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Biomedical subjects

A Puissant

Publications and source records attributed to A Puissant.

At least 55 records · Page 3Linked to original sources

[Deep skin lesions of lupus erythematosus].

The authors report 2 cases of lupus erythematosus (LE) with deep cutaneous lesions. In both cases the lupus panniculitis presented as hard, infiltrated subcutaneous plaques situated symmetrically on the external aspect of both arms and on the upper parts of the buttocks. The diagnosis was confirmed on clinical, histological, immunohistological and therapeutic data. These 2 reports and the 43 cases in the literature dating from 1967, illustrate the main features of lupus panniculitis. The clinical appearances and sites are very stereotyped and immediately suggest the diagnosis even in the absence of other clinical and/or biological signs of lupus. The evolution is very slow and is characterised by dramatic regression of the inflammatory signs with synthetic antimalarial drugs. These deep skin lesions are observed in both purely cutaneous chronic lupus and in systemic lupus which is usually relatively inactive. This prevents the identification of a special form of this disease with a specific evolution and prognosis.

Adult↗

[Type IV Ehlers-Danlos syndrome of acrogeria type].

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.

Adult↗

[Enteropathy caused by clofazimine].

A case of Clofazimine enteropathy is reported, diagnosed on the demonstration of red brown birefringent crystals in the small bowel mucosa and submucosa as well as in the mesenteric lymph nodes whereas the clinical and radiological signs appear to be non specific. After the withdrawal of the drug, a clear improvement of the symptoms was achieved only under a gluten free diet. The possibility of such accidents, sometimes lethal, suggests the restriction of clofazimine to leprosy.

Clofazimine↗

[Hair loss during treatment with oral contraceptives].

Oral contraceptives with a dominant androgen component can cause or worsen androgen-dependent alopecia in women. This diagnosis can only be made if other causes of alopecia (which can occur at the same time as treatment with oral contraceptives) have been excluded. The patient's endocrine profile must be investigated sometimes, this being in order to detect any excess production of androgens. These types of alopecia call for the stopping of the oral contraceptive and sometimes also calls for oral anti-antigen treatment.

Alopecia↗

[Histiocytoid hemangioma of the scalp].

We report a case (the second from France) of histiocytoid haemangioma, an entity created by Rosai et al. (14) in 1979 to accommodate several cutaneous and extra-cutaneous diseases hitherto known under other names and characterized histologically by the proliferation of vessels with peculiar "histiocytoid" endothelial cells. Our patient was a 56-year old woman who developed, within a few weeks, a slightly haemorrhagic nodule of the scalp. The histological examination, performed in a private laboratory, led to a diagnosis of cavernous angioma or angioleiomyoma. Two months after the lesion was removed, the condition recurred in the form of about 10 sessile subcutaneous nodules, 3 to 20 mm in diameter; the nodules were firm, painless, little pruriginous and sometimes ulcerated. An old-standing lymph node enlargement was palpable on the right side of the posterior aspect of the neck. The rest of the scalp was normal. Physical examination of the skin and mucosae revealed no other abnormality. These alarming lesions were suggestive of metastasis or angiosarcoma. X-ray films of the skull were normal, as were the results of blood examination; in particular, there was no eosinophilia. Histology showed a papillomatous epidermis, while the superficial and mid dermis were occupied by a lobulated vascular proliferation. The vessels were of the capillary type; they were lined with two or three layers of endothelial cells remarkable for their large indented nucleus and for their abundant eosinophilic cytoplasm bulging out into the lumen. These vessels were surrounded by fairly dense nodular masses of small lymphocytes with a few histiocytes and some neutrophils. There were no lymphoid follicles.(ABSTRACT TRUNCATED AT 250 WORDS)

Diagnosis, Differential↗

Monoclonal gammopathy and mycosis fungoides. Report of four cases and review of the literature.

Of four patients with mycosis fungoides and monoclonal gammopathy, one died of multiple myeloma that developed 4 years after the mycosis fungoides. The other three had monoclonal gammopathy of undetermined significance. This association does not appear to occur with a high frequency in patients with mycosis fungoides than in the general population. However, it demonstrates that B cell proliferation can occur in patients with primary cutaneous T cell disease.

Aged↗