Dermatologic manifestations of relapsing polychondritis. A study of 200 cases at a single center.

Dermatologic manifestations of relapsing polychondritis (RP) have been relatively poorly studied compared to other manifestations. In this study we describe dermatologic manifestations in a large series of patients with RP and the corresponding pathologic findings. In this retrospective, single-center review of 200 patients diagnosed with RP according to Michet's criteria, we analyzed separately those suffering from associated diseases with potential dermatologic involvement or chronic dermatitis. Skin or mucosal biopsies taken from 59 patients were examined without knowledge of the clinical data. Among the 200 patients with RP, 73 had chronic dermatitis or associated diseases with potential dermatologic involvement, especially hematologic disorders (n = 24) and connective tissue diseases (n = 22). Among the other 127 patients, 45 (35.4%) had dermatologic manifestations: aphthosis (n = 21; oral in 14 and complex in 7), nodules on the limbs (n = 19), purpura (n = 13), papules (n = 10), sterile pustules (n = 9), superficial phlebitis (n = 8), livedo reticularis (n = 7), ulcerations on the limbs (n = 6), and distal necrosis (n = 4). Dermatologic manifestations were the presenting feature of RP in 15 cases (12%), and appeared concomitantly (n = 23) or not (n = 22) with attacks of chondritis. Histologic findings included vasculitis (n = 19, leukocytoclastic in 17 and lymphocytic in 2), neutrophil infiltrates (n = 6), thrombosis of skin vessels (n = 4), septal panniculitis (n = 3), and minor changes (n = 2). Patients with and without dermatologic manifestations did not differ with regard to male/female ratio; age at RP onset; frequency of auricular, nasal, or tracheobronchial chondritis; or frequency of rheumatologic, ocular, audiovestibular, renal, arterial, or venous involvement. The frequency of dermatologic manifestations (91% versus 35.4%; p < 0.0001), sex ratio (18 male/4 female versus 44 male/83 female, p < 0.0001), and age at first chondritis (63.3 +/- 14 yr versus 41.4 +/- 17 yr; p < 0.0002) were significantly higher in the 22 patients with myelodysplastic syndrome than in the 127 patients without any associated disease. In conclusion, although dermatologic manifestations occur frequently in patients with RP, especially in association with myelodysplasia, they are nonspecific and sometimes resemble those observed in Behçet disease or inflammatory bowel diseases. Their presence in the elderly warrants repeated blood cell counts to detect a smouldering myelodysplasia.

IgA antineutrophil cytoplasmic antibodies in cutaneous vasculitis.

BACKGROUND: Antineutrophil cytoplasmic antibodies (ANCA) of the IgA isotype have, for the most part, been detected in patients with Henoch-Schönlein purpura (HSP) or inflammatory bowel disease. OBJECTIVES: We have evaluated the prevalence of IgA ANCA in a series of patients with different causes of cutaneous vasculitis. METHODS: Forty consecutive patients with histologically proven leucocytoclastic vasculitis were included in the study: 18 had systemic vasculitis as well as cutaneous lesions, 10 of whom were diagnosed as having HSP, and 22 had only cutaneous vasculitis (with no identified cause in 10 cases). IgA ANCA were sought by indirect immunofluorescence using ethanol-fixed human neutrophil preparations as the substrate. RESULTS: IgA ANCA were detected in six of 40 patients (15%) (one each with HSP, ulcerative colitis, Sjögren's syndrome, hypergammaglobulinaemia associated with Castelman's disease, erythema elevatum diutinum and bacterial endocarditis). Three of these patients also had IgG ANCA whose target antigen remained unidentified. CONCLUSIONS: IgA ANCA are rarely observed in HSP (10%) and can be detected in a wide variety of other cutaneous vasculitides.

Allogeneic bone marrow transplantation in aggressive non-Hodgkin's lymphoma (excluding Burkitt and lymphoblastic lymphoma): a series of 73 patients from the SFGM database. Sociét Française de Greffe de Moelle.

The place of allogeneic bone marrow transplantation (BMT) in the treatment of aggressive non-Hodgkin's lymphoma (NHL) remains controversial. We conducted a retrospective study of French experience in allografting NHL between 1984 and 1994. To improve the homogeneity of the study population, cases of low-grade, Burkitt and lymphoblastic NHL were excluded. 73 patients were included in the analysis. Median age at transplantation was 35 years (range 9-61 years); 64 patients were in stage IV and 45 had bone marrow involvement at diagnosis. At the time of transplantation, 46 patients had sensitive disease (25 in complete remission; CR). The overall survival (OS) and progression-free survival (PFS) rates were 41% and 40% respectively at 5 years (median follow-up of survivors 90 months). The probability of disease progression was 30% at 5 years, and only one relapse occurred after 15 months. 32 patients died of transplantation-related complications. In multivariate analysis, pretransplant complete remission was the main factor associated with longer survival (OS at 60 months of 76% among the 25 patients in CR at transplant and of 23% among the 48 patients not in CR at transplant). Neither acute nor chronic graft-versus-host disease (GvHD) influenced the relapse rate. In conclusion, in this high-risk population the overall results of allogeneic BMT were encouraging, despite a high transplant-related mortality rate. We believe this procedure should be studied further in prospective controlled trials.

Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients.

Sneddon syndrome is characterized by the association of livedo reticularis and cerebral ischemic arterial events (stroke or transient ischemic attack). Reported prevalence of antiphospholipid antibodies is highly variable. We conducted this study to compare the clinical and pathologic features of patients with Sneddon syndrome according to the presence or absence of antiphospholipid antibodies. Forty-six consecutive patients with Sneddon syndrome were analyzed. All were examined by the same dermatologist who classified the livedo of the trunk according to the regularity of the fishnet reticular pattern and according to the thickness of the fishnet reticular pattern (> or = 10 mm = large; < 10 mm = fine). Skin biopsies were systematically performed, from both the center and the violaceous netlike pattern in 38 patients. Antiphospholipid antibodies-positive Sneddon syndrome was defined by the presence of lupus anticoagulant or abnormal titers of anticardiolipin antibodies on repeated determinations. Group I consisted of 27 antiphospholipid antibodies-negative patients and Group II, of 19 antiphospholipid antibodies-positive patients. All patients except I in Group II had irregular livedo reticularis. Large livedo racemosa was more frequently observed in Group I (89%) than in Group II (21%, p < 0.001). On skin biopsy, arteriolar obstruction was detected in only 8 patients (4 in each group). The following parameters were not statistically different between the 2 groups: gender, mean age at detection of livedo, mean age at first clinical cerebral event, hypertension, Raynaud phenomenon, patients with extracerebral and extracutaneous arterial or arteriolar thrombosis or stenosis, patients with venous thrombosis, and women with 2 fetal losses or more. In contrast, seizures (11% in Group I versus 37% in Group II, p < 0.05), mitral regurgitation on echocardiogram (19% versus 53%, p = 0.02), and thrombocytopenia < 150,000/muL (0% versus 42%, p < 0.005) were more frequently observed in Group II. The number of events per year of follow-up was lower with antiplatelet therapy (0.08 versus 0.5) in Group I, but was not different with anticoagulation (0.056 versus 0.06). Antiphospholipid antibodies-negative and -positive patients with Sneddon syndrome belong to close but different subsets of Sneddon syndrome.

[Pruritus and the hepatitis C virus. The MULTIVIRC Unit]. / Prurit et virus de l'hépatite C. Le Groupe Multivirc.

OBJECTIVE: Determine the characteristics of pruritus in a selected population of patients with positive hepatitis C virus serology. PATIENTS AND METHODS: In a retrospective study, we re-examined outpatient reports for patients who consulted for pruritus from January 1993 to April 1996 and were followed in a hepatology unit for hepatitis C infection. The following data were collected: age, sex, risk factors, HIV and HBV serologies, duration of pruritus and diagnosis, ALAT, gamma GT and total bilirubin, METAVIR score, HCV RNA PCR, search for cryoglobulins, antiviral and dermatology treatments. There were 1,060 patients followed in the hepatology unit during this period, including 327 with cirrhosis. RESULTS: Twenty-seven patients were retained for study, 16 men and 11 women, mean age 53 years. None of the patients had HIV infection, 7 had a past history of hepatitis B infection (positive for anti-Hbc antibodies). Median duration of pruritus prior to consultation was 3 months (95 p. 100 CI: 3 months-2 years). Pruritus was associated with non-specific lesion in 19 cases (70 p. 100: 95 p. 100 CI: 51-85 p. 100). There were excoriated eczema-like lesions in 11 cases (41 p. 100: 95 p. 100 CI: 15-49 p. 100). Other causes were urticaria in 5 cases (18 p. 100: 95 p. 100 CI: 7-36 p. 100), including 1 case of urticarial vasculitis with cryoglobulinemia, 2 cases with atopic dermatitis, and 1 case of primary biliary cirrhosis. In four cases, lichen planus was associated. Skin biopsies were obtained in 10 patients and showed eczema-like alterations in 9 and urticarial vasculitis in 1. Mean ALAT and gamma GT levels were 2.6 N (95 p. 100 CI: 1.9 N-3.3 N) and 2.2 N (95 p. 100 CI: 1.4 N-3 N) respectively, including 11 cases without cholestasis (normal gamma Gt). PCR was positive in 13 cases out of 15. Cryoglobulinemia was found in 10 cases out of 24. At consultation, 3 patients were given ursolvan, 7 interferon, 1 ursolvan with ribavirin, and 3 an interferon-ribavirin combination. Dermatology treatment associated antihistamine agents, emollients, and corticosteroids. This population of hepatology patients referred for pruritus comprised 2.5 p. 100 of all patients (95 p. 100 CI: 1.7-3.6 p. 100). Among them, 1.8 p. 100 (95 p. 100 CI: 1.1-2.7 p. 100) had eczema-like lesions associated with cutaneous xerosis. DISCUSSION: Pruritus in our patient population was generally associated with non-specific excoriations, prurigo or xerosis in 19 cases (70 p. 100). As only ambulatory patients were retained for analysis, this is not a comprehensive population and the percentage of non-specific pruritus, evaluated at 1.8 p. 100, is probably an underestimation. Cholestasis cannot explain alone these manifestations since 11 patients had normal gamma GT levels. Several etiologies could be involved: a direct effect of HCV, interferon. A prospective study should allow an estimation of frequency, risk factors and possible impact on quality of life.

Annular atrophic lichen planus and Sneddon's syndrome.

We report the case of a patient who had 2 rare diseases, annular atrophic lichen planus (AALP) and Sneddon's syndrome (SNS). This patient had also digital nodules with histological abnormalities suggestive of SNS vasculopathy, which have not been reported so far. AALP is the most rare of all varieties of lichen planus since this case is the third reported to date. The association of livedo racemosa and cerebrovascular disease is the hallmark of SNS, the incidence of which is estimated to be 4 cases per year per million inhabitants. In both diseases, an abnormal production of elastic-tissue-degrading enzymes or a constitutional abnormality of the elastic tissue can be postulated, since SNS is characterized by arteriolar changes with deterioration of the internal elastic lamina and AALP by destruction of the dermal elastic tissue.

[Hamartoma of Brunner's glands. Report of two cases]. / Hamartome des glandes de Brünner. A propos de deux observations.

Two cases of duodenal hamartoma of the Brunner's glands are reported in a 41-year-old woman and a 61 year-old man. These polypoid lesions measured more than 4 cm and presented with haemorrhages. These forms are infrequent. Because of their atypical and sometimes severe clinical symptoms, and their endoscopic appearance, a diagnosis of malignancy could be done. Their pathological features demonstrate that these benign lesions have a dysembryoplastic pattern and can be called hamartomas.

[Granular cell tumor of the gingiva in the newborn. Report of a case in Martinique]. / Tumeur à cellules granuleuses de la gencive chez le nouveau-né. A propos d'un cas en Martinique.

The authors report a case of a gingival granular cell tumor, also called "congenital epulis", occurring in a female newborn. It is a rare congenital, benign tumor of uncertain histogenesis, with marked predilection for female newborn. The tumor arises, as a polypoid mass, on the gingiva of the anterior maxillary or mandibular alveolar ridge. Typical histologic features are represented by sheets of large cells with pale granular cytoplasm. Despite remarkable similarities between gingival granular cell tumors and others ubiquitous granular cell tumors (granular cell myoblastoma or Abrikossoff's tumor), histogenesis of the gingival granular cell tumors seems different. Unlike those of granular cell myoblastoma, granular cells of gingival granular cell tumor do not express S-100 protein and Neuron-Specific-Enolase. Considering the positivity for oestrogen and progesterone receptors, a hormonal factor may be important in the development of gingival granular cell tumor. The generally accepted treatment is simple excision.

[Carcinosarcoma of the breast. Anatomic-clinical study of a case]. / Carcinosarcome du sein. Etude anatomoclinique d'un cas.

The authors report the case of a 66 year old woman, who underwent surgery for carcinosarcoma of the breast. This tumour consisted of an infiltrating ductal carcinoma intermingled with sarcomatous proliferation, resembling malignant fibrous histiocytoma, without evidence of any transition between the two. Carcinosarcomas of the breast are rare tumors. Their definition and their histogenesis are discussed in the literature. Their prognosis is worse than that of metaplastic carcinomas of the breast, particularly spindle cell carcinomas, justifying precise histological classification of these biphasic tumors.

[A rare ovarian pseudotumor: massive edema of the ovary]. / Une pseudo-tumeur ovarienne rare: l'oedème massif de l'ovaire.

The authors report a case of massive ovarian edema which declared itself by pain in the abdomen and pelvis and an ovarian mass measuring 13 cm in diameter, occurring in a 22-year-old woman. Since it was not possible to make a diagnosis by any frozen-section examination, histology was carried out on the ovary that had been removed. This showed that the stroma of the ovary had become separated by massive edema preserving the albuginea and the superficial cortex. This case history of massive edema of the ovary shows the characteristics of this ovarian pseudotumour as described in the literature. The principal differential diagnoses of the condition are oedematous fibroma, and myxoma of the ovary. Apart from the fact that torsion of the adnexae can occur in some of these cases, the pathogenesis is still unexplained. When an ovarian tumour is found in a young woman a frozen-section examination must be carried out to make the diagnosis and perhaps avoid oophorectomy, particularly when untwisting a torsion can lead to resorption of the edema.

[Sensory fibers sensitive to capsaicin can modulate secretion of the duodenal mucus. A morphometric study in rats]. / Les fibres sensitives sensibles à la capsaïcine peuvent moduler la sécrétion de mucus duodénal. Etude morphométrique chez le rat.

Many in vitro and in vivo models have been used to study the modulation of intestinal mucosecretion. In such studies, quantification of mucus secretion is usually difficult, due to several technical problems. Whether sensory mechanisms participate in the modulation of intestinal mucosecretion remains unknown. The development and assessment of a morphometric method with computer assisted image analysis that allows to detect and quantitate mucin secretion by duodenal goblet cells are reported. Using this method, the secretory effect of pilocarpine on villus and crypt goblet cells was confirmed. We also determined whether sensory neurons can regulate intestinal mucosecretion by using capsaicin, a vegetal neurotoxin specific of a subpopulation of afferent primary sensory neurons. Intravenous capsaicin administration (10 micrograms/kg) increased mucus secretion by the goblet cells of the duodenal crypts. This suggests that sensory neurons may modulate duodenal mucin secretion. The "local effector function" of these neurons might be involved, in part through the release of substance P because exogenous substance P was able to increase mucin excretion by goblets cells of duodenal villi. Substance P, however, did not exactly mimic the capsaicin effects, suggesting that other factors were involved.