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The Vogt-Koyanagi-Harada disease is an unusual affection, characterized by a meningeal syndrome, an ocular syndrome involving the anterior or posterior segment of the eye, and at last, manifestations of skin involvement such as poliosis, vitiligo and alopecia. Nowadays, the physiopathologic hypothesis, the most admitted, is the one of auto-immune process regarding the uveal pigment. Nosologically speaking, clinical epidemiologic and etiopathogenic arguments have allowed to join the Harada and Vogt-Koyanagi diseases under the same name: the Vogt-Koyanagi-Harada disease. The same arguments, except the epidemiologic ones, allow to make a similarity between this affection and uveo-meningitis on the hand, sympathetic ophthalmitis on the other hand. The use of oral corticosteröids and eventually immuno-suppressive agents constitute the only therapeutic methods, nowadays.

A case of diffuse plane xanthomatosis assoicated with systemic amyloidosis and multiple myeloma at its outset is reported. Plane xanthomatosis is certainly an autonomous entity in comparison with systemic amyloidosis, for there are no amyloid deposits in xanthoma. The patient had lambda type IgG paraproteinemia, with Bence-Jones proteinuria. Lipid tests were considered as normolipemic though some levels recall a type IV hyperlipoproteinaemia. A review of literature about the association "xanthomatosis-multiple myeloma" was made, after the important work of Bazex, Dupré and Mrs. Christol-Jalby. It allows us to distinguish two differnet descriptions: 1. When there is hyperlipoproteinemia, all clinical types of xanthomas may exist; multiple myeloma is generally typical (but sometimes not very progressive). 2. When there is normolipidemia, the main clinical type is diffuse plane xanthomatosis; multiple myeloma is atypical and often only a monoclonal gammapathy is found. 3. However in both cases, the outstanding clinical type is diffuse plane xanthomatosis: whether normo- or hyperlipemic, this therefore indicates a possible underlying disease, and above all a multiple myeloma.

The possibility of cutaneous manifestations in Down's syndromes not a surprise because we known that this affection affects the three embryonic parts. This cutaneous disorder is described by many authors but it is variable and not specific. The authors had occasion to see four cases of alopecia areata in mongolian children. They do an analytic study of variable manifestations. They insist on frequency of hyperkeratotic lesions, alopecia areata, alterations of vasomotricity and elastic dystrophies. In the group of chromosomic aberrations, down's syndrome is alone where the cutaneous manifestations are studied because the patients have a good survival.

The authors report the observation of pyoderma gangrenosum (P. G.) leading to the discovery of an underlying biclonal gammapathy, which despite the absence of bony lesions, almost certainly represents a malignant myeloma. The authors have reviewed the different known associations with pyoderma gangrenosum, with special reference to benign and malignant gammapathies. Rare cases of biclonal gammapathies with pyoderma gangrenosum have been published but none of a malignant nature. This therefore appears to be the first reported cases. The authors are aware of the reported tendency of pyoderma gangrenosum lesions to appear in areas of previous trauma, as may be the case in this patient. Finally systemic corticosteroid therapy produced a rapid remission of the skin lesions, but with the reactivation of known treated pulmonary tuberculosis.

The authors have analyzed the results of cultures in Sabouraud's agar performed over a 20-year period in 6,000 patients with lesions of the skin, skin appendages and mucosae of suspected fungal origin. Throughout this period all patients, almost exclusively urban or suburban, were seen at the Dermatology out-patient consultation of a central city hospital. The 6,000 specimens were analyzed globally, with special attention to time-related changes in the 10 groups of 6,000 specimens each whenever a significant modification was observed. The analysis was divided into two parts: a review of the fungi isolated, of the manifestations they produced and of the time-related changes in their respective prevalence; a study of the fungi responsible for the main dermatological syndromes. Out of 6,000 cultures, 2,502 strains of pathogenic fungi were isolated, representing a growth rate coefficient of 41.66 p. 100. There were 1,237 dermatophytes and 1,265 yeasts of the Candida group. However, this overall impression of two equal groups gives a wrong idea of the changes that occurred during this long period. Figure 1 clearly shows that in 20 years the number of dermatophytes increased whereas the number of Candida decreased. Figures 2 and 3 demonstrate that the rise in dermatophytes was almost exclusively due to an increase in T. rubrum and the fall in yeasts, to a decrease in Candida albicans.(ABSTRACT TRUNCATED AT 250 WORDS)

The authors have studied clinically and histologically two cases of " erythrosis interfollicularis colli de Leder ". It's a frequent affection, principally between 40 and 50 years of age but not very known because the patients rarely consult for it. Clinically they insist on the respect of a triangulary and lozengical median zone, and on the sight in "plucked chicken skin"; histologically on the dilatation of sebaceous glands, the frequency of a folliculary corneous plug and the elastic tissue alterations. On the etiological plan, the part of the age, the sun exposure, and in their two cases on the possible photosensibilisative part of the scents.

The authors have recently come across a female patient with pseudo-pyogenic granuloma and have taken this opportunity to recall the clinical and histological features of this disease which was described in 1969 by Wilson Jones and Bleehein. In this connection, they have studied the relationship of this disease with similar entities, i.e. subcutaneous angiolymphoid hyperplasia with eosinophilia (described by Wells and Whinster in 1969) on the one hand and kimura's disease (described by Kimura in 1948) on the other hand. Whereas subcutaneous angiolymphoid hyperplasia with eosinophilia seems to be an evolutive form developing from pseudopyogenic granuloma, Kimura's disease differs from it clinically and histologically and is considered as a borderline form.

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.

The authors make a survey of a series of a hundred Becker's nevi diagnosed in a recruiting center of the French National Service. It is a statistic study bearing on the male population, aged 17 to 26, in a French region which is supposed to be representative of the French male population of the same age. First of all the authors focus on the various clinical descriptions since this nevus was first described by Becker in 1949, then discuss the problems raised by pathogenic interpretation and the results of microscopic studies. About this lesion, usually said to be pigmentary and hairy, and generally appearing before 20 years of age, is still very little known. Then after a short explanation of their method of study the authors give original results. A hundred Becker's nevi were diagnosed among 19,302 young men, that is to say in a proportion of 0.52 p. 100. A study of the racial, genetic and pigmentary background doesn't enable to retain general causes but rather leads to think of local causes. Half of these Becker's nevi appeared before ten years of age, the other half appearing between 10 and 20. In only one quarter of the cases can the role of the sun be pointed out. The location of the nevi is variable; they are mainly situated on the trunk and only 32 p. 100 of them are located above nipples, which was believed to be the usual place of the lesion. Pigmentation in three quarter of the cases is light brown. The average surface is 125 cm2. Hairs can be seen in only a little more than half of the cases of no lesion associated to the nevus could be found. The study points out that Becker's nevi seem to be more various in their clinical expression and more pigment than could be thought through more classical description.

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.

The fragility of epidermal cells in Hailey-Hailey's disease end even more in Darier's disease seems to favour the development of viral infections. The authors present six cases of herpes virus infections in three siblings with an intermediate form between Darier's disease and Hailey-Hailey's disease, both having a tendency to recur. One of the flares demonstrated signs of Kaposi and Juliusberg's pustulosis vacciniformis which is often found in the literature as a complication of Darier's disease. The authors discuss the frequency of the recurrences, which could be due to different viruses. Herpetic virus is often found, less frequently vaccinial virus, but sometimes also Coxsackie A 16 virus.

Among the systemic manifestations of Behçet's syndrome, venous and arterial involvements are discussed. Venous thrombosis is a common manifestation and may be considered as a cardinal sign in the diagnosis. Venous and arterial thrombotic complications may be regarded as agents of morbidity and mortality. The pathogenesis of the occlusive process is still some what obscure, although vasculitis and impaired local vascular fibrinolytic activity play a part in it. Therapeutic measures using fibrinolytic enhancing agents may prevent and control the thrombotic complications of the disease.

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 demonstrate, with the help of electron microscopy, the location of the polymorphonuclear neutrophils and of the keratinocytes in the Kogöj's pustule. Unlike to the classical ideas, the polymorphonuclear neutrophils are not located in the epidermal cells but in the intercellular spaces. The lesions of keratinocytes, the content of the spaces and the histogenesis have been studied. The results of this study are compared to thoses formed in the literature.

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.

With regard to 2 cases of a same family associating: multiple lentigos, osseous signs and impairment of E.C.G., the authors recall the differents visceral manifestations which can be associated to lentigos and the relative frequency of every one of them. The authors report 38 indisputable observations of the literature that associated the 2 criterions: multiple lentigos symmetrical in their distribution and histological confirmation of sun spots. The authors perfer the term: multiple lentigos syndrome to the LEOPARD syndrome. The last term (LEOPARD), in spite of his attractive character contains a part of possible associated signs (osseous signs, primordial in our observations, are not present in that syndrome). But another way, contrary to, not any observation, up to now, allowed all the symptomes present in the acrostic LEOPARD. Through it should appear difficult to define actually the scope of the multiple lentigos syndrome, the knowledge of that entity offers a real interest for the dermopath: the discovery of a symmetrical lentiginosis, extensive or not, must lead to the exploration, as well as possible, of organs susceptible to be out of order: heart, nervous system and hearing, skeleton and facial malformations, genital-organs, and also to a family inquiry.