Biomedical subjects
Publications and source records attributed to M C Boullie.
The authors report the case of a 30-year old man who suffered spontaneous rupture of the right subclavian artery. Treatment consisted of carotid-axillary graft since the fragility of the vessel precluded direct suture. The clinical symptoms, together with histological and ultrastructural examinations led to a diagnosis of Ehlers-Danlos syndrome with purely arterial manifestations.
A 68-year old man presented with erythemato-telangiectatic nodules on his right lumbar fossa, associated with right-sciatica. A skin biopsy was performed. Microscopic examination showed tumour embolism in the lumen of the dermal vessels. Immunohistochemical staining and electron microscopy confirmed the diagnosis of angiosarcoma metastasis. An arteriography showed the primary neoplasm as a polypoid tumour with anterior pedicle, narrowing the aortic lumen below the renal arteries. The patient died a few months later, with multiple metastasis in the lower half on his body. To our knowledge, this is the twelfth case of arterial primary angiosarcoma reported in the literature and the fifth one with skin metastasis. The previously published cases are reviewed.
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A case of argyria in a 14 year-old child caused by abuse of silver containing pulveriser for sore throat is described. The distinctive skin blue gray discoloration that occurs in argyria is due to deposition of silver. Pigmentation is permanent. Self treatment is stigmatized.
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A young woman in a good health noticed the occurrence of inelegant wrinkled plaques on her trunk and limbs. The histological aspect was particular in so far as there were no layers of elastic fibres left in the middermis. None of the classical diagnoses could be accepted. Five similar cases have been reported in the literature and make it possible to discuss a new entity among cutaneous elastolysis.
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In a family displaying the familial atypical multiple-mole melanoma syndrome, linkage analyses were performed between HLA and an assumed dominant gene respectively determining each of the following affected phenotypes: precursor lesions; cutaneous malignant melanoma (CMM); and precursor lesions or CMM or both. The results suggest that there is a complex mechanism involving several factors, genetic and environmental interacting with the gene determining precursor lesions to cause the neoplastic transformation.
A familial case of acrogeria, occurring in a mother and her daughter, is reported. A 12-year old mentally retarded girl was seen with a history of repeated ecchymoses despite normal hemostasis. On clinical examination, the skin was diffusely atrophic with atrophy and wrinkling most pronounced on the extremities. The venous pattern was noticeable on the upper aspect of the anterior trunk and the extremities. The feet were small and the nose was pinched. "Cigarette paper" scars and molluscoid pseudotumors were seen over the elbows and the knees. Some skin hyperextensibility was noted and loose-jointedness of the fingers was demonstrated. The fingernails, hair, dentition, and bone roentgenograms were normal. The mother, a 34-year old woman, presented with the same abnormalities and reported the same condition in her mother. A younger brother was normal and there was no history of consanguinity. In both cases, the skin biopsy specimens showed dermal atrophy with irregular increase of the elastic staining material in the mid dermis. Ultrastructural studies showed a widened endoplasmic reticulum within the fibroblasts. Biochemical studies, performed in the daughter, showed complete lack of collagen III in the dermis. This, together with the visceral complications occurring in the mother and in previously reported patients, suggests that acrogeria represents a clinical variant of Ehlers-Danlos type IV. Its association with phenylketonuria in the daughter is considered to be coincidental.
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The localization of hyaluronectin has been studied in normal skin and in basal cell carcinoma. In fetal skin it is abundant in the dermis but absent from the epidermis, and in adult skin it is totally absent except in the hair sheaths and bulbs. In basal cell carcinoma it is abundant only in the stroma reaction. The presence of this protein in mesenchymatous tissues seems to be linked to zones of physiological or neoplastic proliferation.
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The use of strontium 90 has proved to be efficient and practical, because handy, and permitting short treatment, not only, to cure benign superficial tumors and, as reported in this study, of pre-cancerous lesions such as actinic keratosis, Bowen's disease of the skin but also some carefully chosen cases of superficial carcinomas. Hundred lesions have been so treated and followed for 3 years; two only have relapsed. The cosmetic result has been excellent in 80 p. 100.
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