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R Kuffer

Publications and source records attributed to R Kuffer.

88 records · Page 5Linked to original sources
PubMedDate not supplied

[Corticotherapy and mucocutaneous pathology].

The tremendous advances in treatment brought about by corticotherapy applied to cutaneo-mucosal pathology should not be allowed to obscure the fact that its action is merely palliative, that it should only be proceeded with after careful diagnosis and that it may trigger undesirable side-effects. General corticotherapy is definitely indicated in certain serious dermatoses (e.g. pemphigus vulgaris) in large doses at the beginning of the course of treatment which often has to be kept up indefinitely; it is in these patients that the most serious accidents occur. It is also indicated in other dermatoses (e.g. lichen planus) in smaller doses and in separate courses, generally triggering incidents and accidents of a less serious nature which to a certain extent seem to be attenuated by taking the drug on alternate days. It is counter-indicated in one particular condition: psoriasis. Corticotherapy by intra- and sub-lesional local injection is most useful in the treatment of certain localised skin lesions (e.g. cheloids) and of the oral mucosa (e.g. erosive lichen planus). Either a few drops are injected or a larger quantity in a suspension of microcrystals. Complications have sometimes been observed in the skin (leukoderma, dermoepidermatrophia and, particularly, amaurosis), but never so far after sub-mucosal injections. Local corticotherapy by external application, very widely used in the form of ointments, creams and lotions for numerous cutaneous conditions may cause various more or less serious local side-effects, the systemic effects with depression of the hypophyso-adrenal axis, only seem to occur to any extent with occlusive dressings. It can also be used in the treatment of some conditions of the oral mucosa (e.g. some forms of lichen planus, benign mucous membrane pemphigoid) by means of either a corticosteroid incorporated into a special excipient which adheres to the mucous membrane or in tablets of 17-betamethasone valerate which gradually break up in the saliva. With the usual posology of 10 tablets of 0.1 mg per day, even over several months, there are no systemic effects, 17-betamethasone valerate (unlike phosphate) having an action which is primarily topic and being practically unabsorbed as has been shown by assessment of plasmatic cortisol.

Administration, Topical↗
PubMedDate not supplied

[Oral aphthoid toxic dermatoses].

In a description of 4 cases (phenindione, 1 case; niflumic acid, 1 case; gold salts, 2 cases), the authors describe a new variety of oral toxicodermatitis characterized by an eruption of painful, infiltrated aphthoid ulcerations, not precededbybull ae, small (less than 1 cm), roundish, with a greyish yellow necrotic centre surrounded by an erythematous halo. Histological investigation reveals a compact, polymorphous inflammatory infiltrate with numerous polynuclear neutrophiles or, less commonly, eosinophiles some of which are in a state of pyknosis of leukocytoclasia, associated with phenomena of spongiosis or necrosis of the epithelium.

Adult↗
PubMedDate not supplied

[Digestive symptoms of scleroderma (author's transl)].

The authors successively study the oral, peri-oral, esophageal, intestinal and anal manifestations of systemic scleroderma. They point out that dental, salivary, and esophageal involvement is of great interest for diagnosis and prognosis. They particularly describe the malabsorption syndrome including anatomical and ultrastructural lesions of the smooth muscle fibers. They present an original study of arrector pili in clinically and histologically non-involved skin of patients undergoing scleroderma. Finally they state that any suspected or diagnosed scleroderma needs a digestive check-up as follows: radiologic examination of the teeth, biopsy of the minor salivary glands of the lower lip, radiologic study of esophageal motility and radioactive C 14 glycocholate test.

Esophageal Diseases↗
PubMedDate not supplied

[Cutaneous-mucous hyalinosis (Urbach-Wiethe disease). Histologic and ultrastructural study of a case].

Hyalinosis cutis et mucosae is a rare genetic disorder, primarily involving skin and mucous membranes, that has been described under various titles as lipoidosis lipoproteinosis and Urbach-Wiethe disease. A 17-year-old girl presented cutaneous macular lesions, skin scars, thickened lips and enlarged firm tongue; hoarseness was known since birth. The labial biopsy specimen was investigated by light and electron microscopy. Histologically there are deposits of amorphous, extra-cellular eosinophilic hyaline material in the upper thickened connective tissue. In the lower part changes are focal with hyaline mantle around the vessels. Histochemical studies have revealed that the deposits were PAS-positive and amylase-resistant, indicating the presence of neutral mucopolysaccharides. Lipid stains were positive, especially in vessel walls. Ultrastructurally hyaline substance is composed of 1 to 2 nm protofilaments, larger ones (5 to 10 nm wide) and amorphous material. Fibroblasts contain in their dilated cisternae amorphous material. A frequent feature is marked multilamination of the capillary basal lamina which is interpenetrated by hyaline substance. Pathogenesis is still unknown but recently it is suggested hyalinosis cutis et mucosae could be a systemic disease of basal lamina.

Adolescent↗