Knowledge and Influence of Predatory Journals in Dermatology: A Pan-Austrian Survey.

The aim of this study was to assess the knowledge and influence of predatory journals in the field of dermatology in Austria. A total of 286 physicians (50.5% men) completed a questionnaire. The vast majority of subjects read scientific articles (n = 281, 98.3%) and took them into consideration in their clinical decision-making (n = 271, 98.5% of participants that regularly read scientific literature). Open access was known by 161 (56.3%), predatory journals by 84 (29.4%), and the Beall's list by 19 physicians (6.7%). A total of 117 participants (40.9%) had been challenged by patients with results from the scientific literature, including 9 predatory papers. Participants who knew of predatory journals had a higher level of education as well as scientific experience, and were more familiar with the open-access system (p < 0.001). These results indicate that the majority of dermatologists are not familiar with predatory journals. This is particularly the case for physicians in training and in the early stages of their career.

Characterization of the early local immune response to Ixodes ricinus tick bites in human skin.

Little is known about the immunomodulation by tick saliva during a natural tick bite in human skin, the site of the tick-host interaction. We examined the expression of chemokines, cytokines and leucocyte markers on the mRNA levels and histopathologic changes in human skin biopsies of tick bites (n=37) compared to unaffected skin (n=9). Early tick-bite skin lesions (<24 hours of tick attachment) were characterized by a predominance of macrophages and dendritic cells, elevated mRNA levels of macrophage chemoattractants (CCL2, CCL3, CCL4) and neutrophil chemoattractants (CXCL1, CXCL8), of the pro-inflammatory cytokine, IL-1ß, and the anti-inflammatory cytokine, IL-5. In contrast, the numbers of lymphocytes and mRNA levels of lymphocyte cell markers (CD4, CD8, CD19), lymphocyte chemoattractants (CXCL9, CXCL10, CXCL11, CXCL13, CCL1, CCL22), dendritic cell chemoattractants (CCL20), and other pro- (IL-6, IL-12p40, IFN-γ, TNF-α) and anti-inflammatory cytokines (IL-4, IL-10, TGF-ß) did not differ from normal skin. With longer tick attachment (>24 hours), the numbers of innate immune cells and mediators (not significantly) declined, whereas the numbers of lymphocytes (not significantly) increased. Natural tick bites by Ixodes ricinus ticks initially elicit a strong local innate immune response in human skin. Beyond 24 hours of tick attachment, this response usually becomes less, perhaps because of immunomodulation by tick saliva.

Clinical spectrum of skin manifestations of Lyme borreliosis in 204 children in Austria.

The spectrum of skin manifestations of Lyme borreliosis in children is not well characterized. We conducted a retrospective study to analyze the clinical characteristics, seroreactivity to Borrelia burgdorferi sensu lato, and outcome after treatment in 204 children with skin manifestations of Lyme borreliosis seen in 1996-2011. Solitary erythema migrans was the most common manifestation (44.6%), followed by erythema migrans with multiple lesions (27%), borrelial lymphocytoma (21.6%), and acrodermatitis chronica atrophicans (0.9%). A collision lesion of a primary borrelial lymphocytoma and a surrounding secondary erythema migrans was diagnosed in 5.9% of children. Rate of seroreactivity to B. burgdorferi s.l. was lower in solitary erythema migrans compared to other diagnosis groups. Amoxicillin or phenoxymethylpenicillin led to complete resolution of erythema migrans within a median of 6 (solitary) and 14 days (multiple lesions), respectively, and of borrelia lymphocytoma within a median of 56 days. In conclusion, erythema migrans with multiple lesions and borrelial lymphocytoma appear to be more frequent in children than in adults, whereas acrodermatitis chronica atrophicans is a rarity in childhood. The outcome after antibiotic therapy was excellent in children, and appears to be better than in adults.

Erysipelas of the thigh and the gluteal region: retrospective multicenter analysis of a very rare entity in 39 patients.

BACKGROUND: Erysipelas of the thigh and the gluteal region are rarely described and not well characterized. Therefore we aim to describe the prevalence, clinical characteristics, and risk factors of these erysipelas types. METHODS: The files of 1,423 patients with erysipelas were analyzed. Data from patients with erysipelas of the thigh or the gluteal region were compared between the two groups and with a control group with erysipelas of the lower leg. RESULTS: The thigh was exclusively affected in 2.1%, and the gluteal region in 0.6% of erysipelas patients. Gluteal erysipelas had conspicuous irregular borders and sometimes appeared bilaterally. Major risk factors for erysipelas of both sites were previous surgical interventions. Gluteal erysipelas was common in patients with the metabolic syndrome and required a more intense antibiotic therapy. CONCLUSION: Erysipelas of the thigh and the gluteal region are rare and significantly associated with prior surgical disruption of lymphatic vessels.

Bullous papular-purpuric gloves and socks syndrome in a 42-year-old female: molecular detection of parvovirus B19 DNA in lesional skin.

Papular-purpuric gloves and socks syndrome is a self-limited febrile illness of children and young adults. Only 50 well documented cases have been reported, most of which were associated with parvovirus B19 infection. Molecular detection of the virus from lesional skin has been described in only 5 patients. The syndrome is characterized by a papular-purpuric edematous rash in a distinct "gloves and socks" distribution. Extracutaneous manifestations are usually mild and transient. We report a 42-year-old female with a highly unusual expression of the syndrome, including bullous lesions, lingual aphthae, and conjunctivitis, accompanied by arthritis and a high-grade fever. She had immunoglobulin M and immunoglobulin G antibodies to parvovirus B19, which was detected from lesional skin with the use of polymerase chain reaction for the first time in such a clinical constellation. Because parvovirus B19 infections are generally more severe in adults, we suspect that the unusual disease expression in our patient was related to being more than 40 years of age.

Carbamazepine as the only effective treatment in a 52-year-old man with trigeminal trophic syndrome.

Trigeminal trophic syndrome is a rare condition resulting from self-manipulation of the skin after a peripheral or central injury to the trigeminal system. The syndrome consists of a classic triad of anesthesia, paresthesias, and secondary persistent or recurrent facial ulcerations. The most common causes include destruction of the trigeminal ganglion, rhizotomy, and stroke. We describe a patient who developed the syndrome as a sequel to brainstem infarction and trigeminal neuropathy. Whereas a-lipoic acid and gabapentin were ineffective, a remarkable benefit was achieved by administering carbamazepine (200 mg 3 times a day), which influences both neuropathic and behavioral factors in this rare syndrome. Our experience with the presented case, together with the scarce information in the literature, indicates that carbamazepine should be the first treatment option for trigeminal trophic syndrome.

Skin manifestations of lyme borreliosis: diagnosis and management.

Lyme borreliosis is a multisystem infectious disease caused by tick-transmitted spirochetes of the Borrelia burgdorferi sensu lato complex. The three characteristic cutaneous manifestations are erythema migrans, borrelial lymphocytoma, and acrodermatitis chronica atrophicans. Erythema migrans occurs in acute Lyme borreliosis, lymphocytoma is a subacute lesion, and acrodermatitis is the typical manifestation of late Lyme borreliosis. Clinical appearances of erythema migrans and lymphocytoma (when located on the ear or breast) are characteristic, whereas acrodermatitis is often confused with vascular conditions. The diagnosis of erythema migrans is made clinically. Serologic analyses often yield false-negative results and are not required for the diagnosis. However, serologic proof of the diagnosis in lymphocytoma (approximately 90% positive) and acrodermatitis (100% positive) is mandatory. Histopathologic examination often adds substantial information in patients with skin manifestations of Lyme borreliosis and is recommended in clinically (and serologically) undecided cases of erythema migrans or lymphocytoma and is obligatory in acrodermatitis. Polymerase chain reaction for Borrelia-specific DNA (rather than culture of the spirochete) and immunohistochemical investigations (lymphocytoma) are sometimes necessary adjuncts for the diagnosis. Antibacterial treatment is necessary in all patients to eliminate the spirochete, cure current disease, and prevent late sequelae. Oral doxycycline, also effective against coinfection with Anaplasma phagocytophilum, is the mainstay of therapy of cutaneous manifestations of Lyme borreliosis. Other first-line antibacterials are amoxicillin and cefuroxime axetil. Erythema migrans is treated for 2 weeks, lymphocytoma for 3-4 weeks, and acrodermatitis for at least 4 weeks.

Immunoblot analysis of the seroreactivity to recombinant Borrelia burgdorferi sensu lato antigens, including VlsE, in the long-term course of treated patients with erythema migrans.

OBJECTIVE: We evaluated whether immunoblotting is capable of substantiating the posttreatment clinical assessment of patients with erythema migrans (EM), the hallmark of early Lyme borreliosis. METHODS: In 50 patients, seroreactivity to different antigens of Borrelia burgdorferi sensu lato was analyzed by a recombinant immunoblot test (IB) in consecutive serum samples from a minimum follow-up period of 1 year. Antigens in the IgG test were decorin-binding protein A, internal fragment of p41 (p41i), outer surface protein C (OspC), p39, variable major protein-like sequence expressed (VlsE), p58 and p100; those in the IgM test were p41i, OspC and p39. Immune responses were correlated with clinical and treatment-related parameters. RESULTS: Positive IB results were found in 50% before, in 57% directly after therapy and in 44% by the end of the follow-up for the IgG class, and in 36, 43 and 12% for the IgM class. In acute and convalescence phase sera, VlsE was most immunogenic on IgG testing (60 and 70%), and p41i (46 and 57%) and OspC (40 and 57%) for the IgM class. By the end of the follow-up, only the anti-p41i IgM response was significantly decreased to 24%. CONCLUSIONS: No correlation was found between IB results and treatment-related parameters. Thus, immunoblotting does not add to the clinical assessment of EM patients after treatment.

The importance of serum bile acid level analysis and treatment with ursodeoxycholic acid in intrahepatic cholestasis of pregnancy: a case series from central Europe.

BACKGROUND: Intrahepatic cholestasis of pregnancy (ICP) is a severely pruritic form of reversible cholestasis that is associated with significant fetal risks. Because precise diagnostic and therapeutic guidelines are lacking, we performed a retrospective investigation of dermatologic and biochemical features, treatment, and neonatal outcome in patients with ICP seen from 2000 through 2005 at a university-based dermatologic hospital in central Europe. OBSERVATIONS: The 13 observed cases of ICP (11 patients) represented 6% of all pregnancy-associated dermatoses at our department. Intrahepatic cholestasis of pregnancy started at a mean+/-SD of 30+/-4 weeks' gestation, with pruritus as the leading symptom, followed by secondary skin lesions in 11 cases (85%). Total serum bile acid levels were markedly elevated in all patients and correlated with impaired fetal prognosis. Only 10 cases (77%) had other liver function test result abnormalities. Fetal distress occurred in 3 pregnancies (23%). In the 10 cases treated with ursodeoxycholic acid, 3 (30%) involved preterm deliveries compared with a 100% preterm delivery rate in the cases not treated with ursodeoxycholic acid. CONCLUSIONS: Severe pruritus with or without skin changes in the second half of pregnancy should alert the physician to the possibility of ICP. Elevated total serum bile acid levels are the clue to diagnosis, which should be established as early as possible. Close obstetric surveillance and prompt treatment with ursodeoxycholic acid are warranted.

Clinical relevance of different IgG and IgM serum antibody responses to Borrelia burgdorferi after antibiotic therapy for erythema migrans: long-term follow-up study of 113 patients.

OBJECTIVES: To investigate the kinetics of anti-Borrelia burgdorferi antibodies for a minimum of 1 year after antibiotic therapy in patients with erythema migrans (EM) and to correlate antibody titer kinetics with clinical variables. DESIGN: Retrospective study of serial anti-B burgdorferi antibodies in correlation to clinical variables. SETTING: University-based hospital. PATIENTS: One hundred thirteen patients with EM. INTERVENTIONS: Pretreatment and a median of 4 consecutive posttreatment serum samples from median follow-up of more than 400 days were simultaneously investigated for anti-B burgdorferi IgG and IgM antibodies. Semiquantitative titers were plotted to identify different groups of antibody kinetics. Individual patients were then stratified to those groups according to their antibody development. A statistical comparison of clinical and therapy-related characteristics among the serologic groups was performed. RESULTS: Anti-B burgdorferi IgG and IgM antibody titers developed in 3 distinct courses: persistent positivity across follow-up (IgG: 12 patients, 11%; IgM: 14, 12%), persistent negativity (IgG: 63, 56%; IgM: 47, 42%), and decrease of a positive pretreatment titer to a negative titer approximately 5 months after therapy (IgG: 34, 30%; IgM: 49, 43%). Statistics revealed significant correlations only between persistent positive IgG titers and long disease duration or large EM lesions before therapy. CONCLUSIONS: Long duration or large size of EM before therapy correlates with persistence of a positive anti- B burgdorferi IgG antibody titer after therapy. Serologic profiles do not depend on the type or duration of therapy or the clinical course thereafter. Thus, antibody testing in the follow-up of patients with EM is inappropriate for the assessment of therapeutic response.

The specific dermatoses of pregnancy revisited and reclassified: results of a retrospective two-center study on 505 pregnant patients.

OBJECTIVES: We sought to evaluate the frequency and clinical characteristics of pruritic dermatoses in pregnancy and to assess a rationalized classification. METHODS: Data of 505 pregnant patients seen at two university-based dermatologic hospitals (1994-2004) were retrospectively studied. RESULTS: Diagnoses included eczema in pregnancy (49.7%), polymorphic eruption of pregnancy (PEP) (21.6%), pemphigoid gestationis (PG) (4.2%), intrahepatic cholestasis of pregnancy (ICP) (3%), prurigo of pregnancy (0.8%), pruritic folliculitis of pregnancy (0.2%), and miscellaneous dermatoses (20.6%). Eczema in pregnancy, prurigo of pregnancy, and pruritic folliculitis of pregnancy showed considerable overlap and were summarized as atopic eruption of pregnancy (AEP). While PEP, PG, and ICP presented in late pregnancy, AEP started significantly earlier. Primigravidae and multiple gestations were characteristic for PEP, abdominal involvement for PEP and PG, and a history of affected pregnancies for ICP. LIMITATIONS: This was a retrospective study. CONCLUSION: We propose classifying the dermatoses of pregnancy as PG, PEP, AEP, and ICP. Stereotypic immunofluorescence and laboratory findings are diagnostic of PG and ICP, whereas distinct clinical characteristics facilitate discrimination between PEP and AEP.

Skin infections in pregnancy.

A wide array of infectious diseases can occur in pregnancy. Their acquisition, clinical presentation, and course during gestation may be altered due to an impairment of the maternal cellular immunity. Some infectious diseases can lead to serious consequences for the mother or the offspring, including congenital malformations. This review describes in detail the clinical presentation, course, management, and associated maternal and fetal risks of selected viral (varicella-zoster virus infections, condylomata acuminata), fungal (candida vulvovaginitis), bacterial (Lyme borreliosis), and parasitic (scabies) infections. The treatment options are critically reviewed. First-line therapies include acyclovir and varicella-zoster virus immunoglobulin for varicella-zoster virus infections, surgical modalities for genital warts, topical clotrimazole and oral fluconazole for Candida vulvovaginitis, amoxicillin and cefuroxime for Lyme borreliosis, and permethrin for scabies. A synopsis of maternal and fetal risks of other important infections is also included.

Soft tissue cervicofacial emphysema after dental treatment: report of 2 cases with emphasis on the differential diagnosis of angioedema.

BACKGROUND: The development of soft tissue cervicofacial emphysema after dental treatment is a rare complication, with few descriptions in the dermatologic literature. It is usually restricted to only moderate local swelling. However, spread of larger amounts of air into deeper spaces may sometimes cause serious complications, including airway compromise due to accumulation of air in the retropharyngeal space, pneumomediastinum, and pneumopericardium. Fatal air embolism and soft tissue infections through dissemination of oral flora microorganisms along the emphysematous tracts have also been described. Therefore, early recognition is important, but the unfamiliarity of dermatologists with this condition often causes diagnostic problems. Important differential diagnoses include angioedema, soft tissue infections, and hematoma. OBSERVATIONS: We describe 2 patients with different severity of the emphysema and airway compromise, representing the wide spectrum of its clinical expression. Our first case was remarkable, because the emphysema was massive and extended far into deep spaces, including the orbita, mediastinum, and pleural cavity. The present case is only the third report of pneumothorax associated with dental treatment published to date. The patient's condition was initially misdiagnosed and treated as angioedema. CONCLUSIONS: Dermatologists should be aware that soft tissue emphysema can cause acute swelling of the cervicofacial region after dental procedures. Angioedema is an important differential diagnosis, because it may be caused by the use of nonsteroidal anti-inflammatory drugs or local anesthetics, which are often administered during dental treatments.

Acrodermatitis chronica atrophicans in a 15-year-old girl misdiagnosed as venous insufficiency for 6 years.

Acrodermatitis chronica atrophicans, the characteristic cutaneous manifestation of late Lyme borreliosis, typically occurs in elderly women. To our knowledge, only 4 cases of acrodermatitis chronica atrophicans in children have been described. Prompt diagnosis and treatment are important to prevent progression of disease and extracutaneous complications. We describe a 15-year-old girl with acrodermatitis chronica atrophicans of the left leg that had been misdiagnosed as chronic venous insufficiency for 6 years. Because of the long-standing disease course, skin changes expanded and progressed to marked atrophy. The correct diagnosis was finally based on clinical, histopathologic, and serologic findings. The girl was treated with oral doxycycline for 6 weeks, but her skin changes did not fully normalize. This case illustrates the possibility of acrodermatitis chronica atrophicans appearing in childhood and the difficulties in differentiating vascular disorders from acrodermatitis chronica atrophicans on the basis of the clinical appearance alone.

Dermatological manifestations of Lyme borreliosis.

Lyme borreliosis is a multisystem infectious disease caused by the tick-transmitted spirochete Borrelia burgdorferi sensu lato. About 80% of all Lyme borreliosis cases represent skin manifestations (dermatoborrelioses). The three characteristic dermatoborrelioses are erythema migrans, borrelial lymphocytoma, and acrodermatitis chronica atrophicans, which occur in different stages of the disease. Erythema migrans is the hallmark of early Lyme borreliosis, whereas acrodermatitis chronica atrophicans is the characteristic manifestation of late Lyme borreliosis. Several spirochetal factors (e.g. infection with different genospecies, co-infection with other tick-transmitted pathogens) as well as host factors (e.g. cytokine patterns at the site of infection) influence the course of the disease. Diagnosis in the early stage of Lyme borreliosis relies on the clinical picture, whereas serological, molecular, microbiological, and histopathological findings are important adjuncts in the diagnosis of later stages of the infection. Antibiotic treatment is necessary for all stages and manifestations of Lyme borreliosis. Doxycycline is the antibiotic of choice for most patients with dermatoborrelioses.

Detection of Candidatus Neoehrlichia mikurensis, Borrelia burgdorferi sensu lato genospecies and Anaplasma phagocytophilum in a tick population from Austria.

Candidatus Neoehrlichia mikurensis DNA was discovered in Ixodes ricinus ticks in 1999 and is referred to as an emerging human pathogen since its first detection in patients with febrile illness reported in 2010. In recent years, Ca. Neoehrlichia mikurensis has been detected in ticks from several European, Asian, and African countries. However, no epidemiological data exist for Austria, which is a highly endemic region for tick-transmitted diseases. To assess the geographic spread and prevalence of Ca. Neoehrlichia mikurensis sympatric with other tick-transmitted pathogens, we analysed 518 I. ricinus ticks collected in 2002 and 2003 in Graz, Austria. The prevalence of Ca. Neoehrlichia mikurensis was 4.2%, that of Borrelia burgdorferi sensu lato 25.7%, and that of Anaplasma phagocytophilum 1%. Coinfections with Ca. Neoehrlichia mikurensis and B. burgdorferi sensu lato were found in 2.3% of all ticks. Thus, the results show a relatively high prevalence of Ca. Neoehrlichia mikurensis in Austrian ticks suggesting a high probability for the occurrence of undiagnosed human infections in Austria.

Chemokine signatures in the skin disorders of Lyme borreliosis in Europe: predominance of CXCL9 and CXCL10 in erythema migrans and acrodermatitis and CXCL13 in lymphocytoma.

The three skin disorders of Lyme borreliosis in Europe include erythema migrans, an acute, self-limited lesion; borrelial lymphocytoma, a subacute lesion; and acrodermatitis chronica atrophicans, a chronic lesion. Using quantitative reverse transcription-PCR, we determined mRNA expression of selected chemokines, cytokines, and leukocyte markers in skin samples from 100 patients with erythema migrans, borrelial lymphocytoma, or acrodermatitis chronica atrophicans and from 25 control subjects. Chemokine patterns in lesional skin in each of the three skin disorders included low but significant mRNA levels of the neutrophil chemoattractant CXCL1 and the dendritic cell chemoattractant CCL20 and intermediate levels of the macrophage chemoattractant CCL2. Erythema migrans and particularly acrodermatitis lesions had high mRNA expression of the T-cell-active chemokines CXCL9 and CXCL10 and low levels of the B-cell-active chemokine CXCL13, whereas lymphocytoma lesions had high levels of CXCL13 and lower levels of CXCL9 and CXCL10. This pattern of chemokine expression was consistent with leukocyte marker mRNA in lesional skin. Moreover, using immunohistologic methods, CD3(+) T cells and CXCL9 were visualized in erythema migrans and acrodermatitis lesions, and CD20(+) B cells and CXCL13 were seen in lymphocytoma lesions. Thus, erythema migrans and acrodermatitis chronica atrophicans have high levels of the T-cell-active chemokines CXCL9 and CXCL10, whereas borrelial lymphocytoma has high levels of the B-cell-active chemokine CXCL13.

Successful outcome of haemodialysis-induced pseudoporphyria after short-term oral N-acetylcysteine and switch to high-flux technique dialysis.

Pseudoporphyria is a blistering disease with skin fragility and shallow scarring that clinically and histopathologically closely resembles porphyria cutanea tarda. The two conditions can be distinguished by porphyrin levels that typically are elevated in porphyria cutanea tarda, but not or only slightly in pseudoporphyria. Pseudoporphyria can be induced by various medications (e.g. non-steroidal anti-inflammatory drugs, antibiotics, diuretics, retinoids), intense UV(A) exposure, or haemodialysis. Treatment of haemodialysis-associated pseudoporphyria is not yet standardized. We report here a 65-year-old male patient with chronic renal failure due to Waldenström's macroglobulinaemia who was treated with conventional 3 times/week haemodialysis. He developed blistering skin changes on both hands, which were diagnosed as pseudoporphyria based on clinical, histopathological, and laboratory findings, and could be successfully managed with initial oral N-acetylcysteine and a switch from low-flux to high-flux membrane haemodialysis. The beneficial effect of the high-flux membrane technique in haemodialysis-associated pseudoporphyria has not been previously reported.