Informative value of monitoring of immune status and genome expression in blood leukocytes in psoriatic patients.

Comparative analysis of association with psoriasis before and after treatment of 53K/H2A and 43K/H2A leukocyte protein markers and parameters of leukocyte population (18 indexes) including concentration of peripheral blood lymphocyte subpopulations as markers of the immune status revealed advantages of the former method of patient monitoring for the evaluation of treatment efficacy. The method showed 100% sensitivity and correlation with the dynamics of clinical symptoms. A significant correlation of 53K/H2A parameter with blood content of CD3+, CD4+, CD8+, and CD72+ lymphocytes was established.

Abnormalities of human genetic apparatus manifested in leukocyte proteins in psoriasis.

The content of 53K/H2A and 43K/H2A proteins in leukocytes of patients with psoriasis, newborns, and healthy donors was studied. In all patients one or both parameters differed from normal. Mean values and distribution of 53K/H2A in healthy siblings of psoriatic proband significantly differed from those in newborns and donors. It was concluded that 53K/H2A is a marker of pathological changes in the genome responsible for predisposition to psoriasis. This marker can be revealed in peripheral blood leukocytes before skin manifestations of the disease.

[Congenital ichthyosiform erythrodermas: aspects of their etiology and pathogenesis]. / Vrozhdennye ikhtioziformnye éritrodermii: nekotorye aspekty étiologii i patogeneza.

The term "inherited ichthyosiform erythrodermia" (IIE) designates a heterogeneous group of inherited disturbances of keratinization. Lamellar ichthyosis (LI), inherited nonbullous ichthyosiform erythrodermia, bullous inherited ichthyosiform erythrodermia (BIIE) and some other conditions. Processes of terminal cell differentiation and epidermal keratinization are affected in all the above conditions. A decrease of transglutaminase activity is observed in L1, this being connected with mutations in chromosomes 14q, 2g33-35. In BIIE which is characterized by dense groups of tonofilaments in the suprabasal layer, mutations of keratin I and X coded by chromosomes 2 and 17 are identified. When sporadic forms of the disease occur, the main question is establishment of the genotype-phenotype correlation which depends on detailed characteristics of each patient and accurate study of the ultrastructural disturbances.

[Mapping the gene for palmoplantar hyperkeratosis (thylosis) to chromosome 17 in the 17q12-q24 region]. / Kartirovanie gena ladonno-podoshvennogo giperkeratoza (tiloza) na 17-i khromosome v raione 17q12-q24.

Mapping of the genetic defect causing dominant palmoplantaris hyperkeratosis (PPHK) was continued based on the material of an extended Uzbek pedigree. No linkage between the PPHK gene and hypervariable DNA markers from 8p, 12p, 14q, and 22q were revealed. The study of PPHK gene linkage with DNA markers covering the entire length of 17th chromosome mapped the PPHK gene to 17q12-q24 and revealed close linkage with KRT10 and D17S800 loci (zero recombination frequency at a lod score > 7). The possible location of a PPHK mutation in one of the keratin genes mapped to the same region on the 17th chromosome is discussed.

[Interferon as a stimulator of DNA repair in patients with xeroderma pigmentosum]. / Interferon kak stimuliator reparatsii DNK u bol'nykh xeroderma pigmentosum.

Natural leucocyte interferon (IF) upon injection to two patients affected with Xeroderma pigmentosum for three weeks stimulated the inhibited DNA replication and Host reactivation at the level of healthy donors. Tigasol--the drug used traditionally for the treatment of diseases caused by Xeroderma pigmentosum proved less effective than IF.

Identification of the genetic locus for keratosis palmaris et plantaris on chromosome 17 near the RARA and keratin type I genes.

Familial keratosis palmaris et plantaris (KPPF) is characterized by extreme keratinization and desquamation of the skin of the palmar and plantar surfaces of the hands and feet. We have mapped the causative genetic defect to an 8 cM interval on 17q12-24 in or close to the acidic keratin (type I) gene cluster. We show that KPPF co-segregates with a rare, high molecular weight allele of an insertion-deletion polymorphism in the C-terminal coding region of the keratin 10 gene (Z = 8.36 at theta = 0.00) and segrates as a true autosomal dominant trait. Some pedigrees with familial hyperkeratosis of the palms and soles have co-inherited diseases such as congenital malformations and familial cancers. Our analysis provide a region which should be investigated for contiguous gene syndromes in such pedigrees.

[The detection of Trichomonas vaginalis in the rectum of patients with urogenital trichomoniasis]. / Vyiavlenie vlagalishchnykh trikhomonad v priamoi kishke bol'nykh mochepolovym trikhomoniazom.

Inoculations of scrapings of the rectal mucosa from 53 women with various urogenital diseases and urogenital trichomoniasis yielded Trichomonas growth in Johnson-Trassel's medium in 8 cases. Vaginal and rectal cultures of 6 patients were examined by scanning and transmission electron microscopy. Vaginal Trichomonas were detected in rectal and vaginal cultures of 6 patients. Intestinal Trichomonas were identified in rectal isolates from 5 patients.

[Hereditary pemphigus: the problems of classification, diagnosis and prevention]. / Nasledstvennaia puzyrchatka: voprosy klassifikatsii, diagnostiki i profilaktiki.

Basic principles of present-day classification of hereditary epidermolysis bullosa are outlined, and morphologic grounds for distinguishing common, borderline, and dystrophic e. bullosa are validated. Potentialities of prenatal diagnosis of grave forms of e. bullosa as one of the major means of prevention are discussed. Data of clinical follow-up of 25 patients with common, 5 with borderline, and 29 ones with dystrophic e. bullosa are presented, as are results of 8 cases of prenatal diagnosis. The authors emphasize the necessity of electron microscopic examination of the skin in every case.

[Rubrophytosis of the face]. / O rubrofitii litsa.

The authors review the data on the incidence and type of fungal involvement of the face in T. rubrum-induced trichophytosis in the USSR and other countries of the world. Exquisite localizations are cited, i.e. involvement of the lacrimal nasal channel, external acoustic meatus, eyelids and eyelashes. Extraordinary variants of trichophytosis of the face are described: tumor-like, wart-like, infiltrative suppurative, seborrheal, as well as the forms simulating photodermatoses, perioral dermatitis, etc. 35 case histories of patients suffering from this condition, observed by the authors over 21 years, are cited, and photographs are presented of 8 of them with the most incident trichophytosis variants, i.e. seborrheal, infiltrative suppurative, and those simulating discoid lupus erythematosus and perioral dermatitis. The authors emphasize that fungal nature should be excluded in cases with uncommon course of this or that dermatosis.

[The ultrastructural changes in the nerve fibers of the skin in psoriasis]. / Ul'trastrukturnye izmeneniia nervnykh volokon kozhi pri psoriaze.

Electron microscopic examination of the upper layers of the derma in skin biopsy specimens from 20 patients with psoriasis, collected both in foci of lesions and those of apparently intact skin, has revealed that changes in amyelinic nerve fibers are more marked in foci of involvement. Here they may be partly explained by inflammatory processes; but since the nerve fibers are also significantly changed in apparently intact skin, we may suppose that these changes are due to other factors as well.

Fetal skin biopsy in prenatal diagnosis of some genodermatoses.

Various methods of obtaining fetal skin for prenatal diagnosis of certain autosomal-recessive congenital genodermatoses have been assessed. An attempt was made to obtain fetal skin by fetoscopy in 15 patients prior to pregnancy termination for a variety of medical reasons at 18-26 weeks. Specimens were obtained only in five cases (8 successful attempts out of 48). In twelve cases, of which five had a history of a child with junctional (Herlitz type) or dystrophic (Hallopeau-Siemens type) epidermolysis bullosa or non-bullous congenital ichthyosiform erythroderma at 16-25 weeks of pregnancy, fetal skin was obtained without fetoscopy under direct ultrasonic control. Specimens were obtained in all cases (33 successful attempts out of 39). In three cases, fetal pathology was diagnosed by the method of semi-thin and ultra-thin skin sections, and the respective pregnancies were terminated.

Morphology of skin microvasculature in psoriasis.

To establish the character of microvascular changes in psoriatic skin and their specificity, 29 skin biopsies of psoriatics (20 in exacerbation and 9 in a stationary stage) were investigated using histologic, histochemical, immunomorphologic, electron-microscopic, and morphometric methods. Five biopsies of uninvolved skin in scleroderma and five of diabetes mellitus patients were studied with the same technique for comparison. The results showed that structural changes depend on disease stage and the clinical appearance of lesions. Microvascular changes precede papule appearance during exacerbation and gradually increase during papule development. They comprise vascular dilatation, bridged fenestrations and gaps in endothelium, edematous areas in the cytoplasm of endotheliocytes, myocytes and pericytes, basement-membrane-zone thickening, and cell extravasation--signs of increased vascular permeability. Immunoglobulin G deposits in vascular walls, degranulation of mast cells, and extravasation of lymphocytes and neutrophils indicate that inflammation is a basic process during exacerbation and that immune mechanisms play an important role in the pathogenesis of inflammation. Microvascular changes in scleroderma and diabetes mellitus are different in nature and do not resemble those in psoriasis.

[Local PUVA therapy of patients with psoriasis]. / Lokal'naia PUVA-terapiia bol'nykh psoriazom.

Various external photosensitizers have been compared in local PUVA therapy of 280 patients with psoriasis treated in Moscow at the Central Research Institute for Skin and Sexually Transmitted Diseases, the USSR Ministry of Health, and in Warsaw at the Dermatology Hospital. In Moscow 127 patients have been administered 0.3% ammifurin solution, 73 ones 0.1% psoralen solution; in Warsaw 50 patients have been administered 0.1% puvaderm ointment (Basan) made in Finland and 30 patients 0.1% oxoralen emulsion manufactured in Austria. A good clinical effect has been achieved in 252 patients (90%). Ammifurin 0.3% solution and puvaderm 0.1% ointment have proved to be the most effective. External photosensitizers are recommended for local or as a supplement to total PUVA therapy.

[Prenatal diagnosis of several hereditary skin diseases]. / Prenatal'naia diagnostika nekotorykh nasledstvennykh zabolevanii kozhi.

Twenty-two control women and 5 women at risk for delivering a baby with Brocq's ichthyosiform erythroderma or fatal epidermolysis bullosa were investigated in order to make prenatal diagnosis of inherited fetal skin diseases. Fetal skin abnormalities were detected in 3 of the 5 high-risk patients, and their pregnancies were terminated. There was a spontaneous abortion with a normal fetus in 1 case. In one woman, pregnancy progressed to term delivery of a normal girl. Methodologic aspects of obtaining fetal skin samples and the results of their morphologic studies are discussed.

Rheological properties of the blood in psoriasis patients during PUVA-therapy.

Rheological properties of the blood in 50 psoriasis patients were studied during PUVA-therapy. The mean rheological indices in half of the patients were above normal before treatment. During the PUVA-therapy the mean indices in this group of patients were near to normal. Bimodal distributions of the obtained indices were connected neither with each other, nor with the form and severity of the disease. Lack of statistical correlation links between quantitative indices of systems defining an aggregate state of the blood leads one to believe that changes in rheological properties of the blood in psoriasis patients before treatment can be connected with the immune status of the major disease. Further research in this field is needed.