[Chronic perineo-buttock ulcerations and suppurations preceding Crohn's disease by 30 years].
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Biomedical subjects
Publications and source records attributed to M P Franc.
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Degos' disease (malignant atrophic papulosis) occurring in mother and daughter is described. Mother's eruption is composed of multiple characteristic lesions (up to 600). She does not have any gastrointestinal, neurological nor other visceral manifestations after a 9-year course. One year after the onset of the disease, she had a daughter. The pregnancy was normal and the newborn did not have any cutaneous manifestation. The daughter's first skin lesion appeared when she was 3-months old. She has now been under observation for nearly nine years and developed only four skin lesions without any visceral manifestation. Laboratory investigations show an increase in fibrinogen in both mother and child, without any sign of disseminated intravascular coagulation. The research for "slow virus" by a primate's intravascular inoculation of mother's skin lesion in negative.
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Five patients present a Jessner-Kanof's lymphocytic infiltration of the skin with 6-year duration for 4 of them. The skin lesions are numerous on the face and the back and characteristic of this disease. Histologic examination shows a lymphocytic infiltrate in the dermis without any modification of epidermis. Direct immunofluorescent study is negative in all cases. In 3 cases, response to chloroquine had been inconsistent or negative. The 5 cases have a excellent result with 100 mg/day of Thalidomide. In 4 cases the skin lesions reappear when treatment is stopped. With a continuous treatment of 25-50 mg/day for more than 2 years, 3 of 5 patients have normal skin. Thalidomide appears to be the best treatment of Jessner-Kanof's disease.
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We report an unusual manifestation of dermatitis herpetiformis in four patients. Three of them fulfill all the criteria for dermatitis herpetiformis: micro-abscesses of polymorphs in the upper papillary dermis, granular IgA deposit on IF examination, gluten-sensitive enteropathy, HLA B8. These peculiar lesions occur on palms and fingers, and appear as a petechial and ecchymotic purpura. A light painful sensation may precede the eruption. In two cases the palmar localisation initiated the course of the disease and remained isolated for more than one year. In only two cases, typical inflammatory lesions were associated on the palms. No lesions occurred on the soles. These peculiar palmar purpura-like lesions cleared with gluten-free diet or Dapsone therapy in the four patients.
We report three cases of 54, 58 and 65-year-old patients presenting annular lesions with centrifugal migratory extension. They occur exclusively on sun-exposed areas. In two cases, the lesions were multiple. In the third case, the lesion was single and mimicked an erythema annulare centrifugum. The past history ranged from 18 months to 8 years. Microscopic examination of the central part showed a disappearance of elastic fibers in upper reticular dermis. Examination of the ring showed in upper reticular dermis an histiocytic granulomatous infiltration with many giant cells, lymphocytes and patterns of elastic fibers phagocytosis. Similar features were found by electronmicroscopy. These three cases illustrate the typical features of O'Brien's actinic granuloma. Relationship between this actinic granuloma and granuloma annulare occurring on sun-exposed areas on one side, and necrobiosis lipoidica, Miescher's granuloma and granuloma multiforme on the other side, are discussed. On the basis of some clinical and histological patterns, the autonomy of O'Brien's actinic granuloma appears to be established.
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A review of the literature concerning the pityriasis lichenoides and the study of 34 personal cases show that three main clinical patterns are found in pityriasis lichenoides: maculo-papular, leukomelanodermal, necrotic. The course is very variable: rarely seven weeks, more often seven months and sometimes seven years. The disease is issued from an angiitis including a mostly lymphocytic infiltration. The epidermis is secondarily invaded by inflammatory cells and shows focal parakeratosis. There is no specific immunologic disorder: immunohistopathologic study is generally normal (rarely IgM or C3 deposits); no circulating immune complex is found. Some patients improved with dapsone or photochemotherapy.
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