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Biomedical subjects

L Schnitzler

Publications and source records attributed to L Schnitzler.

At least 55 records · Page 3Linked to original sources
PubMed1978

[Rudiger (E. E. C.) syndrome: report of a case associated with atopic dermatitis (author's transl)].

The syndrome of ectrodactyly, ectodermal dysplasia and cleft lip-palate is described in a boy aged 3; although inherited in most cases as an autosomal dominant trait, it seems here to be sporadic. An atopic dermatitis clinically evident and particularly resistant to treatment is present, but IgE level is not raised. Ophthalmologic changes are prominent. Systematic intravenous pyelogram showed urinary tract strictures.

Child, Preschool↗
PubMed1978

[Treatment of mycosis fungoides by PUVAtherapy. Report on 18 cases (author's transl)].

A group of 18 patients with mycosis fungoides (M.F.) was treated by PUVAtherapy. According to VanScott classification, they have been set in:--5 parapsoriasis in large plaques = 5 stages I;--6 stages II;--3 stages III;--2 stages IV;--1Sézary syndrom.--1 erythrodermia. There was complete clearing of 12 patients (66 p. 100); 2 patients (11 p. 100) improved cutaneous lesions without a complete clearing, and there was no response to treatment for 3 patients (22 p. 100) (1 with erythrodermia, 1 with Sézary syndrom and 1 stage IV, and 1 forsaking). The method applied here was different in several points: orally administered methoxalen were given according to the body area (mg/m2) and some of the patients had twice a day puvatherapy. Puvatherapy seems to be the least dangerous and most effective treatment for the patients in the early stages of mycosis fongoides (I, II) and nitrogen mustard, electron therapy, corticoids and even mono- or polychimiotherapy could be associated to puvatherapy, if necessary, for the stages III and IV.

Aged↗
PubMed1978

[Multifocal fibrosclerosis (retroperitoneal, mediastinal, mesenteric and pelvic involvement) associated with vasculitis and revealed by a livedo: clinical, histopathological and ultrastructural study in a case (author's transl)].

A 22-year-old male patient was seen with vasculitis and subcutaneous nodules on arms and legs. Discret thoracic venous dilations were noticed and venography demonstrated obstruction of the superior vena cava. Clinical and biological examination, roentgenograms revealed no sign of immunological disease but led to the diagnosis of mediastinal and retroperitoneal idiopathic fibrosis. No drug could be incriminated. Right hydronephrosis obliged to a surgical bilateral ureterolysis combined with systemic steroid therapy. Histological and ultrastructural examinations on retroperitoneal biopsies showed dense collagenous fibrosis, with vasculitis. Perivascular infiltrate was mainly composed of extravased erythrocytes and lymphocytes. Fibrin deposits were seen around implicated vessels. Direct immunofluorescence investigations were negative. These morphological features seem to be in agreement with the physiopathological hypothesis of sclerosis. No retroperitoneal or mediastinal fibrosis extension occurred three years after surgical procedure, and under corticosteroid therapy. Similar vasculitis with mediastinal and retroperitoneal idiopathic fibrosis have previously been reported in only one case by R. W. Carton and R. Wrong. Multifocal fibrosclerosis is the generic term currently used, since Comings et al. (1967), to describe a group of fibrosing conditions which affect separate organ systems. Findings suggest that retroperitoneal fibrois, mediastinal fibrosis, sclerosing cholangitis, Riedel's thyroiditis and pseudotumors of the orbit may be different manifestations of a single disease whose pathogenesis remains obscure.

Adult↗
PubMed1977

Granuloma faciale. Ultrastructural study of three cases.

Light and electron microscopic observations were carried out in three cases of granuloma faciale. The dense granulomatous infiltrate of the corium showed: 1) more eosinophils than suspected by light microscopy, most of them with degenerative changes; 2) Charcot-Leyden chrystals; 3) numerous large histiocytes with their cytoplasms filled with lysosomal vesicles containing various inclusions (lamellar figures, myelin bodies, circular concentric organelles, digested membrane structures). The lack of Langerhans granules in dermal cells seems a valid reason to differentiate granuloma faciale from eosinophilic granuloma of the skin, usually considered a variety of histiocytosis X. No evidence of bacterial or viral infections was noticed. Granuloma faciale, despite its uncertain etiology, seems to represent a true entity.

Adult↗
PubMed1977

[Onychopathy of mushroom-growers].

Particular nail damage appeared in patients working on mushroom beds. In five cases, all similar, the recent use of plastic bags containing the growing medium, seems to be the origin of this onychopathy. Onycholysis with latero-distal "usure des ongles", koilonychia, longitudinal splitting with sometimes splinter haemorrhages are the most specific changes. Chemical damage by phytosanitary products or fungi infection have no significant part in these abnormalities; their cause is traumatic, representing occupational stigmata mark, and results from repeated rubbing of the nails in workers lifting up heavy plastic bags.

Adult↗
PubMed1977

[Lichen aureus or lichen purpuricus; about 5 cases; ultrastructural study].

Clinical, histological and ultrastructural features in five cases of lichen aureus are descirbed. This rare condition with ages ranging here from 6 to 31 consists of a pigmented papular and purpuric eruption. Unilaterally, trunk or limb may be involved, in a possible systematized distribution. The microscopic picture is characterized by a dense histiocytic and lymphocytic infiltration in the upper part of the dermis; extravasation of erythrocytes and iron pigment in the histiocytes are often noticed. In fine structure most cells of the dermal infiltrate are histiocytes (or macrophages) with numerous worm-like structures and even typical Langerhans granules in three cases; histiocytic cells; histiocytes are frequently seen in apposition to lymphocytes. In all these cases, no sign of histiocytosis X is found. Classification of lichen aureus in the group of idiopathic pigmented purpuric eruption is proposed.

Adolescent↗
PubMed1977

[Plasmocytic proliferative lesions of the foreskin. A variety of Zoon's benign circumscribed balanitis (author's transl)].

A peculiar anatomoclinic form is described about the Balanoposthite chronique circonscrite bénigne à plasmocytes (Zoon): the pimpled, erosive, nodular and pseudoangiomatous form. Clinically this balantis realizes a pseudo-oedematous infiltration (which belongs to the usual form) and especially botriomycome-like nodules (obs. 2) or pseudo-neoplastic tumors (obs. 1). Histologically, the lesions consist of a pure plasmocytar infiltration, with total disappearance of the epithelium. In spite of its pseudo-tumoral aspect and of the abundance of plasmocytes, this kind of balanitis is quite benign and chronic, may persist many years without transformation, and is never a sign of dysglobulinemia.

Adult↗
PubMed1977

[Familial epithelioma of Ferguson-Smith about two familial cases].

Two cases of multiple self-healing epithelioma (Ferguson-Smith) are reported in a French family. This dermatosis has been present since the age of 30 in both father and son. The father aged 66 years, farmer, had suffered since 1939 from recurrent spontaneously healing tumors of the face and neck, with deeply pitted scars on the face. He was treated during fifteen years in a cancer institute for multiple well-differentiated " squamous cell carcinoma " on the neck, face, and tongue; local recurrence did not occur after treatment of the active tumors and there was no clinical evidence of secondary lymphnode involvement. His son, aged 30, teacher, came in July with a papular active tumour on the cheek, which was surgically removed; his face and ears soon became affected. In the two cases, numerous biopsies showed in the early stage a great down growth of the epidermis with a central horny plug, like in keratotic papules or small kerato-acan-thomas and in more active lesions, typical appearances of squamous cell epithelioma grade I. The familial incidence is proved in the majority of the 30 cases reported since the first description by Ferguson-Smith in 1934. Only caucasians are concerned, most of them being Scottish. There is no sex predominance. First changes generally occur in young adults on light exposed areas but mucous membranes can be affected. The individual lesions last for several months, but fresh ones appear continuously, so that the disease remains extensive. The cause of these tumors is still unknown; histological and ultrastructural findings cannot explain this condition. Our ultrastructural study is parallel to previous works and fails to find viral particles in keratinocyte nuclei or cytoplasma.

Carcinoma, Squamous Cell↗
PubMed1976

[Chronic urticarial lesions and macroglobulinemia. Apropos of 5 cases].

The authors report 5 cases whose main characteristics appeared very similar. Constantly, they found the same skin signs, urticaria without pruritus, recurring over a long period. The latter was accompanied by a very high E.S.R. and immuno-electrophoresis showed, in all cases, an increase in monoclonal IgB, permitting one to make the diagnosis of macroglobulinemia. In four cases out of five, this clinical picture was accompanied by bony pain associated with radiological signs of condensation. The symptoms were accompanied by prolonged fever and lymphadenopathy. After being well tolerated for a long period, the disease may become worse and lead to death. Thus this seems to be a true disease entity?

Aged↗
PubMed1976

Biopsy of the proximal nail fold in collagen diseases.

A research procedure used to study proximal rail folds in connective tissue diseases is described. The technique is simple and one that is suitable and agreeable to patients. It affords study of tissue from the proximal nail fold in three different ways, namely, light microscopy with histochemistry, electron microscopy, and direct immunofluorescence.

Biopsy↗
PubMed1976

[Cutaneous manifestations in disseminated intravascular coagulation syndrome].

The principal cutaneous manifestations indicating the need to seek intravascular coagulation are: purpura fulminans, extensive sheets of purpura, acralcyanosis, gangrene in a plaque distribution and perhaps, acute necrosis of the digits. The biological, anatomo-pathological and immunological signs of the conditions should be detected early and precisely by virtue of its nature as a medical emergency. The diversity of its clinical manifestations would seem to be related to the greater or lesser extent of the coagulopathy and resultant thrombosis in the microcirculation. Purpura, though of variable degree, is the constant cutaneous accompaniment of this condition, which is considered to be the equivalent of the experimental phenomenon of Sanarelli-Schwartzman. Heparin, which blocks the tendency to thrombosis in the small vessels in the treatment of choice, associated when possible with treatment of the underlying aetiology.

Adolescent↗