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Paraneoplastic mucous membrane pemphigoid, a rare pemphigoid variant is associated with primary malignancy, and characterised by fulminant progression and frequent ineffectivity of classical systemic immunosuppression. In this paper, the clinical features, diagnostic and therapeutical challenges are presented through three cases. Detailed history and analysis of the immunofluorescent samples help the diagnosis. The therapeutic goal is to prevent the progression with systemic immunosuppressive treatment, which can be contraindicated during the ongoing oncological therapy. In absence of consent in the exact diagnostic criteria and management protocol of this rare condition, consultation with other specialists (ophthalmologist, dermatologist, dentist, ear-nose-throat specialist, immunologist) has high importance in early diagnosis and treatment.

Összefoglaló. A glutén, alimentáris környezeti antigénként, különböző szervrendszereket érintő autoimmun betegségeket tud kiváltani. A kórképek hátterében a gluténtolerancia veleszületett hiánya vagy az élet során bekövetkező elvesztése áll. A gluténindukált autoimmun betegségek között a leggyakoribb a coeliakia, melyet különböző súlyosságú enteropátia jellemez, és melynek a szöveti, 2-es típusú transzglutamináz az autoantigénje. A coeliakia extraintestinalis tünetei között azonban néha olyan bőr- és idegrendszeri kórképek jellegzetességei is megtalálhatók, melyek hátterében további transzglutamináz-autoimmunitás kialakulása áll. Idesorolható a hevesen viszkető, polimorf autoimmun bőrbetegség, a dermatitis herpetiformis (transzglutamináz-3-autoimmunitás) és a centrális és/vagy perifériás neurológiai károsodások egy jellegzetes csoportja (transzglutamináz-6-autoimmunitás). Az indukált autoimmunitás reverzibilis, a szigorúan tartott gluténmentes diéta mellett a coeliakia és a bőrtünetek elmúlnak, de az idegrendszeri tünetek egy része maradandó. Az elmúlt évtizedben beszámoltak gluténérzékeny, transzglutamináz-6-pozitív, nem coeliakiás (transzglutamináz-2-negatív) betegekről is. A gluténszenzitivitás sokféle megjelenését ma is erősen kutatják. Fontos a korai felismerés és a kórképek interdiszciplináris szemléletű kezelése. A coeliakia családi szűrővizsgálatokkal való korai felismerése és a tünetmentes egyének diétás kezelése is nagy jelentőségű a gluténérzékenység által kiváltott hiányállapotok és a társuló egyéb betegségek kialakulásának megelőzésében. Orv Hetil. 2021; 162(28): 1107-1118. Summary. Autoimmune diseases induced by digestion of gluten, an environmental antigen, can affect different organ systems. The diseases develop in individuals with congenital or acquired loss of gluten tolerance for life. Amongst the gluten-induced autoimmune diseases, celiac disease is the most common one, characterized by an enteropathy of varying severity. Here the target autoantigen is tissue (type 2) transglutaminase. While the extraintestinal manifestations of celiac disease are complex, they may include characteristics of certain skin and nervous system disorders that develop due to additional transglutaminase autoimmunities. Such diseases are the severely pruritic, polymorphic autoimmune skin disease, dermatitis herpetiformis due to epidermal (type 3) transglutaminase autoimmunity, and a distinctive group of gluten-sensitive neuropathies with central and/or peripheral neurological involvement caused by type 6 transglutaminase autoimmunity. While the celiac and skin autoimmune diseases gradually get into remission under a strict gluten-free diet, some neurological symptoms may persist. In the last decade, gluten-induced transglutaminase 6 positive but non-celiac (transglutaminase 2 negative) patients were reported. Today, various manifestations of gluten sensitivity are under extensive research. Early detection and interdisciplinary treatment of these disorders are important. Family screenings are of particular relevance in early recognition and dietary treatment of latent disease forms in order to prevent enteropathy-induced, malabsorption-related and other associated co-morbidities. Orv Hetil. 2021; 162(28): 1107-1118.

In this review article, the authors summarize the clinical aspects of the novel classification of Ehlers-Danlos syndrome, which is a group of rare, hereditary connective tissue disorders. The leading symptom of the Ehlers-Danlos syndrome group is joint hypermobility, skin hyperextensibility and generalized tissue fragility. Ehlers-Danlos syndrome displays a high clinical and genetic heterogeneity and harbors many multidisciplinary properties. Certain subtypes only affect the quality of life, while other forms may lead to severe, even fatal vascular or intestinal complications. Last year, based on the data of various international genotype-phenotype correlation studies of large populations, a new classification of the syndrome's clinical subtypes was introduced. The novel international nosology of Ehlers-Danlos syndromes published in 2017 delineates 13 clinical subtypes, describes their genetic background and defines major and minor diagnostic criteria for each subtype. We gathered the complex, multidisciplinary symptoms of Ehlers-Danlos syndromes in a table to assist the diagnosis from a differential diagnostic point of view. In the clinical practice, the proper diagnosis of patients affected by the Ehlers-Danlos syndrome group is essential to give optimal clinical care and to prevent the development of severe complications. Orv Hetil. 2019; 160(16): 603-612.

Over the past decade a rare cell population called cancer stem cells has been identified in both solid tumors and hematologic cancers. These cells are reminiscent of somatic and embryonic stem cells and play a critical role in the initiation and progression of malignancies. As all stem cells, they are able to undergo asymmetric cell division and hence renew themselves and create various other progenies with heterogenous phenotypes. A growing body of literature suggested that stem cell subpopulations contribute significantly to the growth and metastatic properties of melanoma. This review gives a comprehensive overview of the current literature on melanoma stem cells, with a special emphasis on the signaling pathways responsible for the homeostatic growth of melanocytes and the uncontrolled proliferation of melanoma cells. The importance of the local microenvironment are demonstrated through summarizing the role of various cell types, soluble factors and cell adhesion molecules in the progression of melanoma and the creation of treatment resistant cancer cell clones. Last but not least, the models of melanoma progression will be introduced and a variety of cellular markers will be presented that may be used to identify and therapeutically target melanoma. Orv. Hetil., 2016, 157(34), 1339-1348.

Sexually transmitted infections of the urogenital tract are most commonly caused by the intracellular bacteria Chlamydia trachomatis worldwide, resulting the clinical picture of acute urethritis in men as well as urethritis and endocervicitis in women. As women often present with few symptoms only or a completely symptom-free disease course, one of the most important long-term complications is chronic pelvic inflammatory disease often followed by the development of infertility caused by chronic scar formation. Well-organized screening programs are considered to have a leading role in the prevention of disease spreading and long lasting unwanted complications. Antibiotic treatment options are often influenced by special circumstances, such as pregnancy and several complicated clinical forms. The aims of the authors are to give a concise review on the current knowledge regarding Chlamydia trachomatis infections and summarize typical clinical signs, modern diagnostic techniques as well as accepted treatment protocols and basic aspects of screening.

Coinfections of sexually transmitted infections are frequent due to the same transmission routes which may facilitate the transmission of other sexually transmitted infections. Sexually transmitted coinfections are associated with atypical and generally more severe clinical features, more complications, resistency to treatment, unfavourable outcome, and worse prognosis. Sexually transmitted infections may increase the likelihood of acquiring and transmission of HIV infection. The authors summarize the most important characteristics of sexually transmitted infections (such as HIV and hepatitis B virus, HIV and hepatitis C virus, HIV and syphilis, HIV and gonorrhoeae, HIV and chlamydia coinfections). These infections are more frequent in HIV infected patients than in the normal population. The shared transmission routes, impairment of the immune response, elevated cytokine levels and the associated inflammatory milieu produce local tissue damage, breaches in mucosal epithelium, which increases the risk of human immunodeficiency virus infection. Regular screening for sexually transmitted infections, use of more sensitive diagnostic methods, improved reporting and avoidance of unsafe sexual behaviour among certain subpopulations as well as education are essential in the prevention of sexually transmitted coinfections.

The authors report the history of a patient with syphilitic glomerulonephritis, a rare complication of syphilis. The patient was admitted to the hospital with clinical symptoms of neurosyphilis. During his hospital stay urine analysis revealed an extremely high proteinuria, that had not been known before. Intravenous penicillin treatment improved the renal protein loss, but it took a total of six months until complete resolution was achieved. The serology that confirmed the syphilis, the concomitant nephrotic syndrome and the improvement after penicillin therapy met the criteria of syphilitic glomerulonephritis. This case prompted the authors to review the literature about this rare complication of syphilis that has a great clinical significance.

Introduction: The incidence of anal cancer has increased in recent decades, particularly among human immunodeficiency virus infected men who have sex with men. Anal intraepithelial neoplasia is a potential precursor lesion of anal cancer. Anal cytology is the primary screening test for anal intraeptithelial neoplasia., Aim: The authors aimed to analyze the results of anal cytology of patients with human immunodeficiency virus infection at the National Centre of STD, Department of Dermatology, Dermatooncology and Venereology, Semmelweis University., Method: 155 anal cytological examinations were performed in 140 patients between November 1, 2012 and August 31, 2014., Results: 44% of patients were found to have anal dysplasia, and only 1.6% of patients had high-grade lesions. This rate is lower as compared to published studies including larger number of patients., Conclusions: The study underlines the necessity of screening for anal lesions in the population at-risk.

The recently observed accelerated increase of human immunodeficiency virus infection in Hungary poses a major public concern for the healthcare system. Given the effective only but not the curative therapy, prevention should be emphasized. Current statistics estimate that about 50% of the infected persons are not aware of their human immunodeficiency virus-positivity. Thus, early diagnosis of the infection by serological screening and timely recognition of the disease-associated symptoms are crucial. The authors' intention is to facilitate early infection detection with this review on human immunodeficiency virus-associated skin symptoms, and highlight the significance of human immunodeficiency virus care in the everyday medical practice.

Lymphogranuloma venereum is a sexually transmitted infection caused by the Chlamydia trachomatis serovars L1-3. It has been found to be endemic in tropical countries. In the last decades several cases have been reported in Western Europe, particularly in men who have sex with men population infected with human immunodeficiency virus. The authors present three cases of lymphogranuloma venereum infections, observed at their department in 2013 and 2014. The three human immunodeficiency virus infected patients who belonged to men who have sex with men population had casual sexual contacts in Western Europe. The symptoms included urethral discharge, discomfort and inguinal lymphadenomegaly in two patients, and rectal pain, discharge and perianal ulceration in one patient. The diagnosis was confirmed by nucleic acid amplification test performed in samples obtained from urethral discharge and exudate of perianal ulcer; lymphogranuloma venereum 2b serovars were demonstrated in two patients and serovar 2 in one patient. Doxycyclin (daily dose of two times 100 mg for 21 days) resolved the symptoms in all cases. The authors conclude that lymphogranuloma venereum is a diagnostic challenge in Hungary, too. It is important to be aware of the altered clinical features of this disease to prevent complications and spreading.

Emergence and spread of antimicrobial resistance in Neisseria gonorrhoeae is a major public health concern worldwide. The current study aims to determine the antimicrobial resistance in N. gonorrhoeae and associated molecular typing to enhance gonococcal antimicrobial surveillance in Hungary. In the National N. gonorrhoeae Reference Laboratory of Hungary 187 N. gonorrhoeae infections were detected in 2013, antibiograms were determined for all the isolated strains, and 52 (one index strain from every sexually contact related group) of them were also analysed by the N. gonorrhoeae multi-antigen sequence typing (NG-MAST) method. Twenty-two different NG-MAST sequence types (STs) were identified, of which 8 STs had not been previously described. In Hungary, the highly diversified gonococcal population displayed high resistance to penicillin, ciprofloxacin and tetracycline (the antimicrobials previously recommended for gonorrhoea treatment). Resistance to the currently recommended extended spectrum cephalosporines were rare: only two of the expected strains, an ST 1407 and an ST 210, had cefixime MIC above the resistance breakpoint. By the revision of our National Treatment Guideline, it must be considered, that the azithromycin resistance is about 60% among the four most frequently isolated STs in Hungary.

Introduction: Psoriasis is a frequent, chronic, systemic immune-mediated disease mainly affecting the skin and joints., Aim: To assess health related quality of life and cost-of-illness in moderate to severe psoriasis associated with psoriatic arthritis., Method: A cross-sectional questionnaire survey was conducted at two academic dermatology clinics in Hungary., Results: Fifty-seven patients (65% males) completed the survey with a mean age of 54.3±11.6 years and mean EQ-5D score of 0.48±0.4. Mean annual total cost was €8,977 per patient, of which 71% occurred due to biological therapy and 21% were indirect costs, respectively. Permanent work disability due to psoriasis accounted for €1,775 (95% of the indirect costs). Per patient costs of subgroups not receiving systemic therapy (21%), traditional systemic therapy (32%), and biological systemic therapy (47%) amounted to the sum of €1,729, €1,799, and €16,983, respectively., Conclusions: Patients on biological therapy showed significantly better health related quality of life. As for health economics, the efficacy of systemic treatments is appropriate to be assessed together in patients with moderate to severe psoriasis associated with psoriatic arthritis, since actual health gain might exceed that reported in psoriasis or psoriatic arthritis separately.

Introduction: Mastocytosis is a clonal mast cell proliferative disease, divided into cutaneous and systemic forms. The characteristic symptoms are caused by neoplastic mast cell infiltrations in different organs and/or the release of mediators., Aim: The aim of the authors was to summarize their clinical observations in patients with mastocytosis., Method: 22 adult patients diagnosed consecutively with mastocytosis were enrolled in the study. Skin and bone marrow biopsies were taken to establish the diagnosis and perform c-KIT mutation (D816V) analysis., Results: One of the 22 patients had teleangiectasia macularis eruptiva perstans, while 20/22 patients had urticaria pigmentosa. All patients had cutaneous lesions. In 12 patients iliac crest biopsy was performed and 9 of them had bone marrow involvement, classified as indolent systemic mastocytosis. The c-kit mutation D816V was found in one subject both in skin and bone marrow samples. The patients were treated with antihistamine, PUVA, interferon-α or imatinib., Conclusions: The authors draw attention to this rare disease in order to help recognition of relevant signs and symptoms and establish an early diagnosis.

Background: STIs like HIV and syphilis are acquired as comorbidities by high risk populations and may influence their original course and prognosis., Methods: Between January of 2005 and 2013 data of syphilis and HIV patients were collected at the Department of Dermatology of Semmelweis University, Budapest. Diagnostic procedures included clinical analysis and screening of serum samples for Treponema pallidum and HIV antibodies., Results: A total of 1,401 new syphilitic and 338 new HIV infections were diagnosed. In syphilis patients 86.58% had monoinfection,7.92% already had an HIV infection and 5.5% had acquired syphilis and HIV infection simultaneously, so 22.78% of the new HIV patients acquired the infection with syphilis together. Male gender, MSM (men who had sex with men) orientation and positive past venerological history were dominant in all groups. Most patients were diagnosed in a latent infectious stage based on the result of a serological check-up. Secondary stage and neurosyphilis were more common in coinfections., Conclusion: (i) male gender, MSM orientation, and positive venerological history are risk factors for acquiring new STIs, (ii) clinical course were different in HIV infected patients, (iii) but their timely and regular check-ups resulted in earlier diagnosis of syphilis, suggesting the necessity for frequent screening.

Introduction: Survival of patients with malignant melanoma primarily depends on tumor stage. Hungarian National Cancer Registry does not specify tumors according to TNM stages., Aim: The authors aimed to survey the stage distribution of melanomas at the Department of Dermatology, Dermatooncology and Venerology, Semmelweis University., Method: 1160 patients (558 males and 602 females, aged 60.5±16 and 57±17 years, respectively) diagnosed with cutaneous melanoma between 2004-2009 were included., Results: In comparison with international studies, the case distribution was favorable in stages IA and IV, i.e. the proportion of early melanomas was relatively high (IA: 43.8%), while the incidence in stage IV was low (0.4%). In stages IB-IIA the incidence was significantly lower, while in IIC, IIIA, IIIB it was higher as compared to published data from Western-Europe, Australia and the United States., Conclusions: The study underlines the necessity of prevention and awareness campaigns that may result in increase of early diagnosis of melanomas.

Gonococcal conjunctivitis is a rare infection induced by Neisseria gonorrhoeae and it usually manifests as a hyperacute purulent conjunctivitis. Ocular access of the infectious secretion during sexual intercourse is the way of transmission among adults. Inclusion conjunctivitis caused by the serovars D-K of Chlamydia trachomatis also affects the sexually active population. Authors present a case of a 33-year-old homosexual man who was treated for late latent syphilis formerly. Clinical symptoms were yellow purulent discharge for 3 weeks without any urological or upper respiratory tract symptoms. Conjunctival Neisseria gonorrhoeae and Chlamydia trachomatis infection was identified using cultures and polymerase chain reaction; pharyngeal swab culture and polymerase chain reaction showed positive results for both pathogens. The patient was probably under influence of party drugs at the time of sexual abuse when he became infected. After parenteral and oral cephalosporin and azithromycin therapy the patient had complete recovery within three weeks.

Infliximab is a TNFα inhibiting recombinant monoclonal antibody, which provides an efficient therapeutic opportunity in the treatment of psoriasis and other immune-mediated inflammatory diseases. It is well tolerated and improves quality of life significantly. The authors present a case of drug-induced lupus erythematosus as a possible side effect of this medication. The patient developed psoriasis 9 years ago when she was on beta-receptor blocker therapy. The symptoms deteriorated despite topical and systemic treatments and, therefore, biological therapy was introduced. In the third year of treatment drug-induced lupus erythematosus was diagnosed on the background of general symptoms. After cessation of the biologic treatment a low dose corticosteroid therapy was introduced which proved to be effective. Symptoms as well as pathological laboratory parameters showed an improvement. The authors conclude that biologicals are effective and safe in the treatment of psoriasis, nevertheless, they have risks too. To reduce side effects a meticulous follow-up of patients is essential. Any general symptom requires careful examination since they might be linked to serious side effects of the biological therapy.

From January 1, 2009 through December 31, 2011, from 33,753 blood samples for syphilis screening, Treponema pallidum infections were confirmed in 241 pregnant women at the Department of Dermatology, Venerology, and Dermatooncology of Semmelweis University Budapest. In this period, four children born to inadequately or untreated women were confirmed to have connatal syphilis. The height of rapid plasma reagin (RPR) titer was measured to determine the stage of the infection and to examine the success of the antilues therapy. The diagnosis of maternal syphilis infection was confirmed with enzyme linked immunosorbent assay (ELISA), T. pallidum particle agglutination (TPPA), and IgG and IgM immunoblots. Maternal IgM immunoblot results identify mothers at risk of delivering babies with connatal syphilis better than the height of maternal RPR titer. The standard serological tests are less useful in newborns because of IgG transfer across the placenta. IgM test which depends on the infant's response has more specificity in diagnosing connatal syphilis.

Unlabelled: Ureaplasma urealyticum and Mycoplasma hominis have important role among the causative agents of sexually transmitted diseases., Aim: The aim of the study was to determine the frequency and antibiotic resistance of Ureaplasma urealyticum and Mycoplasma hominis in genital samples obtained from patients examined in the Sexually Transmitted Diseases Centre of the Department of Dermatology, Venerology and Dermatooncology, Semmelweis University, Budapest between May 1, 2008 and July 31, 2010., Patients and Methods: Samples were taken from the urethra in men and from the cervix and urethra in women by universal swab (Biolab®) into Urea-Myco DUO kit (Bio-Rad®) and were incubated for 48 hours at 37 C°. Antibiotic sensitivity of positive samples was determined in U9 bouillon using SIR Mycoplasma kit (Bio-Rad®)., Results: Samples for 4154 patients aged 16-60 years were examined. In 247/4154 samples (6%) U. urealyticum and in 26/4154 samples (0.63%) M. hominis was isolated from the genital tract. Most U. urealyticum and M. hominis strains (75% and 77%, respectively) were cultured from cervix, while the remaining 25%, and 23% from the male and female urethra, respectively. U. urealyticum and M. hominis were most commonly detected in patients aged between 21 and 40 years. The majority of U. urealyticum strains were sensitive to tetracycline (94%), doxycycline (95%), azithromycin (88%) and josamycin (90%), but were resistant to ofloxacin (21%), erythromycin (85%) and clindamycin (79%). Seventy-seven percent of the U. urealyticum strains were simultaneously resistant to erythromycin and clindamycin, suggesting that ex iuvantibus therapies may select cross-resistant strains to both antibiotics. The resistance of M. hominis to clindamycin, doxycycline, ofloxacin and tetracycline varied between 4% and 12 %., Conclusions: Because none of the strains was sensitive to all examined antibiotics, the antibiotic sensitivity of U. urealyticum and M. hominis strains should be determined. The high rate of ofloxacin, erythromycin and clindamycin resistance should be considered in the therapy of U. urealyticum infections in Hungary. This is the first such a clinical microbiological study in this topic in Hungary.

Authors report a case of a 35-year-old male with right-sided mild paresis, incontinence, dysexecutive syndrome, short-term memory loss and behavioral changes. Bilateral cerebral infarcts in the region of the caudate nuclei and the adjacent white matter were proved by brain MRI and multiple stenoses of the branches of Willis-circle were confirmed by MR angiography. Elevated protein level and pleocytosis were found in the cerebrospinal fluid with intrathecal IgG synthesis. Serum rapid plasma reagin, Treponema pallidum Particle Agglutination test, Treponema pallidum ELISA, liquor Venereal Disease Research Laboratory tests were positive. Meningovascular neurosyphilis was diagnosed. 24M U/day intravenous penicillin-G treatment was given for 14 days. The patient has vascular dementia due to the bilateral strategic infarcts disconnecting the prefrontal circuits; his symptoms are similar to general paresis. Laboratory and radiologic improvement was observed. Still, the patient have severe residual cognitive decline.

Fabry disease is a rare, X-linked lysosomal storage disorder that leads to accumulation of globotriaosylceramide in different tissues of the body. The disease is progressive and the first symptoms usually present in childhood. Consequences of the disease are disability and premature death. The disease in females could be as severe as in males although women may be asymptomatic. The possibility of enzyme replacement therapy has made it necessary to elaborate a comprehensive guideline for the diagnosis and treatment follow-up. The guideline has been summarized by a Hungarian multi-disciplinary working group consisting of physicians who are involved in diagnosis and care of Fabry patients. Previous clinical studies, published articles, and recently established international treatment guidelines were reviewed by the group.

Rosacea is one of the most common chronic dermatological diseases. It is characterized by transient or persistent facial erythema, teleangiectasias, papules and pustules, usually on the central portion of the face. Rosacea can be classified into four main subtypes: erythemato-teleangiectatic, papulopustular, phymatous, and ocular. These subtypes require different therapeutic approaches. Regarding to the pathomechanism, several hypotheses have been documented in the literature, including genetic and environmental factors, vascular abnormalities, dermal matrix degeneration, microorganisms such as Demodex folliculorum and Helicobacter pylori, but the cause of rosacea is still not known. Authors in this article review current literature on new classification system of rosacea, as well as the main pathogenetic theories and current therapeutic options.

Fabry disease is a rare, X-linked lysosomal storage disorder that leads to accumulation of globotriaosylceramide in different tissues of the body. The disease is progressive, first symptoms usually present in childhood. Consequencies of the diseases are disability and premature death. The disease in females could be as severe as in males although women may also be asymptomatic. The possibility of enzyme replacement therapy has made it necessary to elaborate a comprehensive guideline for the diagnosis and treatment follow-up. The guideline was established by a Hungarian multi-disciplinary working group, consisting of physicians who are involved in health care of Fabry patients. Previous clinical studies, published materials, and recently established international treatment guidelines were reviewed by the group.

The STD Department of Semmelweis University Budapest is the National Centre of Hungary, which is responsible for screening and care of sexually transmitted diseases (STD), including syphilis and gonorrhoea. 42,114 patients attended the STD Department and 25,362 anonymous screening (HIV: 12,337, syphilis: 13,025) were done between January 2005 and December 2008. During this period 600 syphilitic and 339 gonorrhoea infections were diagnosed. The obligatory HIV screening of patients with sexually transmitted infections (STI) resulted positive result in 47 cases, and 63 patients infected with HIV acquired new syphilitic or gonorrhoea infection. Contact tracing was successful in around 400 syphilis cases, and 150-200 gonorrhoea cases per year. We present our statistical data in order to call attention to the resurgence of syphilis and gonorrhoea and the importance of STD co-infections.

The 54 year-old vegetarian patient has taken oral colloidal silver for two years to stimulate his immune system. The silver intake resulted in diffuse grayish discoloration on the face. His laboratory values were in the normal range, and no internal organ disease was detected. The histology of the forehead skin confirmed the diagnosis of argyria. Argyria is a condition associated with chronic local or systemic exposure to silver-containing products. The silver is typically deposited in skin, fingernails, oral mucosa and conjunctival membranes. There is no effective treatment for this condition. With the availability of pharmacologic alternatives, physician-directed use of silver-containing products had significantly declined. We review the literature and call attention to the adverse effects and dangers of the widely used paramedicinal colloidal silver products.

Recognizing the adverse drug reactions and confirming the role of the drug causing the symptoms is a great challenge for a medical team. The aim of this article is to review the diagnostic methods for confirming drug-allergy and to evaluate the lymphocyte transformation test according to dermatological aspect. Lymphocyte transformation test relies on the observation that specific T-cells divide and expand after encountering the antigen. T-cell sensitization is measured by 3H-thymidine uptake in dividing cells. The main advantage of this test is its applicability with many different drugs in different immune reactions, as drug-specific T-cells are almost always involved in drug hypersensitivity reactions. Its disadvantage is that the test is cumbersome and technically demanding. The method is not unequivocally accepted, it is necessary to carry out a systematic evaluation of its sensitivity and specificity to make the test more widely appreciated. Despite of its deficits, the lymphocyte transformation test plays an important role in diagnosing drug-hypersensitivities.

Neonatal lupus erythematosus (NLE) is a disease of the first few months of infancy. It is caused by anti-SSA and anti-SSB antibodies, which are products of maternal autoimmune disorders (SLE, Sjögren, rheumatoid arthritis) and can be passively transported across the placenta. The prevalence of NLE is low. The major clinical findings are cutaneous (typical annular erythematous plaques), cardiac, hepatic and hematologic alterations. Its most severe consequence is third-degree heart block, which is irreversible, requires pacemaker-implantation and responsible for the 20-30% mortality rate. Symptoms usually resolve spontaneously at age of 6-9 months in association with disappearance of maternal antibodies from the infant's serum. In our case the typical cutaneous manifestations covered virtually the whole body, were present at birth, however, no conduction defects developed. The fact that the mother's sickness was not known at birth made it difficult to establish the diagnosis. The significant thrombocytopaenia, progressive skin-changes and the elevated liver function tests necessitated systemic steroid treatment.

Paraneoplastic pemphigus is an autoimmune bullous skin disease induced by underlying malignant or benign neoplasias. The diagnostic and immunological criteria of the disease were characterized by Anhalt et al. in 1990. Clinical symptoms are variable, consisting of polymorphous blistering skin eruption and severe, painful mucocutaneous ulcerations. In a subset of patients, only papular lesions develop, resembling lichen planus, or graft-versus-host disease; in some cases blisters may develop later. Severe dyspnea, progressive respiratory failure with clinical features of bronchiolitis obliterans is a rather frequent and severe complication. The diagnosis can be established with direct and indirect immunofluorescent studies and immunoblot analysis. The autoantigens identified to date include cytoplasmic proteins of the plakin gene family: envoplakin (210 kD), periplakin (190 kD), plectin (approximately 500 kD), desmoplakin I (250 kD), desmoplakin II (210 kD) and bullous pemphigoid antigen 1 (230 kD). The desmosomal cadherins: desmogleins 1 and 3, and desmocollins 2 and 3, as well as bullous pemphigoid antigen 2 (180 kD) and an undetermined 170-kD transmembranous antigen are also target autoantigens in the disease. The mortality rate is more than 90 percent. Beside treatment of the underlying tumor, a combination of systemic steroids with immunomodulators, cytostatic drugs, plasmapheresis, plasma exchange, intravenous gammaglobulin, or anti-CD20 monoclonal antibody (rituximab) may be the most appropriate treatment.

Unlabelled: A rare case, when radical vulvectomy had to be done to treat a benign skin disorder is presented., Patient and Method: A 56-year-old white woman suffered from severe vulvar acne inversa. The systemic treatments, the incisions and drainages were not successful. The only solution was the radical excision of the seriously damaged vulva, with a satisfactory cosmetic and functional result. The pathology, the diagnosis and the treatment of the disease are also discussed., Conclusion: The authors put emphasis on the importance of the interdisciplinary collaboration.

The prevalence of atopic diseases, including allergic rhinitis, asthma bronchiale and atopic dermatitis is increasing both in children and adults at different parts of the world. Atopic dermatitis is a chronic inflammatory skin disease affecting mostly children, but the atopic trait continues, not only for later respiratory allergies, but also for skin symptoms in adulthood. In this form dry skin, flexural lichenification, head and neck dermatitis, hand dermatitis are typical. The exact etiology of atopic dermatitis is unknown, in the background interactions of genetical predisposition, skin barrier defects and immunological and environmental factors can be verified. In the complex approach of atopic dermatitis, a pivotal role is ascribed to the evaluation and possibly the elimination of provoking factors, like gender, family structure, clothing, aero-, alimentary and contact allergens, psychosocial stress, migration, infections, and personal home environment. Authors review clinical manifestations, triggering and prognostic factors of the adulthood atopic dermatitis.

The authors' report a case of a 26-year-old female. In 1996, and 1997 she underwent cosmetic lip augmentations (polymethyl methacrylate and silicone). After a six-year symptom free period sudden swelling of the lips developed and red-brown papules and subcutaneous nodules appeared in the scars of various body parts ( permanent make-up, tattoos, umbilical piercing). The clinical features, radiological signs and histology proved the diagnosis of systemic sarcoidosis. Systemic corticosteroid treatment was introduced. After 4 months of treatment the pulmonary and clinical symptoms showed regression. To be able to suspend the systemic treatment the silicone implant was removed.

The authors discuss the case of a patient suffering from an auditory-pigmentary syndrome. The patient in whom the Waardenburg syndrome had never been diagnosed before was observed because of another disease, which quite frequently causes hearing loss in childhood. In a short review of the literature, the authors describe the other components of the syndrome and the recent information about the genetic background of the disease.

Introduction and Aim: Human herpesvirus 7 in pityriasis rosea, this and other viruses in papular-purpuric gloves-and-socks syndrome have been implicated, but their primary or recurrent infections are still in question., Patient and Methods: In one available blood sample, therefore, IgM, IgG and its high avidity fraction characteristic for recurrent infections were quantitated by indirect immunofluorescence. Peripheral lymphocytes were subjected to nested polymerase chain reaction to detect viral DNA, or cocultivated with several cell cultures., Results: One third of 33 pityriasis rosea patients had elevated IgM, another third had elevated IgG without high avidity molecules to human herpesvirus 7 suggesting primary infection. Thirty percent of controls, more than half of the patients had virtual DNA in their lymphocytes, but only one in 5 skin biopsy specimens were PCR positive. All three co-cultivation attempts yielded viruses extremely rapidly, verified by electron microscopy, polymerase chain reaction and monoclonal antibodies as human herpesvirus 7. These are the first isolates in the geographical regions of Hungary. These data suggest that pityriasis rosea is the consequence of a primary human herpesvirus 7 infection in seronegative adults, and only occasionally is due to virus reactivation. One patient with gloves-and-socks syndrome had an acute, another patient had a persistent coinfection with human herpesvirus 7 and parvovirus B19, two others had a primary herpesvirus 7 infection. Interestingly, this disease might be elicited by both viruses individually or in synergism., Conclusion: Neither human herpesvirus 7 nor parvovirus B19 infect skin cells, but both can be detected in the infiltrating lymphocytes of skin eruptions, in which they induce an altered mediator production, that might be responsible for the general and local symptoms.

Authors present 5 cases of generalized contact and photocontact dermatitis due to topically applied ketoprofen, a non-steroidal anti-inflammatory drug. They investigated the sensitisation and photosensitisation to the drug and also the possible cross-reactivity.

Authors summarise the two-decade experience of their department in severe tissue necrosis of penis and scrotum. Presented cases represent a shared field of urology with dermatology, infectology, nephrology and andrology. Tissue necrosis extended to different depth of the penis, from superficial skin and subcutaneous tissues, fascias to the entire organ. Underlying diseases are acute and chronic inflammations, metabolic diseases, vascular lesions. Diagnostic difficulties and therapeutic choices, both conservative and operative, are discussed.

Authors report a prenatal diagnosis in a family of Herlitz junctional epidermolysis bullosa with risk of recurrence. The diagnosis in the family was established in the case of the affected older brother using electron microscopy and immunofluorescence microscopy. The underlying mutation was also detected in the gene LAMB3. The affected older brother was homozygous, the parents and one of the siblings proved to be heterozygous carriers for the mutation and the other sibling was genotypically normal. The chorionic villous sample biopsy was carried out in the 11, week of pregnancy in order to extract fetal desoxyribonucleic acid. The previously detected mutation generates new restriction enzyme site, which was tested after the polymerase chain reaction amplification of the exon. Desoxyribonucleic acid samples of the family members and an unaffected unrelated control person were used as controls. The fetus proved to be genotypically normal.

A study of 16 patients with chronic bullous dermatosis of childhood (CBDC) is reported. They were followed up for 0.5 to 4.5 years. Histological and immunofluorescent (IF) study of the skin and local provocation of skin lesions by Trafuril test were carried out in every case, intestinal biopsy in twelve cases. Four patients were found to have IgG, and three IgA, along the skin basement membrane. In nine patients, no immunoglobulins were detectable; these IF negative cases seemed to be pseudonegative. Repeated biopsy and immunoelectronmicroscopic study of the skin may be helpful. In the three patient groups classified on the basis of skin IF studies, there was no difference in the clinical and histological picture, in reaction to Trafuril test and in the result of treatment. The small intestinal mucosa was abnormal in three of twelve patients. A gluten-free diet introduced in two patients had no effect on the course of the disease. CBDC with negative or positive IF, juvenile pemphigoid and IgA linear dermatosis seem to be a common clinical entity, different in severity and with individual variations. The condition is not identical with dermatitis herpetiformis Duhring.