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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)

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.

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.

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.The cases of 3 middle aged women admitted to the hospital because of cerebrovascular accidents in whom examination disclosed idiopathic livedo reticularis are reported. This association has only been described recently, but does not seem fortuitious; some of the clinical manifestations are characteristic but histology is usually nonspecific and the basic abnormality remains unknown. The main problem is prognostic and therapeutic. In an attempt to prevent neurological defects, tobacco and contraception should not be permitted for those women showing widespread livedo acquired reticularis; high blood pressure should be treated systematically. (author's modified)

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.

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.

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.

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.