[Sweet's syndrome as the presenting manifestation of relapsing polychondritis]. / Syndrome de Sweet révélant une polychondrite atrophiante.

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.

[Congenital histiocytoma: a benign disease]. / Histiocytome congénital: une lésion bénigne.

BACKGROUND: 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. CASE REPORT: 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. DISCUSSION: 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.

[Sarcoidosis-like granulomatosis revealing a tertiary syphilis]. / Granulomatose pseudosarcoïdosique révélant une syphilis tertiaire.

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.