Search

Pseudoxanthoma elasticum (PXE) is a rare form of congenital dystrophy of the elastic tissue, which is characterized by cutaneous, ocular and vascular impact. There are four clinical forms which correspond to 4 different genetic types (I or II, dominant or recessive). The vascular damage caused by the illness general limits the obstetric prognosis. Under our observation, the pregnancy of a woman 34 years of age suffering from type II PXE proceeded without problem.

Twenty-two measurements repeated non-sequentially on each of 10 patients by five observers were undertaken to determine their reliability for routine clinical use. Measurements without significant inter-observer variation or with a coefficient of reliability greater than 0.70 were cervical rotation, cervical lateral flexion, tragus to wall distance, fingertip to floor distance on sagittal and lateral flexion, C7 to iliac crest line distraction and modified Schober index. It is concluded that many of the currently used measurements are either statistically unreliable or clinically unhelpful in mild or moderate ankylosing spondylitis. The most clinically useful were cervical rotation using a protractor, cervical lateral flexion using a goniometer, thoracolumbar flexion as the C7 to iliac crest line distraction, thoracolumbar lateral flexion as the fingertip to floor distance and the modified Schober index.

1,25-Dihydroxyvitamin D3 [1,25(OH)2 D3] may be an immunomodulatory drug which could have a role in controlling collagen deposition, and inducing reversal of fibrosis in some tissues. These observations prompted a study of the possible use of this hormone for the treatment of scleroderma. A 35-year-old woman, who had been suffering from localized scleroderma for 2 years, was given oral 1,25(OH)2 D3 for 6 months. The effects of the treatment were evaluated using clinical and physical measurements (skin thickness, extensibility properties of the skin). The evolution of the patient's condition during the 6-month therapy suggests that 1,25(OH)2 D3 is beneficial in localized scleroderma. The mechanisms of action are discussed in relation to the literature, which suggests both immunoregulatory and inhibitory effects on fibroblast growth.

The authors review a series of 80 Benign Calcifying Epitheliomas (BCE), and describe their main clinical, histologic and prognostic features, in comparison with those reported in the literature. BCE or pilomatrixoma arises from the hair matrix, and represents approximately 1/1,000 of all skin tumors. Statistically, it is found more frequently among children under the age of 10 years and anatomic locations are mostly located on the face and upper limb. These benign tumors tend to develop slowly in subcutaneous tissue, and their treatment consists of simple surgical excision, except in some instances, where signs of more aggressive behavior can be found.

An exceptional case of nevoid basal cell carcinoma (NBCC) syndrome occurring with acrokeratosis verruciformis (AKV) in the same patient is presented. The occurrence of two rare autosomal dominant diseases in a patient suggests that AKV might be one of the different dyskeratoses of the NBCC syndrome. The clinical aspect of the flesh-colored papules of AKV, indistinguishable from flat warts, might nevertheless prompt performance of a biopsy.

Essential oil from the leaves of Sphaeranthus cyathuloides O. Hoffm. was examined by GC and GC/MS. The oil (2.9%) was found to contain trans-dihydrocarvone (67%) and cis-dihydrocarvone (26%) and some small amounts of isodihydrocarveol and neoisodihydrocarveol and limonene. The flower oil contained only small amounts of trans-dihydrocarvone (0.3%) and cis-dihydrocarvone (0.6%).

An exceptional case of nevoid basal cell carcinoma (NBCC) syndrome occurring with acrokeratosis verruciformis (AKV) in the same patient is presented. The occurrence of two rare autosomal dominant diseases in a patient suggests that AKV might be one of the different dyskeratoses of the NBCC syndrome. The clinical aspect of the flesh-colored papules of AKV, indistinguishable from flat warts, might nevertheless prompt performance of a biopsy.

We report three cases of calciphylaxis, associated with chronic renal disease and adrenal failure. Painful necrotic lesions was associated with hyperphophoremia and subcutaneous calcifications. We propose surgical debridement preceeded by the normalization of calcium-phosphorus product.

Biological and immunomodulatory properties of 1,25 (OH)2 D3 prompted an open prospective study of the hormone administered orally (1,5 μg/day) in 10 patients with systemic sclerosis and 4 patients with localized scleroderma. After the one-year treatment period, a significant improvement was observed in total skin score, range of motion of articles, functional index and carbon-monoxide transfer coefficient. These results suggest that 1,25 (OH)2 D3 may be beneficial in the treatment of patients with scleroderma.

Three cases of Xeroderma pigmentosum are reported, one of whom developed skin carcinogenesis before the age of 3. Defective DNA repair causes skin cancer and neurodegeneration. Management of such patients include the use of topical and systemic photoprotective drugs together with oral administration of retinoids for epithelioma prevention. Antenatal diagnosis by amniocentesis is possible.

Resume Les auteurs rapportent une serie de 9 affections systemiques associees a une serologie positive pour Yersinia pseudotuberculosis. 4 observations ont revele le tableau classique d'arthrite reactive isolee ou associee a un erytheme noueux; 2 celui d'une sacro-ileite ou d'un syndrome de Reiter. Les 3 dernieres ont eu une expression clinique inhabituelle, a savoir un syndrome de Lofgren, une vascularite leucocytoclasique cutanee avec polyarthrite et glomerulonephrite et une pseudopolyarthrite rhizomelique. L'antigene HLA B27 etait present dans 4 observations, soit 45 p. 100 des cas. A l'occasion de ces observations, les auteurs discutent, en raison de l'absence de certitude bacteriologique, les rapports entre ces syndromes et l'infection a Yersinia pseudotuberculosis. Bien que la preuve de l'appartenance au cadre des yersinioses ne puisse etre formellement etablie, certains faits apportent des arguments indirects en faveur de la realite du concept de yersiniose systemique a Yersinia pseudotuberculosis.

Resume A l'occasion de l'etude d'une vascularite cutaneo-systemique, d'une pseudopolyarthrite rhizomelique et d'un syndrome de Lofgren associes a une serologie positive pour Yersinia pseudotuberculosis (Y. pst.) les auteurs discutent, en raison de l'absence de certitude bacteriologique, les rapports entre ces syndromes et l'infection a Y. pst. Si de telles manifestations apparaissent rares ou inedites dans le cadre des infections a Y. pst. , elles contractent en revanche des rapports etroits avec des faits analogues attribues a Yersinia enterocolitica (Y.e.) dont le pouvoir pathogene dans le domaine des manifestations extra-intestinales est mieux demontre. Les particularites cliniques de chaque observation les isolent respectivement du cadre habituel des vascularites systemiques pour la premiere, du tableau classique de la pseudopolyarthrite rhizomelique pour la deuxieme, ou de la sarcoidose pour la troisieme. Leur association a une serologie positive pour Y. pst. leur confere une note d'originalite qui, confrontee au tableau de la fievre scarlatiniforme d'Extreme-Orient due a Y. pst. d'une part et aux cas de septicemie a Y. pst. suivis d'arthrites aseptiques rapportes dans la litterature, d'autre part, conduit les auteurs a ranger ces affections dans le cadre des manifestations systemiques imputables a Y. pst. .

A new method is described for quantitative measurement of the uptake of 99m Tc-methylene diphosphonate (MDP) by the sacro-iliac joints. The method uses ‘regions of interest’ providing advantages over the previously described ‘slice’ method; the two methods are compared in normal subjects, patients with known sacro-iliitis and patients with low back pain. Sacro-iliac activity, as calculated by the sacro-iliac index (SII) in normal patients, was shown to decrease with age in females but not in males. The SII was compared with radiographs of the sacro-iliac joints in the patients with known sacro-iliac joint disease and in those with low back pain. The method is useful for the exclusion of sacro-iliitis as a specific cause of back pain.

Resume Les auteurs rapportent l'observation d'une jeune femme noire, qui, 16 mois apres une suspicion de salpingite presenta une encephalite et un syndrome de Fitz-Hugh Curtis : le role de Chlamydia trachomatis semble pouvoir etre retenu dans ces deux affections sur la base d'elements cliniques, biologiques et evolutifs.

An ultrastructural study of 2 cases of lymphomatoid papulosis (Macaulay) showed the prevalence of lymphocytes in the dermal infiltrate, with a variable admixture of other inflammatory cells. These lymphocytes are normal or in lymphoblastic transformation; some are abnormal in size (up to 12 µm in diameter) and have corrugated or cerebriform nuclei like “small” Sezary cells. Histiocytes have an indented nucleus, an actively phagocytic cytoplasm and sometimes vermiforme inclusions 150–180 A wide. Circulating and dermal polymorphonuclear leukocytes are rich in caryoschisis. In short, the ultrastructural pattern is inflammatory and also close to lymphomas.

A study of anaphylactoid reactions (AR) observed between September 1982 and September 1983 was carried out in the surgical departments of a French regional hospital. The patients who had presented clinical symptoms suggesting an AR (bronchospasm, collapse, tachycardia, with or without skin rash) during a general anaesthesia were included in this study. A precise history of previous anaesthesias and allergy was taken; allergological testing was carried out six to eight weeks after the AR. It included intradermal skin tests (ST) and a human basophil degranulation test (HBDT) with the suspected drugs. Out of 12,855 patients operated on under general anaesthesia in the hospital, 21 AR were seen during the year under study, in 18 women and 3 men, of median age 27 years (extreme values: 11 and 62). The median number of previous anaesthesias was 2 (extreme values: 0--in 4 cases- and 22). Cardiocirculatory abnormalities were the most frequent clinical symptoms of the AR: they consisted of decreased arterial pressure in 13 cases, with 8 cases of vascular collapse. Respiratory symptoms were less frequent but severe bronchospasm was observed in 5 cases. Skin rashes were seen simultaneously in 13 out of the 21 observations. A history of allergy was found in 11 patients. Total IgE serum concentration averaged 134 kU X 1(-1) (extreme values: 32-378). Results of histamine-sensitivity skin tests were not significantly different from those observed in a control group. Calcemia and magnesemia were in the normal range. One to four drugs were tested in each patient: 41 tests combining ST and HBDT were carried out.(ABSTRACT TRUNCATED AT 250 WORDS)

SUMMARY This paper describes our experience with the use of razoxane (ICRF 159), a new anti-mitotic drug, in the treatment of thirty-six patients with severe psoriasis. The drug has proven highly effective both in cutaneous and in arthropathic disease. The drug is well tolerated by patients and appears to be free of hepatotoxicity. However, it causes depression of the neutrophil leukocyte count, an effect which is entirely dose-dependent; the hazard to patients can be minimized by careful supervision of therapy.

Are Epidermodysplasia verruciformis (E. V.) and disseminated flat warts different diseases? Are there any diagnostic criteria between them? In order to attempt answering these 2 questions, fundamental for prognosis and nosology, a comparative ultrastructural study was made of epidermal clear cells of 2 cases of E. V. and 4 flat warts from 4 patients of whom 3 were under immunosuppression drugs. The reason of cytoplasmic electron translucency was mainly a reduction in tonofilaments and keratohyalin amounts in E. V. and a centrifugal edema and vacuolization in flat warts. On the other hand, the number of ribosomes was raised in E. V. and reduced in flat warts. These findings allow differentiation between the 2 diseases and suggest a possible different host-virus relationship.

Incubation of human plantar stratum corneum (SC) with trypsin at various pH suppressed the typical “keratin pattern” in the cytoplasm of the horny cells and disclosed tonofilaments and desmosomal attachment plates as they are seen in living keratinocytes. Tonofilaments were 62–75 A wide and showed a 100–120 A periodicity. This effect was non-specific since it was also obtained by incubation of SC with citric acid or sodium citrate, but it was not due to hydration alone. Partial acantholysis was obtained by incubation with trypsin followed by citric acid. After simultaneous action of trypsin in sodium citrate and in citric acid, cell membranes were separated from the cytoplasm and many of them were broken around desmosomes with subsequent isolation of desmosomes. The role of trypsin in this effect was uncertain. These findings strongly suggest that the filamentous network built up by living keratinocytes remains almost unchanged during the keratinization process although no longer visible under E.M.

Three cases of gold-related neuropathy are reported. Clinical features include an acute, symmetrically progressive polyneuropathy, focal or generalized myokymia and a tendency for initial neurological deterioration followed by improvement, after cessation of chrysotherapy. The degree of clinical recovery related to maximal disability. Morphological findings on sural nerve biopsies revealed both axonal degeneration and segmental remyelination. Similar peripheral nerve histology was seen in a parallel animal study in which the severity of the neuropathy was dose-related.

Resume Une sensibilisation cutanee au dinitrochlorobenzene (DNCB) et des intradermo-reactions a la tuberculine, a la candidine et au vaccin bacterien CCB ont ete pratiquees chez 58 sujets normaux, 9 diabetiques et 16 alcooliques. Le test au DNCB a donne 90 p. cent de reponses positives, contre 60 p. cent a la candidine, 71 p. cent a la tuberculine et 65 p. cent au vaccin CCB. L'indice de positivite est superieur chez la femme pour le DNCB, la candidine et le CCB mais inferieur pour la tuberculine. Cet indice diminue avec l'âge pour les quatre antigenes. Le diabete ne semble pas l'influencer, par contre il est diminue en cas d'hepatite alcoolique.

The study of biological or chemical processes leads generally to a non linear model with a cascade structure and in which the same parameter can appear in different non linear parts. The number of identification method which can be used with such a class of system is very limited. The identification method proposed in this paper is based on the parametric space division. The subspaces are defined so that the parameters which belong to one subspace have the same influence at the same time on the output. This influence can be represented by the sensitivity function. A similarity coefficient of the sensitivity functions is defined and used to determine the parametric subspaces. We have to note that one parameter can belong to different subspaces. The parameter identification is obtained using the gradient method in each subspace, and one subspace after the other. The results obtained using this method are compared with those given by gradient and axis direction method. The model used for this comparison is that of a waste water biological treatment system.

A 75-year-old male presented with typical cutaneous lesions and pulmonary involvement due to lymphomatoid granulomatosis. A legionellosis was associated with this condition. Though the radiological findings in lymphomatoid granulomatosis are suggestive, they are not specific, and set a difficult problem in distinguishing the two conditions. Monoclonal antibody studies indicated the predominance of CD8 T lymphocytes in the infiltrate of the skin, as well as in the bronchoalveolar fluid.

We report the case of a man with epidermolytic hereditary palmoplantar keratoderma. After failure of topical and systemic therapy, excision and skin grafting of both hands was done in several steps. Attention must be given to the skin-repair procedures (split- or full-thickness grafting), and the exact setting of the suture lines in the fingers is important.

In this study, the clinical and histopathological aspects of 50 plantar warts are reported in relation to the type of papillomavirus present in the lesions, as detected by immunofluorescence tests, using specific guinea pig fluorescein-labelled IgG. Warts of plantar localization are not caused by the same human papillomavirus (HPV) since they are found to be associated with both HPV type 1 (HPV-1) and HPV type 2 (HPV-2). HPV-1 is always associated with deep and painful plantar warts (myrmecia), whereas HPV-2 is found to be associated with superficial, painless plantar warts (vulgaris or often mosaic type). Histologically, these two types of plantar warts are quite different. In myrmecia (HPV-1), characterized by an endophytic growth, large eosinophilic, keratohyaline-like granules are observed in the cytoplasm and nucleus of infected, often vacuolated cells. These granules appear early in stratum spinosum and are very numerous in stratum granulosum. In the mosaic type (HPV-2), the histopathological aspect is not different from that of common warts; these lesions have an exophytic growth and are characterized by foci of clear vacuolized cells which are found in stratum granulosum. Their cytoplasm contains round, basophilic keratohyalin granules which often have a heterogenous aspect. These differences are observed in other localizations of morphologically related warts associated with HPV-1 and HPV-2 and seem to be related to a specific cytopathogenic effect of HPV-1 and HPV-2 in human papillomas.

An ultrastructural study of the dermis in a case of acrogeria is presented and compared with a healthy control of same age and sex. The overgrowing orceinophil material was mainly pseudoelastic. The collagen was hypoplastic and mostly immature. Inside and outside the fibroblasts a lot of a granulofilamentous substance intermingled with pseudoelastin and immature collagen was observed. Fibroblast cytoplasm was often distended by ergastoplasmic vacuoles filled with granulo filamentous substance and contained numerous pseudomyelinic bodies. These abnormalities suggest that acrogeria might be a fibroblast disease.

In an attempt to determine the viral content and to observe the morphological features of clear cells in human warts of various clinical types, 17 lesions were studied with the electron microscope and 26 in thick sections. Plantar warts were, by far, the richest in viral particles, which are often found in crystalline arrangement. Morphologically, three groups observed in thick sections were also discovered with the electron microscope. In flat warts, poor in keratohyaline, the vacuolating process was total and massive, with abundant viral particles in the nucleus; two flat warts were taken from immunosuppressed patients and their appearance was quite similar to epidermodysplasia verruciformis lesions. In common warts, poorer in virus particles but rich in keratohyaline, vacuolization was often not complete. In plantar warts, characterized by their peculiar wealth in viral particles sometimes invading the cytoplasm, the production of keratohyaline was tremendous and the vacuolization, although often complete, was, however, not massive. Thus it seems that keratohyaline production is related to viral production but hampered by the vacuolating process and differing according to the ultrastructure of the three types of warts. It is suggested that the oncogenic potential of a wart (papilloma) may be related to decrease in keratohyaline production, i.e. cell maturation, and increase in vacuolization.

In this report we present 21 instances in which live rubella virus was isolated from synovial fluid obtained from 6 cases of inflammatory oligoarthritis or polyarthritis over a period of 2 years in the absence of firm clinical evidence of rubella. In 3 cases (cases 1, 2, 6,) a persistent oligoarthritis predominantly affecting the knee joints occurred in 2 adult women and one man, lasting to date 27, 29, and 18 months respectively, and in one of these cases virions were found in cells of the synovial membrane. In case 3 a boy of 9 presented with an illness indistinguishable from the systemic variety of juvenile chronic arthritis (Still's disease). In case 4 a young man with persistent monoarthritis was found to have ankylosing spondylitis, and in case 5 a progressive erosive polyarthritis developed 5 years after an attack of rubella complicated by rubella arthritis. The virus was identified by a variety of virological techniques and infection confirmed by immunofluorescence and (in one case) electron microscopy.

The response of peripheral blood and synovial fluid lymphocytes to three non-specific mitogens has been studied. The paired samples were taken from patients with a range of inflammatory arthritides. Unstimulated synovial fluid lymphocytes (SFL) tended to have a greater uptake of tritiated thymidine than had unstimulated peripheral blood lymphocytes (PBL). This background uptake of tritiated thymidine by SFL showed a positive correlation with the response these SFL then showed to the mitogens. A significant depression was observed in the SFL response to phytohaemagglutinin when compared with the paired PBL response; this was seen in both the rheumatoid arthritis and other inflammatory joint diseases groups. SFL responses to concanavalin A and poke weed mitogen, although depressed in individual cases, failed to show a significant depression overall. Attempts to restore the SFL response to that of the paired PBL by removal of any possible blocking substance from the cell surface either by pre-incubation of S...