Although sarcoidosis induced by alpha-interferon is rare, clinicians must be aware of this association.

Relapsing polychondritis (RP) is a rare disorder characterized by recurrent inflammatory episodes involving various cartilages. The clinical course of RP is variable, and dermatologic manifestations are uncommon. We report a 73-year-old patient who presented with a neutrophilic dermatosis (Sweet's syndrome) as the initial manifestation of RP. There was no evidence for a myelodysplastic syndrome, as it has been previously reported with RP, but the patient was followed-up for an indolent and untreated chronic lymphocytic leukaemia. Complete remission was obtained with oral corticosteroids. This report highlights the clinical spectrum of the RP.

A few cases of sarcoidosis have been reported in the course of chronic hepatitis C, with or without alpha-interferon treatment. We report a 54-year-old woman who presented a typical sarcoidosis in this context. The sarcoidosis involved skin, lungs and eyes. There are data in the literature supporting a pathogenic link between alpha-interferon and sarcoidosis: alpha-interferon possessing a non-specific immunologic inductor. The treatment of the sarcoidosis in this context is difficult and has to be adjusted to both the severity of the sarcoidosis and the viral hepatitis. Although sarcoidosis induced by alpha-interferon is rare, clinicians must be aware of this association.

Numerous disorders can cause a systemic granulomatosis. We report a patient who presented a biopsy proven granulomatous skin eruption, fever, and atypical thoracic pain. Electrocardiogram showed a first-degree atrioventricular heart bloc. During follow-up he developed a panuveitis and oral corticosteroids were started. A diagnosis of systemic sarcoidosis was considered. Because of unfavourable ophthalmologic outcome, investigations were enlarged and revealed a highly positive serology for syphilis and VDRL both in serum and cerebrospinal fluid. Clinical outcome with penicillin G therapy was favorable. This observation reminds us the clinical polymorphism of syphilis, which can be presented as a systemic granulomatosis.

We report on a newborn presenting a solitary congenital skin nodule due to Langerhans cell histiocytosis. This benign lesion is rare and has been described in the literature under a variety of names; there is no consensus regarding treatment.A 28-day-old newborn presented with a solitary congenital blue-brown nodule measuring 1cm on the left iliac fossa. Histological examination of a skin biopsy showed a proliferation of histiocytes throughout the dermis with an immunohistochemical profile of Langerhans cells. The lesion resolved spontaneously, with rapid reduction of the infiltration, and at 16 weeks only a pigmented scar remained.Thirty-eight similar cases have been reported in the literature under a variety of different names. The lesions noted consisted of a solitary brownish nodule measuring 1cm which was congenital in almost all cases, often ulcerated and exhibited no predilection for any particular body site. Histological examination revealed dermal proliferation of Langerhans cells. Electronic microscopy revealed dense intracytoplasmic bodies and Birbeck's granules. Laboratory and radiological tests did not show systemic involvement in any cases. All lesions other than those surgically removed regressed spontaneously within a mean 8 weeks. It appears necessary to differentiate between congenital and other forms of histiocytoma since therapeutic strategies differ. Spontaneous resolution of lesions and lack of systemic involvement militate in favour of simple clinical follow-up, with surgical excision and further tests required only for cases lasting 6 months and beyond.

Cutaneous infections due to Mycobacterium intracellulare are infrequent. We report a case of bursitis in an immunocompetent patient.A 80-year-old patient with a history of tuberculosis reported swelling of the elbow for six months with effusion due to olecranon bursitis, progressing to an abscess and skin ulceration. Examination of the synovial fluid revealed the presence of M. intracellulare. Histologic investigations showed epithelioid and gigantocellular granulations. Screening for immunodepression was negative. Treatment with clarithromycin and rifabutin proved effective, with partial healing at three months, although hepatic and ocular side effects occurred.While osteomyelitis, arthritis and cutaneous infections due to M. intracellulare have been described in immunocompromised patients, there are very few reported cases concerning immunocompetent individuals, and these suggest the possibility of special individual sensitivity to mycobacterial infections. Genetic mutations of the interleukin-12-interferon-gamma pathway involved in the immune response to mycobacterial infections have been demonstrated. Autoantibodies against interferon-gamma have also been described. Mycobacterium avium-intracellulare is resistant to the classical antibiotics used in tuberculosis. As in pulmonary infections, antibiotics with proven in vivo and in vitro efficacy must be used, taking into account the antibiogram and potential side effects, particularly for combined clarithromycin and rifabutin.This case report emphasizes the benefits of routine mycobacterial investigation of synovial fluid samples.