[Basal cell carcinoma of the vulva: 21 cases]. / Carcinome basocellulaire de la vulve: 21 cas.

INTRODUCTION: Basal cell carcinoma (BCC) accounts for 2 to 3 p. 100 of all vulvar malignancies. PATIENTS AND METHODS: We report a retrospective study of 21 cases treated from 1937 to 1999. RESULTS: Vulvar BCC's occurred in elderly patients (average age: 66 years), with mean delay to diagnosis of 5.5 years. Five patients were referred for recurrence. Lesions were located on the external hairy side of the labia majus, except one located on the internal side. In 4 cases a preexisting risk factor was identified: 2 patients had previously received radiation therapy and 2 other patients had multiple disseminated BCC. Mean BCC diameter was 2 cm. Pathological data were similar to skin BCC, with one case of mixed tumor (BCC and squamous cell carcinoma). The treatment was surgical excision for 19 BCCs. Local recurrence risk was high. Only one patient died of visceral dissemination of the disease. DISCUSSION: More than 250 cases of vulvar BCC have been reported in the literature. Clinical, pathological and follow up data are similar to results in the present series. Treatment of choice consists of surgical excision with tumor-free margins. Because of local recurrence risk and possible association with other primary cancers in this age group, long term follow-up is necessary.

[Association of lichen sclerosus and monomelic scleroderma]. / Association sur un membre d'un lichen scléreux et d'une sclérodermie monomélique.

INTRODUCTION: Coexistence of lichen sclerosus and scleroderma is well demonstrated. However, clinical and histological lesions of lichen sclerosus and scleroderma, in a linear pattern, do not seem to have been reported. CASE REPORT: We report the case in a six years old boy, of both lichen sclerosus and linear scleroderma lesion that involved his left lower limb. Immunological and inflammatory investigation was normal or negative, as well as, bone and muscles analyze of the leg. Further outcome was benign. DISCUSSION: This new case confirms the possibility of an association, inside a same limb, of lichen sclerosus and localized scleroderma, and supports the concept of a common etiological process in these two diseases. In an other hand, it could reflect the possibility of two distinct clinical expressions of the same pathogenic process.

DNA flow-cytometric analysis of basal cell carcinomas and its relevance to their morphological differentiation: a retrospective study.

DNA flow cytometry (FCM) was performed on archived material of 82 basal cell carcinomas (BCC), using Hedley's technique. Sixty-five samples were analysed. Biopsy samples were classified in five groups depending on their morphological differentiation: solid, cystic, adenoid, keratotic and metatypical. Two parameters were studied: proliferative index and DNA index. Statistical analysis revealed that BCC with keratinizing or metatypical differentiation had a greater amount of aneuploidy when compared with BCC without particular differentiation (solid and cystic) or aneuploid differentiation (p < 0.001). The proliferative indices were significantly different in the four groups. DNA FCM of BCC confirms the value of morphological classifications and gives another example of aneuploidy in a tumour with low aggressiveness.

New electron microscopic findings in a case of multicentric reticulohistiocytosis. Long spacing collagen inclusions.

The case of a 68-year-old woman with polyarthropathy and skin nodules is presented. Histologic findings of histiocytes with ground-glass cytoplasm favored the diagnosis of multicentric reticulohistiocytosis. In this article we report new electron microscopic findings of intra- and extracytoplasmic long spacing collagen (type VI). Type VI collagen inclusions are usually found in lymphohistiocytic neoplasms. We believe our findings support the concept of a proliferative rather than inflammatory etiology for multicentric reticulohistiocytosis. It may also give support for the argument of production of collagen by the histiocytic cells of multicentric reticulohistiocytosis.

Methotrexate treatment of multicentric reticulohistiocytosis.

We describe a 68-year-old woman with multicentric reticulohistiocytosis. The patient's symptoms responded well to methotrexate 7.5 and then 10 mg/week orally. After 34 months of treatment, her disease was still in remission.

[Preputial sclero-atrophic lichen in children]. / Lichen scléro-atrophique préputial de l'enfant.

Clinicopathological findings and outcomes in ten pediatric cases of lichen sclerosus and atrophicus of the foreskin are reported. This well-defined entity usually presents as acquired sclerous phimosis. Diagnosis rests on histologic studies of biopsy specimens. Pathologic features change during the course of disease and are useful for selecting the most appropriate therapy. Local corticosteroid therapy may be helpful in early disease. However, circumcision is usually required for advanced disease, failure of medical therapy or prevention of squamous cell carcinoma. Pathophysiology is discussed. The incidence of this condition seems to be underestimated. Possible connections with diabetes mellitus and other conditions are reviewed.

[Digestive involvement in dystrophic recessive epidermolysis bullosa. Apropos of 6 cases and review of the literature]. / Atteinte digestive au cours de l'épidermolyse bulleuse dystrophique récessive. A propos de six cas et d'une revue de la littérature.

Six personal cases of digestive tract involvement in dystrophic recessive epidermolysis bullosa are reported, and the relevant literature is reviewed. The study deals with the clinical aspects of these cases (buccal and dental lesions, digestive symptoms, effects on nutritional status; table I), as well as with their biochemical (table II), radiological and endoscopic aspects (table III, fig. 1 and 2). All patients presented with bucco-dental lesions, including two cases of congenital abnormalities: one with malposition and dysgenesis of the teeth, the other with dysplasia of the enamel in a patient whose dystrophic skin disease was proven by electron microscopic study. The oesophagus was involved in six cases, with tight concentric stenosis (2 cases), retrocricoidal stenosis (1 case) and oesophagitis (2 cases). No gastro-duodenal or intestinal lesions were detected. A case of constipation was related to anal involvement. The patients' nutritional status was investigated clinically and biochemically. A search for intestinal malabsorption by means of specific tests was conducted in 2 patients and proved negative. A study of the literature provided data on the nature and specificity of dental lesions. The morphological features, complications and physiopathology of oesophageal stenoses are described The existence of gastrointestinal lesions is discussed. Nutritional repercussions are presented and their causes are discussed. Attention is paid to the medical and surgical treatments of these lesions.