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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)

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).

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 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 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)

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.

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)

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).