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Introduction: Sjögren-Larsson syndrome (SJS) is an autosomal-recessive disorder. Patients suffer from congenital ichtyosis, mental retardation and symmetric spastic paralysis. Ichtyosis is usually pronounced and associated with erythroderma. Neurological manifestations occur usually between 4 and 13 months of age. This genetic disease is due to fatty acid aldehyde dehydrogenase (FALDH) deficiency, leading to an accumulation of long-chain alcohols. The gene has been mapped to chromosome 17., Case Reports: A 52-year-old woman was hospitalized because of a severe erythroderma with ichtyosis. She suffered from epilepsy, spastic diplegia and mental retardation (Little disease has been diagnosed). The association of spastic paraparesia and ichtyosiform erythroderma suggested the diagnosis of SJS. This was confirmed by the very low level of FALDH activity. A 27-year-old patient was hospitalized for the recent onset paraparesia. Erythematous patches were observed on arm pits and buttocks. The diagnosis of SJS was not confirmed by FALDH assay., Discussion: Diagnosis of Sjögren-Larsson syndrome is a very rare disease in France. It is useful to evoke the diagnosis when spastic paraparesia is associated with these unusual cutaneous signs.

Aicardi syndrome is characterized by infantile spasms, agenesis of the corpus callosum and chorioretinal lacunae. This disorder affects mostly females with early embryonic lethality in males. We present a case of persistent hyperplastic primary vitreous (PHPV) in association with Aicardi syndrome in a 2-year-old girl.

On the occasion of the "Journées Dermatologiques de Paris", in March 1988 we designed a survey to evaluate the sterile practices of French dermatologists in the "AIDS era". During the two days meeting, attended by 2,584 participants, 472 questionnaire forms were filled in (answer rate 18.5 p. 100). Among responders 43 p. 100 were exclusively office-based, 13 p. 100 only worked in hospitals, and 44 p. 100 were practicing both in office and hospital. The overall utilisation of gloves by French dermatologists remained highly variable, depending on the procedure. For example 6.7 p. 100 of responders reported never using gloves for excisions, 13 p. 100 for shave biopsies, 18 p. 100 for punch biopsies, 40 p. 100 for curettage and 47 p. 100 for electrocoagulation. Five per cent of the dermatologists surveyed did not sterilize their curettes regularly and 42 p. 100 their electrocoagulation needles. Heat sterilization was the most commonly used, in 69 p. 100 of cases for curettes and 42 p. 100 for electrocoagulation needles. On the other hand, chemical tray sterilization was used in 58 p. 100 of the cases for electrocoagulation needles, and in 31 p. 100 for curettes. For chemical sterilization, 63 p. 100 of responders used alcohol, 15 p. 100 glutaraldehyde, 15 p. 100 sodium hydrochloride, and 7 p. 100 other agents, mainly quartenary ammonium compounds. Twelve per cent of the physicians surveyed have adopted the practice of using a single electrocoagulation needle, belonging to each patient, for long term repeated procedures.(ABSTRACT TRUNCATED AT 250 WORDS)

D-penicillamine has proved to be an effective treatment of rheumatoid arthritis, but adverse effects of this drug are frequent. The authors report 4 cases of respiratory disease in the course of treatments with D-penicillamine for which the drug may probably be held responsible. Two patients presented with diffuse alveolitis which improved on stopping D-penicillamine. Two patients presented with rapidly progressive dyspnea; chest X-ray showed distended lungs; lung function tests showed severe airflow obstruction with air trapping : treatment with bronchodilators and corticosteroids was ineffective. Several cases of patients with such features have been reported during the last few years; pathologic studies which were performed disclosed bronchiolitis. Clinicians should be aware of the severity of this type of bronchiolitis and stop D-penicillamine immediately in patients presenting with early respiratory symptoms.

Bronchogenic cysts represent about 10 p. 100 of all surgical tumours of the mediastinum. They can never be diagnosed with absolute certainty prior to the operation, but when they arise in their typical sites, they can be suspected with a high probability. However, they can occur in very atypical sites, in which case the diagnosis remains very hypothetical until the operation. The authors report a case of a cyst which developed under the diaphragmatic pleura, in direct contact with the dome of the diaphragm and attached to the mediastinum by a fine vascular pedicle which inserted in the root of the triangular ligament.

The authors report five cases of streptococcal gangrene and stress the fundamental role of surgical excision. Parenteral anti-streptococcal antibiotics should not lead to this essential step being forgotten, its early application being the most important element in prognosis. It is thus essential to clearly understand the local and general criteria of rapid diagnosis in this condition, which is far from rare.

The authors describe two cases of pulmonary fibrosis in patients suffering from ankylosing spondylitis. The diagnosis of tuberculosis was rejected on the basis of negative studies for tubercle bacilli and the total ineffectiveness of specific therapy. No evidence of microbial or parasitic infection was found. This fibrosis was associated with a considerable worsening in general condition. Nevertheless, the first patient remains alive 15 years after the onset of this pathological manifestation. The second patient died but autopsy was not possible. A wide review of the literature is undertaken in the light of these two cases.

Detailed investigations of 9 patients with dermatitis herpetiformis are presented. In all cases cutaneous lesions were controlled by dapsone alone or by dapsone and gluten free diet. Granular IgA deposits were found in 7 patients, linear IgA deposits in one, and C3 component of complement in one. 3 patients out of 8 tested, carried the specific HLA-B8 antigen. Despite an extensive investigation, no malabsorption was detected. Jejunal biopsies were performed in 8 cases. Jejunal villous flattening was observed in one patient. It improved after a 2 months gluten free diet on subsequent jejunal biopsies. D. H. seems peculiar in France as compared with case reports from other countries: low prevalence of gluten sensitive enteropathy; rare occurrence of the specific HLA-B8 antigen; incidence of D. H. seems to be low in France. It is noticeable that french incidence of coeliac disease is low as well. This suggests a genetic difference in the investigated population (low prevalence of HLA-B8 antigen) and/or different alimentary habits, particularly a low dietary gluten amount.

We have studied the incidence of internal malignant neoplasms on patients with squamous cell carcinoma (SCC). This incidence has been compared to expected number of malignant neoplasm in that population by a precise statistical analysis. Two-hundred and eighty-three SCC confirmed by histology are diagnosed on 235 patients (159 men, 76 women). These patients have been followed between December 1970 and November 1982 at the specialized consultation on cutaneous tumors. We have examined all these patients, questioned their doctors, consulted their medical case history, to search an internal malignant neoplasm. Twenty-eight patients had a malignant neoplasm: this malignant tumor has been found 11 times before and 17 times at the same time or after SCC. These 17 malignant neoplasms are the only statistically interesting cases. The annual incidence of malignant neoplasms related to age and sex has been evaluated by consulting French registers. The French incidence has been compared to foreign incidences. This incidence enables us to calculate the expected observed numbers (12 and 5). The expected numbers are 9.15 for men and 2.1 for women. These numbers are not statistically different from observed numbers (12 and 5). The incidence of internal malignant neoplasms on patients with SCC is not different from the incidence of general population.

Non-steroidal anti-inflammatory drugs (NSAI) may elicit various kinds of cutaneous side effects. The commonest ones are non-specific erythematous eruptions, sometimes with a phototoxic distribution, and urticaria. Vasculitis and severe bullous eruptions (Stevens-Johnson's syndrome and Toxic Epidermal Necrolysis) are rare but may have severe outcomes. The overall incidence of cutaneous reactions is about the same for all NSAI, 1 to 3 p. 100, during the clinical studies performed before marketing the drug, but this increases afterwards (up to 45 p. 100 for Benoxaprofen). Drugs with long half-lives may carry a higher risk for severe cutaneous reactions. NSAI are now the main cause of drug induced TEN. Urticarial reactions seem related to pharmacological phenomena while the pathogenic events leading to other kinds of skin reactions remain unknown. An hypersensitivity reaction is postulated. The therapeutic value of corticosteroids for the severe cutaneous side effects of drugs is still controversial.

Collagen is a macro-molecule of polypeptide chains arranged in a triple helix. The biosynthesis and degradation of collagen require complex enzymes systems; considerable progress has been made in their understanding in recent years. Collagen is the most important connective tissue of the body and five types are present in the lower respiratory tract. Genetic disorders of collagen (e.g. the Ehlers-Danlos or Marfan's syndrome) may affect the lung. Furthermore certain pulmonary diseases such as fibrosis are associated with changes in the ratio of the different types of collagen. Collagen can no longer be dissociated from the study of tissue changes in pulmonary pathology; the aim of our study is to provide elementary data concerning this aspect of modern pneumology.

Skin lesions in systemic candidiasis are erythematous maculopapules and maculonodules which appear at the onset of the septicaemic phase. Their presence, especially when associated with diffuse myalgia, suggest the diagnosis which must be confirmed by haemocultures, histological examination of the skin with PAS stain and culture of skin fragments in Sabouraud's medium. Systemic candidiasis with skin lesions seems to be mainly due to Candida tropicalis and to occur with great frequency in patients with underlying blood disease. Of the three cases reported here, one concerned a 24-year-old man with premyelocytic acute leukaemia, the second a 45-year-old woman with drug-induced agranulocytosis and the third one a man aged 25 admitted to hospital for peritonitis secondary to Crohn's disease.

A 60-year-old male patient with small carcinoma of the lung was treated with chemotherapy and radiotherapy. The tumour regressed, but two squamous cell carcinomas appeared in the contralateral lung. The first one was discovered after 3 months' treatment and developed slowly; the second one, diagnosed after 9 months' treatment, was rapidly progressive. The patient died 13 months after the beginning of treatment, and only the squamous cell carcinomas could be confirmed on autopsy. Such cases may reflect either selection of tumours that were present from the beginning of treatment, or development of other malignancies, or treatment-induced histological changes in the original tumour.

A case of agenesis of the pericardium is reported. The condition is are; a search in the literature has yielded 364 cases. It always results from an abnormal embryonic development. The agenesis is more common on the left side (70%) than on the right side (4%) or the inferior side (17%). It is more often partial than total (9%) and may be associated with other malformations, particularly malformations of the heart. The diagnosis rests on radiography of the chest and, mainly, echocardiography. CT, MRI or surgical exploration (if needed for other reasons) confirm the anatomical defect. In patients with partial pericardial agenesis the risk of cardiac entrapment makes surgery mandatory.

A case of syphitic hepatitis is reported. This rare manifestation during the second stage, is characterised by a important cholestasis and a moderated cytolysis. The biologic pattern and the histologic findings allow the difference with viral hepatitis. The manifestation is more frequent in homosexual people. The specific treatment is quickly effective and the short and long-term prognosis is good.

Case records of 26 patients with Lyell Syndrome were reviewed for studying haematologic abnormalities. Eosinophilia, neutropenia, thrombopenia were uncommon. Circulating immature granulocytic cells were frequently encountered during the second week of evolution, mostly when leucocytosis was present. Anemia was frequent, the lowest haemoglobin titer beeing reached by the 15th day. Reticulocytosis was initially low and reached a peak during the second week. At that time biological markers of inflammatory syndrome were getting worse. So the originating anemia seems primarily of medullary origin and independent of inflammatory syndrome. Lymphopenia was constant and sometimes marked, with no circulating lymphocytes in two cases. The lowest numbers of lymphocytes were observed during the first week. These haematological abnormalities may have some pathogenic significance.

Screening tests for circulating anticoagulant were performed systematically in fifteen patients with different stages of syphilis. The lupus-type anticoagulant, an inhibitor of the prothrombin activator complex, was demonstrated in one primary and three secondary stages, without any hemorrhagic complications. The possible relationship between the occurrence of this type of inhibitor and positive antilipoidal tests is discussed in reference to the well-known association of circulating anticoagulant and biologic false positive serologic tests for syphilis observed in patients with SLE.

The occurrence of morphea has been attributed to Borrelia, burgdorferi infection, but the relationship between localised scleroderma and borreliosis remains controverted. Antibodies directed against B. burgdorferi were looked for in 21 patients (18 female and 3 male, aged from 8 to 63 years) whose disease had been present for 6 weeks to 13 years. One patient had a single morphea, two had monomelic scleroderma and 18 had multiple localised morpheas. The search for antibodies was conducted in these 21 patients and in 200 blood donors from the Paris region by indirect immunofluorescence techniques, using the CDC method where only antibody titers of 1/256 or more are significant. Serology was lower than 1/256 in 20 cases and equal to 1/256 in one case. Among the 200 blood donors, five (2.5%) had antibody titers of 1/256 or more. None of the patients studied had been exposed to tick bite due to their occupation or place of residence. We therefore found no evidence of a link between B. burgdorferi infection and morphea in the Paris region.(ABSTRACT TRUNCATED AT 250 WORDS)

Seven patients ingested (2.5 mg/kg) daily dapsone. Their serum samples were analyzed daily during the first week by spectrophotometry for methaemoglobinemia and weekly blood samples for red blood cells, reticulocytes count and haptoglobin level. The acetylator phenotype of each patient was estimated on the capacity to acetylate isoniazid. Methaemoglobinemia was always observed within the 48 hours after drug intake. The dapsone induced anemia was moderate being maximum between day 25 and 40 after the beginning of treatment. Reticulocytemia appeared in the second week and haptoglobin levels decreased between day 5 and 8 of therapy in 5 patients and at day 15 for the two others. The seven patients are divided in 4 rapid acetylator and 3 slow acetylator. The acetylator phenotype was not correlated with methaemoglobin, haptoglobin levels or RBC counts.

A patient, 37 years old in 1966, presented hemoptysis and an excavated lesion of left upper lobe; there were no acid-fast bacilli in the sputum, but he was considered as having tuberculosis. After one year of treatment, the lesion had progressed and was removed surgically; pathologic diagnosis was silicosis. During the following years, three excavated lesions developed in the controlateral lung. When seen in 1979, the patient had consolidation of the whole right upper lobe and an excavated mass of right lower lobe. Amyloidosis was suggested; the pathologic sections of 1967 were reviewed and confirmed amyloidosis.

Cell typing of sub-populations of lymphocytes is facilitated by using monoclonal antibodies (OKT); with this technique we determined the relative proportions of "helper" T-lymphocytes (OKT4) and "Suppressor" T-lymphocytes (OKT8) in the blood and the LBA of 13 patients suffering from pulmonary sarcoidosis. In the control subjects the OKT4/OKT8 ratio was 1.9 +/- 0.3 in the blood (38 subjects) and of 2 +/- 0.2 in the LBA (6 subjects). Six patients suffering from inactive sarcoidosis (stabilised) had an OKT4/OKT8 ratio on average of 1.2 +/- 0.39 in the blood and of 1.86 +/- 0.46 in the LBA. By contrast in 7 patients suffering from "active" sarcoidosis, on average the ratio was 1.88 +/- 1.20 in the blood but 5.13 +/- 3.51 in the LBA; this ratio was notably raised in the LBA in 5 cases and was due as much to an increase in the T-"Helper" (OKT4) percentage as to a fall in the T-"Suppressor" (OKT8). It is probable that the T-"Helper" lymphocytes found in great numbers in the lung in active sarcoidosis cases participate in the formation of sarcoid granulomas and attract phagocytic mononuclear cells. This phenomenon seems to correspond to a local immune disturbance without producing a similar result in the peripheral blood of the patients.

A patient with eosinophilic fasciitis and insulin-dependent diabete mellitus is recorded. Diabete and blood hypereosinophilia occurred four months before acute onset of severe proximal sclerodermic features. The deep seated level of fibrosis, mainly localized in subcutaneous fat and fascia was documented by histologic examination. Prednisone therapy (0,75 mg/kg/day) resulted in cutaneous improvement and disparition of eosinophilia. Survey of pertinent literature lead us to consider Shulman's syndrome as a distinct entity amid the broad scleroderma spectrum. Features of esosinophilic fasciitis are very close to, and sometimes undistinguable from generalized morphea. Simultaneous occurence of diabete mellitus in this case is not fortuitous. The same association has already been recoreded with various sclerodermic syndromes.

87 cases of epidermoid carcinoma of the penis treated by Ir 192 interstitial radiotherapy are studied. The 5 years survival rate, estimated on a 51 patients group, is 61 p. 100. Among these 51 patients, 12 had a local recurrence. The post-radiotherapy early reaction is estimated on a 79 patients group, with a mean duration of 3, 4 months. The early reaction is correlated to tumor size and not correlated to number of radio-active lines. The later dermatologic evolution is studied in 59 patients. In 22 cases, the scar remained supple, without any further local change. Local changes occurring in the 37 other cases are studied in their clinical aspect, signification and frequency. Of 34 patients followed longer than 40 months, 14 had local complications with 4 cases of local recurrence. The carcinologic reliability of this method is similar to that by surgery. It seems however it must be restricted to tumors limited to glans penis.

The mucous lesions are customary in toxic epidermal necrolysis. The acute ocular lesions have led to definitive sequelae. 22 out of 32 patients surviving to a Lyell's syndrome lend themselves to an ophthalmological supervision. 13 of these 22 patients (59 p. 100) presented a sicca syndrome with a decrease of the lacrimal secretion (12 cases) and/or salivary secretion (8 cases). The sicca syndrome appeared sometimes from the beginning of the acute phase of the Lyell's syndrome or more often a few weeks later. This decrease of the lacrimal flux went along with objective corneal lesions among 11 patients, and 6 of them had a change of the visual function. A biopsy of the accessory salivary glands was performed on 7 of these patients having a reduction of the salivary flux. 5 of these 7 biopsies showed lymphocytic infiltrates giving in 2 cases a nodular aspect, grade III of Chisholm's classification, held to be pathognomonic of Sjögren syndrome. The 13 patients having presented a sicca syndrome differed from the other 9 patients (without sicca syndrome) by a higher severity of their Lyell's syndrome and by a higher age. None of our patients with dry syndrome had antinuclear antibody detected by immunofluorescence on sections of rat's livers. Sicca syndromes with sometimes lymphocytic infiltrate similar to those of Sjögren's syndrome were occasionally imputed to drug reactions. The occurrence of "Sjögren like's syndrome" after a toxic epidermal necrolysis suggests the intervening of autoimmune phenomena in the pathogenesis of this toxic epidermal necrolysis.(ABSTRACT TRUNCATED AT 250 WORDS)