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On the basis of three cases, the authors describe the main changes seen in the nails in Bourneville tuberose sclerosis, not only in fingers with a Koenen tumour but also in association with simple cuticular hyperkeratoses and in fingers with no apparent lesion of the matriceal region. They feel that the description of these ungual lesions should take its place alongside the major cutaneous signs of this disorder.

The authors report five new cases associating basal cell epithelioma and multiple Pinkus fibro-epithelial tumors on the spine after radiation damage to the skin. One of them had previously published three similar observations: so his experience refers to eight cases. The authors recall similar cases of the literature, they discuss the relations between basal cell epitheliomas and Pinkus premalignant fibro-epithelial tumors of the skin. Histologically they saw several aspects of transition between the two types of tumors. They study the patterns of cancers found after radiation therapy, influence of type of radiations, dose, age, and intervals since X-rays. As regards such cases, the necessity of systematically searching for previous radiations by X-rays in the affected zone is emphasized.

We report a case of Bazex-Dupré-Christol (BDC) syndrome in a girl who died of prolymphocytic leukaemia at the age of 10 years. This is the first published case where the syndrome is associated with a blood disease. A review of the literature has enabled us to collect the various symptoms of BDC syndrome and to discuss the relationship of the syndrome with basal cell nevomatosis. We do not think that the blood disease was incidental. The child was 5 years' old in June 1972 when she was first seen in our out-patient clinic for severe hypotrichosis which had been present at birth and involved the eyelashes and eyebrows. Examination of the skin showed numerous milia on the face, a small lenticular polycystic lesion on the right ear, follicular atrophodermia on the back of the hands and hypotrichosis of the face. The right ear lesion was removed and found to look like Winer's cutaneous calcinosis. At light microscopy, many hairs showed like pili torti or, more rarely, like trichorrhexis nodosa. Despite the absence of basal cell carcinoma, the diagnosis of BDC syndrome was regarded as final. The patient died aged 10 years of prolymphocytic leukaemia revealed by pancytopenia. Combined chemotherapy with Rubidomycin, Oncovin and methotrexate had proved ineffective. Having collected from the literature 44 adequately documented cases summarized in table I, we describe the anatomico-clinical manifestations of Bazex-Dupré-Christol syndrome. Follicular atrophodermia is present in 85 p. 100 of the cases, but its date and mode of onset are difficult to evaluate.(ABSTRACT TRUNCATED AT 250 WORDS)

The authors report 15 cases of multiple epitheliomas of the back, concerning patients who had been previously submitted to X-ray irradiations. Their cases are divided into two groups: the first comprises 11 patients who had undergone lumbar and sacral anti-inflammatory radiotherapy for rheumatologic diseases; the second concerns 4 patients who had had many fluoroscopic examinations for lung tuberculosis and later developed multiple epitheliomas of the back. For each group of patients, they detail the irradiation procedures, and indicate the interval before the subsequent tumoral development, as well as the histologic features. In both groups, basal cell epitheliomas prevail with any type of histologic pattern (mainly pagetoid, however). Fibroepithelial tumours of Pinkus are not more numerous in the lumbar area (on the contrary: 20 p. 100 against 28.57 p. 100). Precancerous keratoses and Bowen-like conditions are only found in the second group (in fact in a single patient). The important feature is not the topography but the dose of irradiation. Fourteen out of 15 patients had only basal cell epitheliomas and fibroepithelial tumours.

The authors report 4 cases of multiple tumors of the lumbo-sacral region with, on histology, a mixture of basal cell carcinomas and fibro-epithelial Pinkus tumours. They were struck by the fact that three of these four patients had received, in this region, radiotherapy numerous years previously. They discuss the role of this radiotherapy in the onset of the disease and the relative importance of this factor and of constitutional factors (Hamartomas). They recall the characteristics of multiple basal cell carcinomas and of Pinkus fibro-epithelial tumours, together with the close relationship between these two types of tumour.