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Biomedical subjects

H Perrot

Publications and source records attributed to H Perrot.

At least 73 records · Page 4Linked to original sources
PubMed1987

[Barraquer and Simons lipodystrophy. Complement anomalies and cutaneous leukocytoclasic vasculitis].

In a 56-year old woman progressive partial lipodystrophy began at the age of 6 years on the face, thereafter extending slowly down to mid-thigh level (fig. 1 and 2), with moderate hypertrophy of the subjacent fatty tissue and a fatty macroglossia (fig. 3). Histological examination of the lipodystrophic skin not only showed an absence of fatty tissue, but also abnormalities at the dermis-epidermis junction with hyaline bodies (fig. 4). At the age of 23 she developed purpura, predominantly on the legs, which rapidly became chronic (fig. 5); histological examination showed leucocytoclasic vasculitis of dermal vessels (fig. 6) with granular deposits of C3 on the vessels and of IgM at the dermis-epidermis junction. Episodes of polyarthralgia and headaches were frequent. Regressive neuritis of the external popliteal nerve occurred when she was 53-year old. Renal function tests proved normal, but renal biopsy was not performed. There was no diabetes mellitus, but an oral glucose tolerance test and a somatostatin insulin glucose test elicited definite resistance to insulin. A search for a serum factor inhibiting insulin receptors was negative. Permanent abnormalities in serum were a very deep fall in C3, a pronounced fall in CH50 and a low C4 level. Besides, a C3 nephritic factor (NeF) at a high level and circulating immune complexes were present (table I); a mixed IgM-IgG cryoglobulin was found intermittently (fig. 7). Clearance of the immune complexes by splenic macrophages was extremely slow. During a series of plasma exchanges, serum C3 increased transiently, whereas serum C4 remained unchanged (fig. 8).(ABSTRACT TRUNCATED AT 250 WORDS)

Antigen-Antibody Complex↗
PubMed1986

[Ito's hypomelanosis. Review of the literature apropos of 3 cases].

Initially described as incontinentia pigmenti achromians, Ito's hypomelanosis is a congenital disease characterized dermatologically by depigmented maculae arranged in a specific pattern. These maculae appear suddenly, unheralded by an inflammatory process, and are arranged on the limbs as lines and on the trunk as whirlwinds or mottled cakes. Other abnormalities, notably neurological, ophthalmic or musculoskeletal may be associated with this spray-like depigmentation of the skin. We report here three cases of this disease, which is probably more common than the scarcity of cases hitherto published would suggest. Case no. 1. A 9-year-old boy presented since the age of 5 with generalized convulsive fits predominant on the right side. Neurological examinations between fits were negative. IQ, FO and CT scans of the brain were normal. During the first months of life, the child had developed a spray-like depigmentation on the right half of his back and on the anterior and posterior aspects of his right arm. Case no. 2. A 2-year-old girl was examined for a cutaneous depigmentation which had developed when she was about 3 months' old and had progressively extended from her left knee to her left hypochondrium. Case no. 3. An 8-year-old girl presented with mental retardation and myopia, but also with a spray-like depigmentation on the left part of her chest. The skin lesion had been noticed by her parents after she had exposed herself to the sun in a tropical country. Histological examination performed in the first two patients showed some degree of hypopigmentation of the epidermis without pigmentary incontinence.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1983

[Clear cell acanthoma. Ultrastructural and immunohistological study].

We report a new case of a clear-cell acanthoma with an unusual location (para-anal), which was studied by electronmicroscopic and immuno-histological methods, using monoclonal antibodies. The results of this study showed that: a) the inflammatory cellular infiltrate comprised a small number of T-suppressor/cytotoxic lymphocytes and a small number of Langerhans cells, b) Langerhans cells were present in the affected epidermis in reduced numbers (2.2 p. 100) when compared to normal epidermis (4-6 p. 100), c) there was a disorder of both keratinization and epidermal differentiation as was shown by the abnormal reactivity of the tumor to monoclonal antibodies KL1 and BL7, d) human papilloma viruses do not seem to be involved in the genesis of the tumour, e) tumoral cells did not show (secretory) eccrine differentiation, f) the pemphigus antigen was present in the intercellular space of the affected epidermis.

Anal Canal↗
PubMed1983

[Buschke's scleredema and monoclonal dysglobulinemia: apropos of 3 cases].

A monoclonal gammopathy was observed in 3 adult patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. The monoclonal immunoglobulin was in every case of the IgG-kappa type. Bence Jones proteinuria was noted in 1 case (kappa). Deposition of monoclonal IgG-kappa in the skin was not detected by immunofluorescence microscopy. These findings and previous reports of 5 cases suggest that diffuse scleredema may be frequently associated with paraproteinemia, but the role of monoclonal immunoglobulins in the pathogenesis of the disease remains to be clarified.

Adolescent↗
PubMed1982

[Skin reactions to piroxicam (three cases) (author's transl)].

Three cases of skin reactions that occurred in patients under oral administration of Piroxicam are reported here: one erythema-multiforme-like reaction and two cases of acute, pruriginous erythemato-papulous dermatitis, one of which with intraepidermal blister formation. No past history of skin disease could be formed; lesions spontaneously improved when the drug was stopped. Only in one patient, receiving Piroxicam and Fenoprofene, an acute nephritis was observed. The role of Piroxicam, a new non-steroid antiinflammatory agent in the pathogenesis of the skin reactions observed is discussed. However, the renal involvement formed in one case is likely due to Fenoprofene, as in previously reported cases.

Aged↗
PubMed1982

[Acquired epidermolysis bullosa with Crohn's disease. Report of two cases and review of literature (author's transl)].

Two cases of acquired epidermolysis bullosa (A. E. B.) are reported in patients suffering from Crohn's disease. Microscopic examination showed in both cases a subepidermal blister with pieces of basal membrane among the roof of the blister. In the second case, linear deposits of IgG and C3 were observed by direct immunofluorescence at the dermoepidermal junction in the juxtalesional skin. No circulating antiepidermis antibody were found by indirect immunofluorescence in any of the two cases. Presence of immune complexes was evidenced. A review is presented of similar cases in the literature and of the frequence of the pathological associations. No peculiar feature of A. E. B. when associated to Crohn's disease was found: the course of the two diseases is independent; however, we did observe an improvement of skin lesions by the time of the intestinal remission in the first case. The pathogenesis of this association is still unclear. Autoimmune mechanism might possible play a role, but is still unknown.

Adult↗
PubMed1982

[Acquired leukomelanoderma caused by topical depigmenting agents].

Three patients applied a cream containing monobenzyl ether (MBEH) (2 cases) or monomethyl ether of hydroquinone (1 case) on acquired hyperpigmentary disorders. We observed depigmentation locally and at a distance from the treated areas. The depigmenting ability of MBEH was founding in 1939 by Oliver, at the time of an occupational accident. This happended among black workers wearing rubber gloves containing MBEH as an anti-oxydant. The depigmenting ability has been used as a treatment for acquired hyperpigmentary disorders, but adverse effects were observed--irritation and local sensitization--depigmentation locally and at a distance from the treated areas--melanoleukodermia among asiatics--no repigmentation: therefore treatment with MBEH has been used by Mosher in extensive vitiligo on the unaffected areas. As regards to the mechanism of these pigmentary disorders, knowledge is incomplete, particulary concerning depigmentation at a distance: hypothesis was: destruction by degrees of the melanocytes, as in Lerner's theory concerning vitiligo, autoimmune mechanism? Genetic predisposition? Electron microscopy shows cellular disturbances in melanosomes and degeneration of intracytoplasmic membrane thus killing cell. The physiopathology was the work of Riley: he has shown that MBEH is selectively incorporated into cultured melanocytes. Evidence of free radical formation has been obtained in guinea-pig skin treated with this agent. Cultured melanocytes are irreversibly damaged by exposure to low concentrations. Riley postulates that these compounds diffuse into melanosomes of the pigment cell where they are oxidized by tyrosinase to produce free radicals; the latter bring about lipid peroxidation of cell membrane lipids, thus killing the cell.

Adult↗
PubMed1981

The histopathology and ultrastructure of liver disease in erythropoietic protoporphyria.

The light, polarization and electron microscopic changes in the liver of four cases of erythropoietic protoporphyria (EPP) are described. In one of these cases liver involvement resulted in death, whereas in the other three there were no symptoms of referable to hepatic disease. In two cases hepatic pigment was readily visible on light and polarization microscopy and corresponded to characteristic crystalline aggregates seen ultrastructurally. Pronounced micronodular cirrhosis and cholestasis were present in the fatal case, while in the other only mild periportal fibrosis and mononuclear inflammatory cell infiltrate occurred. The third and fourth cases showed no evidence of hepatic involvement on light microscopy. Electron microscopy, however, revealed characteristic crystal-containing vacuoles indicative of protoporphyrin deposition. The need for early detection of the hepatic lesion is emphasized.

Adolescent↗
PubMed1981

[Bilateral parotid enlargement from malignant lymphoma (author's transl)].

A rare case is reported of a 32-year-old man presenting with bilateral enlargement of the parotids only, later found to be due to a malignant lymphoma of the non-Hodgkin's type. Diagnosis was impossible at this stage because of the absence of other clinical signs, except for asthenia, and the negative results of complete investigation. Diagnosis became possible after the appearance of cutaneous lesions followed by a parotid nodule, which were shown by biopsy, over a period of two years, to be extensions of a non-Hodgkin's type 2 malignant lymphoma, according to the classification of Bryon et al. from Lyon. The initially prescribed corticoid treatment was replaced by multiple chemotherapy, and parotid enlargement, asthenia, and skin lesions were no longer present eight months later. The authors emphasize that no lesions occurred in the hematopoietic organs during the course of the disease, which clearly differentiates this case from the parotid lesions seen fairly frequently in blood diseases and Gougerot-Sjögren syndromes complicated by lymphoid leukemia.

Adult↗