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We report the case of a young woman whose son had X-linked chronic granulomatous disease (CGD) while she was gene transmitter and presented with erythematous-squamous dermatosis predominant in unprotected regions, photosensitivity and oral ulcerations. The diagnosis of discoid lupus erythematosus, suggested by the clinical complex, was not confirmed by paraclinical examinations. CGD is an X-linked or, less often, autosomal recessive disease underlain by a selective deficiency of intraleucocytic bactericidal ability. Its clinical manifestations are repeated and severe infections involving most of the body organs. A review of the literature yielded 20 cases resembling that of our patient; all concerned mothers or sisters of children with X-linked CGD. The skin disease usually begins during childhood. The authors describe cutaneous manifestations as a photosensitive infiltrating erythema, frequently suggestive of lupus erythematosus, and oral ulcerations. At light microscopy, these skin lesions show a lymphohistiocytic infiltrate which sometimes erodes the basement membrane or remains at a distance from it. Such images suggest lupus erythematosus or, for some authors, a Jessner-Kanoff syndrome or a lichenoid infiltrate. Direct immunofluorescence was negative in all but one case, and there was usually no laboratory evidence of autoimmunity. In women who transmit the X-linked form of the disease, the bactericidal activity of granulocytes is reduced by 50 p. 100, but infection is not a major problem. The pathogenesis of these disorders is not yet fully understood. However, we would like to draw attention to the studies by Roberts et al. who demonstrated a defective degradation of bacterial DNA by circulating monocytes in patients with systemic or discoid lupus erythematosus.(ABSTRACT TRUNCATED AT 250 WORDS)

Three heroin addicts had Candida folliculitis of the scalp, beard, and pubis associated with fever, chills, headache, and fatigue. In each case, pseudohyphae were found within a hair and yeasts around it and Candida was recovered from urine. These facts support a systemic dissemination. Since serious ocular and osteoarticular lesions have been described with this type of skin lesion, prompt diagnosis may be important to initiate treatment and prevent sequelae.

The authors report one case of Pyoderma gangrenosum whose unusual cervical localization was particularly bothering. Besides, the patient, an 18-year-old girl, had clinical symptoms of Crohn's disease confirmed by radiological investigations and bowel pathology. A benign monoclonal gammapathy and an auto-immune haemolytic anaemia were also found. After four plasma exchanges Pyoderma healed rapidly, Crohn's disease stabilized and biological abnormalities disappeared. Comments and review of the literature are reported. It appears that such a case is quite exceptional, as far as clinical, biological data and therapy (plasma exchange) are concerned.

The authors report a new case of the association Hodgkin's disease and Kaposi's sarcoma. A woman of North African origin and suffering from mediastinal Hodgkin's disease developed, after the commencement of polychemotherapy with radiotherapy, Kaposi's cutaneous sarcoma, typical, clinically, histologically and by electron microscopy. In the light of this new case, the authors review 41 cases of the Hodgkin-Kaposi association found in the literature and discuss the inter-relations between Kaposi's sarcoma and diseases of the haemoreticular system, in particular carcinomas, immuno-depressive states (notably in association with renal transplants) and virus disorders (essentially herpes). It might be possible to incriminate in the aetiology of Kaposi's disease a carcinogenetic process of viral origin during a state of immuno-depression.

Montgomery's syndrome is distinguished by skin and mucous membrane xanthomatosis, predominant on folds, associated with diabetes insipidus and normal lipidic balance. From one typical case history occurring in a young gravida, the authors stresse upon: --Review of literature's cases. --Peculiar therapeutic problems during pregnancy. --Nosological discussion: the position of Montgomery's syndrome compared with histocytosis X and other nonnormolipidic xanthomatosis. This problem is all the more important as in this observation xanthomatosis was first with a normal lipidic measurement, lipidic disorders appearing only secondarily.

The effects of chronic hypervitaminosis A and long-term isotretinoin treatment on bone include cortical hyperostosis, ligament calcification and premature epiphyseal closure. Similar effects have now been reported in patients under maintenance treatment with etretinate in high doses. Etretinate, an oral, aromatic, synthetic vitamin A derivative, is widely used in Europe for disorders of keratinization. We report the cases of two patients--one with lamellar ichthyosis, the other with pachyonychia congenita--who developed such bone diseases during treatment with etretinate over 2 and 6 years respectively. The doses ranged from 0.5 to 1 mg/kg/day. Two years after starting treatment (total dose 25 g), the patient with lamellar ichthyosis complained of mechanical pain in the lumbar region and hips. Radiography showed calcification of the extraspinal tendons and ligaments and hyperostosis of the calcaneus bone at the insertion of the plantar ligament. After six years of etretinate treatment (total dose 50 g), the patient with pachyonychia congenita presented with scoliosis and limb length discrepancy. The musculoskeletal abnormalities resembled chronic hypervitaminosis A, with such osseous changes as demineralization, thinning and increased curvature of long bones with osteopenia, and premature closure of the epiphyses. Acroosteolysis was also present. Etretinate has been implicated in the formation of spinal hyperostoses and calcification of extraspinal ligaments in patients who had taken the drug for many years. The occurrence of premature epiphyseal closure in children certainly is a consequence of therapy with relatively high doses of etretinate for six years. But premature epiphyseal closure may also result from trauma to a fragile bone.(ABSTRACT TRUNCATED AT 250 WORDS)

The authors report the case of three middle-aged women admitted to the hospital because of cerebro-vascular accidents, in whom examination disclosed idiopathic livedo reticularis. This association has only been described recently, but does not seem fortuitous; some of the clinical manifestations are characteristic, but the histology is usually nonspecific and the basic abnormality remains unknown. The main problem is prognostic and therapeutic. In an attempt to prevent the neurological defects, tobacco and contraception should not be permitted to female patients showing widespread acquired livedo reticularis; high blood-pressure should be treated systematically.

40 unrelated patients with Psoriasis vulgaris were studied for their HLA-A, B and DRw antigen phenotypes. All underwent a skin biopsy to confirm diagnosis. Like most of the authors, we observed an increase in B13 and B17 antigens (17,50 and 25%) whereas in the healthy population the percentage was (5 and 6,02%) respectively. The strong increase in DRw7 suggests that psoriasis vulgaris was associated with DRw7 antigens, with an unbalanced linkage between the B13 and DRw7 antigens and between the B17 and DRw7 antigens.

Observation of skin lesions in patients who frequent swimming pools in the dermatology section of the Nancy Hospital, coupled with isolation of Mycobacteria marinum led to a preliminary survey of 20 swimming pools in the Lorraine region for Mycobacteria. Six representative pools were then chosen for more in-depth research during 1977 and 1978: 3 chlorinated, 2 brominated and 1 ozonated pool. The following parameters were analyzed: chemical (pH, conductivity, residual disinfectant, and oxidizable matter by potassium permanganate); microbiological (standard plate counts, total and faecal coliforms, Streptococci, Clostridia, Mycobacteria, and pathogenic Staphylococci). During the survey, different species were isolated: saprophytic Mycobacteria such as M. gordonae, M. flavescens, M. terrae, M. aurum and M. parafortuitum; and opportunistic pathogens such as M. marinum, M. kansasii, and M. fortuitum. There was no correlation between the presence of Mycobacteria and classical microbiological indicators. In one pool, M. marinum was repeatedly isolated, this same pool supplied by thermal water was being used by some patients with skin granuloma. Increased chlorination eliminated this pathogenic species. The level of saprophytic Mycobacteria was very low while opportunistic Mycobacteria were absent in pools with a residual chlorine concentration of only 0.5--0.6 mg/l. The same results were found for brominated pools with a residual of 2 mg/l, and for the ozonated pool without a residual. The Mycobacteria contamination was due partly to the water which replaced eliminated water and partly to swimmers and pool surfaces (walls, floor).

Two patients with pyoderma gangrenosum have responded remarkably well to treatment with Clofazimine (Lamprène). The first patient, a 68-year old women suffered from pyoderma gangrenosum of the buttock and left leg and on the incision scar for cancer of the breast. Laboratory findings showed monoclonal dysglobulinemia (alpha 2-kappa 2). A daily dose of 300 mg of Clofazimine resulted in complete healing with ten days. The second patient was a 24-year old women suffering from ulcerative colitis and a rapidly progressing pyoderma gangrenosum of the left leg. The lesions was completely healed after two weeks of Clofazimine therapy. The dosage was 200 mg daily and was increased to 400 mg daily. Our cases showed decreased cellular immunity and their phagocytic activity was variable.

Hyperpigmentation of the lower limbs does not always reflect a phlebological abnormality. The authors briefly recall other pathological circumstances which result in cutaneous hyperpigmentation : cutaneous porphyria (hyperpigmentation of the exposed parts), malignant melanoma, Recklinghausen's neurofibromatosis (café-au-lait spots and naevo-cellular naevi), pigmented dermatoses (lichen planus), fixed pigmented toxidermias, drug induced hyperpigmentations (minocyne, synthetic anti-malarials which can provoke the development of large areas of greyish coloured s in), reticular "hot water bottle" dermatitis and malingering syndromes.

40 unrelated patients with psoriasis vulgaris were studied for their HLA-A, B and DRw antigens phenotypes. All underwent a skin biopsy to confirm diagnosis. Like most of the authors, we observed an increase in B 13 and B 17 antigens (17,50 % and 25 %) whereas in the healthy populations the percentage was (5 % and 6,02 %) respectively. The strongest increase in DRw7 suggest that the psoriasis vulgaris was associated with DRw7 antigens, with a linkage desequilibrium between the B 13 and DRw7 antigens and between the B 17 and DRw7 antigens.

The physiological colour of the skin is due, above all, to the presence of melanin in the dermis and the epidermis, but also to the presence of other pigments of endogenous or exogenous origin, such as iron and carotene. The authors present a study of melanogenesis. They review the structure of the eumelanins and the phaeomelanins and recall the steps in their biosynthesis. They examine the functioning of the epidermal unit of melanization, which is a functional unit consisting of a melanocyte, derived from the neural crest, and the keratinocytes which depend on it. The melanocyte synthetizes pigment granules, the melanosomes, which undergo maturation before being transferred and then broken down by the keratinocytes. Finally, the authors study the principal factors which influence the functioning of the epidermal unit of melanization : genetic control, ultra-violet radiation and endocrine factors.

The free sale of "self-defence sprays" and the use of such weapons by the police and riot squads account for the increase observed in the frequency of cutaneous accidents. Tear gases. 1. CN, or chloracetophenone, is a compound insoluble in water and soluble in alcohol, ether and carbon sulphide. MACE is CN dissolved in methylchloroform. 2. CS, or orthochlorobenzylidene malononitrile is also insoluble in water and can be used in sprays in concentrations of 2 to 8 p. 100, propelled by such gases as freons. Clinical effects. 1. Experimental. When these gases are suspended in air they mostly act on the eyes, producing blepharospasm, conjunctivitis and photophobia; they have only minor effects on the skin. When applied directly onto the skin they produce extreme irritation with erythema and vesicles. The higher the degree of humidity, the more severe the lesions. In animals and in man CN and CS behave as potential allergens on repeated exposures. 2. Accidental. Tear gases may have two effects: they usually produce irritant dermatitis, but also sometimes a genuine eczema. In aggressors exposed to these sprays the lesions develop in two stages: first, redness and burning sensation on the face--which characteristically is only affected on one side owing to the lateral projection of the tear gas--then, on the following day there appears an oedema similar to Quincke's oedema, with swelling of the eyelids. Oozing rapidly turns to crusts, and in the absence of treatment infection is the rule. Cure requires as many as 10 to 15 days of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

The authors describe a child who presented multiple congenital malformations affecting the cranio-facial region and the extremities associated with a rare skin lesion (incontinentia pigmenti achromians).

Two epileptic children treated with phenobarbital and suffering from tinea capitis, not responding to a long lasting treatment with Griseofulvin, healed only after phenobarbital has been discontinued. We ascribe our therapeutic problems to the metabolic interference of Griseofulvin with phenobarbital. We have measured the blood concentration of Griseofulvin several times in one of our 2 children and in a control of the same age living in the same dietary conditions and in the same conditions of Griseofulvin intake; these measurements confirm these data. The point of interest of this report is to draw attention on: -the pharmacologic hypotheses put forward to explain this drug interference, -the other factors of a bad therapeutic response to a treatment with Griseofulvin.

The variations in electrophoretic mobilities found in different lymphocyte populations present in circulating blood of three patients with Sézary syndrome have been studied. Evidence is given for variations in electrophoretic mobilities being a function of the cytological type of the pathological cells found. The existence of a significant increase of "null" cells is shown, which seem to correspond to Sézary cells.

We report one case of amicrobic pustulosis cured with Dedrogyl (25-hydroxycholecalciferol), active metabolite of the vitamin D. The importance of our case stems from the physiopathologic study of this cure. The patient is a 16-year-old boy suffering from a hypoparathyroidism since his younger days. Consequently, he shows a hypocalcemia and convulsive seizures due to the hypoparathyroidism. Those seizures had been wrongly attributed to a primary epilepsy of a neurologic origin and a treatment with Phenobarbital had been instituted. In fact, they were side-effects of the hypoparathyroidism and the Phenobarbital had only aggravated the hypoparathyroidal hypocalcemia as a result of its effect on the metabolism of the vitamin D (deviation of this metabolism by enzyme induction at the level of the liver). The Dedrogyl (25-hydroxycholecalciferol) has restored a normal phospho-calcium balance and the hypocalcemia has disappeared as well as the convulsive seizures which, one year later, had not reappeared while they had previously been continual. And above all, standing back one year, we have recorded a complete cure of the amicrobic pustulosis on account of the Dedrogyl.

There is an increased risk of cancer (mainly breast cancer) in patients with Cowden disease. Little is known of the etiopathogenic mechanisms of this condition, but immunologic abnormalities may be evoked. A young patient with Cowden disease was submitted for gynecologic examination and immunologic investigations. Although no neoplastic disorder had appeared, laboratory investigations disclosed abnormalities of the humoral and cellular immune compartments in the peripheral blood. This rare observation of gynecologic involvement in a very young girl with family antecedents of Cowden disease prompted a review of the gynecologic features and etiopathogenic bases of Cowden disease. The possible role of neoplasia such as breast cancer in this syndrome makes early diagnosis a criterion for good prognosis.

After having reviewed the conditions of use of an antimycotic-corticoid combination, the authors are reporting their clinical results on 183 patients of a new drug combining econazole and triamcinolon. This clinical trial demonstrates that the own activity of each component of the combination is entirely maintained, and that the new combination shows an original interest in the treatment of all inflammatory dermatomycoses, as well as inflammatory supra-infected dermatoses.

In the plant of a large newspaper, the manufacture of printing plates using photopolymer acrylic resins, in the place of lead, resulted in a series of orthoergic and allergic manifestations in the workers. Chemical analysis resulted in the isolation of a tetraethylene glycol diacrylate, the harmful nature of which was proven by allergological studies. Its replacement by tetrathylene glycol dimethacrylate eliminated the problems seen (study based upon 300 allergological tests).

A t(X;9)(p11;q34) is reported in a girl with incontinentia pigmenti (IP). The X breakpoint is at p11.21. Although no similar case has been reported, this breakpoint may be significant insofar IP is considered an X-linked dominant mutation and could be of help in a specific X DNA probes study.

Although clinical studies have been supplying strong arguments in favour of a hereditary transmission of atopy for a long time, the latest works have not made it possible yet to specify the biologic support of this heredity. An obvious correlation, especially, has not been demonstrated between, on the one hand, atopic ground and HLA group and, on the other hand, between a high production of total IgE and HLA group. At present, the production of specific IgE only seems to be really subordinated to the HLA system; it is likely to be the expression of complex immunological disorders of a genetic determinism. Various environmental factors will contribute, on this predisposed ground, to the disclosure or to the worsening of the atopy. Some prevention of atopy may be undertaken. Extended breast feeding, eviction of some environmental nuisance and family affective balance thus represent useful preventive measures. The influence of the date of conception and of the pregnancy and delivery conditions does not appear to be insignificant; but, there, the prevention is more difficult to set up. Finally, the counting of the IgE in the cord blood could lead to the detection of high-risk newborns.

The patient, a 75-year old man, was admitted in May, 1986 for separation of the epidermis and extensive ecchymotic patches. Physical examination showed numerous haemorrhagic erosions on the extensor aspect of the limbs, feet and hands, and wide patches of epidermal separation in the axillary and dorsal regions. Ecchymotic purpura was present on the limbs, abdominal wall, neck and right orbital region. Nikolsky's sign was positive at the periphery of the lesions. Epidermal cysts, 1 to 5 mm in diameter, were visible on the back of the hands and on the upper part of the neck. There was no macroglossia. Several biopsies were performed in both diseased and healthy skin. Light microscopy of the diseased skin showed, at the junction of the papillary and middle dermis, a band of eosinophilic deposit in which were true intradermal bullae containing red cells. Congo red and thioflavine T stainings were positive, forming a dermal band. At direct immunofluorescence IgG, IgA, IgM as well as the C3 and C9 components of complement were absent. At electron microscopy there was no bullous separation at the dermoepidermal junction; the dermal deposits had a dense amyloid-like fibrillar structure without ramifications. Laboratory examinations showed lambda-2 monoclonal gammopathy with normal levels of IgG and IgA and slightly decreased IgM. Bence-Jones protein was found in the ruin. Bone marrow examination showed 8 p. 100 plasmocytes. The diagnosis was: non myelomatous lambda-2 monoclonal dysglobulinaemia. Amyloid deposits were found in biopsies of the gums and rectum. Other investigations gave negative results. Bullous lesions have been reported in about 20 cases of primary amyloidosis.(ABSTRACT TRUNCATED AT 250 WORDS)