[Pseudoxanthoma elasticum and nephrocalcinosis]. / Pseudoxanthome élastique et néphrocalcinose.

INTRODUCTION: Pseudoxanthoma elasticum (PXE) is a hereditary disease that presents clinically as cutaneous, ocular and cardiovascular problems. The associated clinical signs are due to calcification of elastic fibres and blood vessels. The case we describe below involves a 24 year-old woman hospitalised for pseudoxanthoma elasticum and also diagnosed with asymptomatic renal nephrocalcinosis. OBSERVATION: A diagnosis of pseudoxanthoma elasticum was suspected in this patient based upon yellowish papules on the head and neckline together with angioid streaks. This diagnosis was confirmed by histological examination of the skin biopsy sample. Levels of calcium and phosphorus in blood and urine were normal, as were the cardiovascular test results. Abdominal ultrasound revealed bilateral nephrocalcinosis. DISCUSSION: This case is original in terms of the association of pseudoxanthoma elasticum with nephrocalcinosis. There are in fact increasing reports of diffuse visceral calcifications in pseudoxanthoma elasticum and the literature contains reports of association with mammary and testicular calcifications. To our knowledge, associated nephrocalcinosis has only been described in two subjects in a series of 11 PXE patients. The question is whether this association was entirely fortuitous or if it in fact constitutes a rare manifestation of the disease.

[Ten cases of Verneuil's disease]. / La maladie de Verneuil: dix cas.

INTRODUCTION: Verneuil's disease is a chronic inflammatory, suppurating and fistulizing disease. It is often misdiagnosed and taken for another pathology. The aim of this study was to describe the epidemiological, clinical, and therapeutic features of 10 patients presenting with this disease, in our dermatology unit. PATIENTS AND METHODS: We retrospectively analyzed 10 observations of Verneuil's disease from 1994 to 2005. We studied the clinical, epidemiological, and therapeutic characteristics. RESULTS: Nine male and one female patients were included. The mean age was 35 years. The mean delay between onset of symptoms and diagnosis was 6 years. All patients presented with a severe form of the disease. The localizations were the buttocks in 4 cases, the pubis in one case, and the axilla in another case. Four patients also presented with buttock and anoperineal lesions. The medical treatment was antiseptics, antibiotic therapy, or isotretinoin. All patients were treated surgically. CONCLUSION: A suppurated and fistulized nodule on an anoperineal or axillary location must suggest this disease. Our series is characterized by the prevalence of men and the frequency of severe forms.

[Pseudoainhum and peripheral neuropathy]. / Pseudo-aïnhum et neuropathie axonale.

BACKGROUND: Pseudoainhum is a rare disease characterised by gradual fibrous constriction of the fingers and/or toes eventually resulting in their amputation. In this article, we report the first case seen in Morocco, highly unusual in terms of its severity. CASE REPORT: A 46-year-old woman with no toxic habits was hospitalised for spontaneous amputation of the fingers and toes. This condition began when the patient was 12 years old with the appearance of a circular constriction band at the base of the fifth toe, eventually resulting in its loss. The patient gradually lost all her other toes and fingers except for the first joint of her left index finger. There was no family history of any similar condition. Clinical examination also revealed perforating plantar disease in two of the stumps and peripheral neuropathy in all four limbs, comprising predominantly axonal disease responsive to electromyogram. Amputation of the index finger was completed and histological examination of the removed section showed nothing unusual, with no signs of diabetes. DISCUSSION: The peculiarity of our case resides in the exceptional severity of these amputations and their association with isolated polyneuropathy. Pseudoainhum has been described chiefly in patients with congenital keratoderma, certain systemic diseases, diabetes and alcohol dependence. A number of etiopathogenic hypotheses have been suggested: traumatic, infectious, vascular, neurological and genetic processes.

[Descending necrotizing mediastinitis: a diagnosis not to miss]. / La cellulite cervico-médiastinale nécrosante: un diagnostic à ne pas méconnaître.

Descending necrotizing mediastinitis is a severe disease which occurs after a mild otorhinolaryngologic or dental infection. The diagnosis must be established rapidly with the help of clinical and computed tomography of the neck and chest data. Treatment is based on antibiotics, surgery and hyperbaric oxygen. The outcome is poor with high mortality. We report a case of septicemia complicating descending necrotizing mediastinitis after dental infection.