Aphthous Lesions Research Articles

Demographical and Clinical Characteristics of Behcet's Disease in Southeastern Turkey.

BackgroundWe aimed to determine the demographic and clinical features of patients with Behcet’s disease (BD) in Southeastern Turkey.MethodsIn this study, files of 132 patients with BD (76 females and 56 males) who were diagnosed with BD according to the International Study Group criteria at the Department of Dermatology of Dicle University Faculty of Medicine from 2005 to 2009 were evaluated retrospectively. Demographical and clinical characteristics of the cases were recorded.ResultsMean age of the cases was 32.40 ± 9.4 years (range 15 - 59 years) and male/female ratio was 0.73. The mean age at diagnosis was 28.71 ± 9.1 years. Six cases were diagnosed as juvenile BD (4.45%). Oral aphthous lesions (100%) and genital ulcers (94%) were found to be the most common findings of the disease, followed by pathergy positivity (75%), papulopustular lesions (74.2%), erythema nodosum (43.2%), thrombophlebitis (6.8%) and extragenital ulcers (6.1%). Systemic involvement was noted as joint involvement in 79.5%, ocular involvement in 28.8%, vascular involvement in 9.8%, pulmonary involvement in 2.3%, neurologic involvement in 2.3% and genitourinary system involvement in 0.8%. There was no significant difference between mucocutaneous findings and systemic involvement ratios of male and female cases.ConclusionDemographic and clinical features of BD may vary according to geographical region, gender and ethnicity. We hope that this study will contribute to the epidemiologic data of BD which may exhibit different clinical and demographic features in different parts of the world.

Microchimerism in Behçet's disease

Behçet's disease (BD) is a multisystemic disorder of unknown etiology characterized by chronic relapsing oral aphthous lesions, genital ulcers, and uveitis. Immunological dysfunction is the most emphasized etiopathogenetic factor. Microchimerism is existence of an allogeneic DNA in a living creature. There are variable studies investigating the role of microchimerism on etiopathogenesis of autoimmune diseases. To our knowledge, no report has investigated the relationship between microchimerism and BD. We aimed to investigate the possible role of microchimerism on BD as an autoimmune disorder. We analyzed the SRY gene as an indicator of fetal microchimerism in our patient and healthy control groups. The patients were 105 women with BD over 18years old who had applied to multidisciplinary Behçet's disease clinic at Mersin University between 2005 and 2011. Patients were divided into two groups: group 1 consisted of 39 patients having a son, and group 2, 15 patients either nulliparous or having a daughter. Controls comprised two groups of women according to whether or not they had a son, i.e., group 3 (n=51) and group 4 (n=52), respectively. All patients with BD fulfilled International Study Group criteria for the diagnosis of BD. Forty-one of 54 (75.9%) patients and four of 103 (3.9%) controls showed presence of the SRY gene. The difference between the patient and control groups was statistically significant (P<0.001). As a result of our study, microchimerism may be associated with the etiopathogenesis of BD. However, we think there is a need for a larger series of studies to support this hypothesis.

Hand, foot, and mouth syndrome in an immunocompetent adult: a case report

BackgroundHand, foot, and mouth syndrome (HFMS) is a common acute illness. It is characterized by mild clinical symptoms including fever, blisters, and sores in the mouth and on the palms and soles following a 3- to 7-day incubation period. This syndrome is rarely seen in adults.Case presentationA 35-year-old male Caucasian patient had a history of multiple episodes of acute pharyngitis, hypertension, hypercholesterolemia, and occasional abdominal pain. He presented with polyarthralgia in the knees and hands and odynophagia, followed by fever, oral mucosal aphthous lesions, and vesicles on the palms and soles. Three weeks after presentation, he was admitted to the emergency room with acute myocarditis. The in-hospital evaluation revealed positive serology for coxsackie A9 (1:160), positive anti-transglutaminase and anti-gliadin antibodies, normal immunoglobulins, and human immunodeficiency virus negativity.ConclusionWe herein describe a case of HFMS that was associated with coxsackie A9 infection complicated by acute myocarditis. Although an association between celiac disease and HFMS has not been described, this patient’s immunologic disruption could have favored the development of infection and ultimately HFMS.

Incidence, Paris Classification, and Follow‐up in a Nationwide Incident Cohort of Pediatric Patients With Inflammatory Bowel Disease

The aim of the study was to evaluate the incidence, baseline disease characteristics, and disease location based on the Paris classification in pediatric inflammatory bowel disease (IBD) in the Hungarian nationwide inception cohort. In addition, 1-year follow-up with therapy was analyzed. From January 1, 2007 to December 31, 2009, newly diagnosed pediatric patients with IBD were prospectively registered. Twenty-seven pediatric gastroenterology centers participated in the data collection ensuring the data from the whole country. Newly diagnosed patients with IBD younger than 18 years were reported. Disease location was classified according to the Paris classification. A total of 420 patients were identified. The incidence rate of pediatric IBD was 7.48/10⁵ (95% confidence interval [CI] 6.34/10⁵-8.83/10⁵). The incidence for Crohn disease (CD) was 4.72/10⁵ (95% CI 3.82-5.79), for ulcerative colitis (UC) 2.32/10⁵ (95% CI 1.71-3.09), and for IBD-unclassified 0.45/10⁵ (95% CI 0.22-0.84). Most common location in CD was L3 (58.7%); typical upper gastrointestinal abnormalities (ulcer, erosion and aphthous lesion) were observed in 29.9%. Extensive colitis in patients with UC (E4, proximal to hepatic flexure) was the most common disease phenotype (57%), whereas only 5% of children had proctitis. A total of 18.6% of patients had ever severe disease (S1). Frequency of azathioprine administration at diagnosis was 29.5% in patients with CD, and this rate increased to 54.6% (130/238) at 1-year follow-up. In UC, only 3.3% received azathioprine initially, and this rate elevated to 22.5% (25/111). Use of corticosteroid decreased from 50% to 15.3% in patients with UC. Rate of bowel resection in patients with CD during the first year of follow-up was 5%. The incidence of pediatric IBD in Hungary was among the higher range reported. This is the first large, nationwide incident cohort analyzed according to the Paris classification, which is a useful tool to determine the characteristic pediatric CD phenotype.

Bechet vs Behçet: The Name Is Not the Same

Recently, during a superb lecture on lupus erythematosus (LE), the followingphraseslipped in: “hypertrophicLEwasdescribedbyBehcet.”This is a commonmistakewith a simple explanation. Every dermatologist is proud to be able to spell Behcet, and when confronted with the name Bechetwiththemiddle2 letters transposed,one’s “intrinsicspell checker” converts Bechet to Behcet. When one searches for Bechet on www .jamadermatology.com, Behcet turns up quite often. The describer of hypertrophic LE was Paul Esnard Bechet (Be-shay) (1881-1962) (Figure,A), a dermatologist of French descent fromNewOrleans, Louisiana, who practiced in New York City after training at Tulane University. Bechet frequentlypresentedcasesat theNewYorkDermatological Society, and the proceedings of thesemeetingswere published in theArchivesofDermatologyandSyphilology. In 1940hereportedthecase ofa49-year-oldmanwithdestructivehyperkeratoticnodulesonhis lipthat histologicallywere interpretedasLE. Inhis lateryears,Bechetbecamefascinatedwith dermatological history, writing an array of articles, including oneson thedevelopmentofAmericandermatology,NewYorkdermatology, the Skin and Cancer Hospital, and the American Dermatological Association, aswell asmany biographical sketches and obituaries. Bechet’smost famousnamesake is the legendary jazzmusician Sidney Bechet (1897-1959), a Creole whowas also born in NewOrleans. A childprodigyandmulti-instrumentalist, Bechet’smusical giftswere first recognized at the age of 6 years. After playing clarinet for years with a variety of local and nationally touring bands, in 1919 Bechet traveled to London,England,wherehetookupthesopranosaxophone,which,along withhispopular compositions suchas “PetiteFleur,”would catapult him to life-long international fame. Our othermain protagonist, Hulusi Behcet (Beh-chet) (1889-1948) (Figure, B), was the first professor of dermatology in Turkey, being appointed in 1933. In 1937he reported the caseof apatientwith recurrent oral aphthous ulcers, genital ulcers, and eye lesions to the Istanbul Dermatological Society. He later published a description of this patient and several others. AfterWorldWar II, both GuidoMiescher in Zurich, Switzerland, and Helen Ollendorff Curth in New York proposed the name Behcet disease for this constellation ofmucosal and ocular findings, often associated with severe ischemic vasculitis. Adamantiades-Behcet disease isprobablyamoreappropriatename.BenediktosAdamantiades (1875-1962), aGreekophthalmologistpracticing in theancientByzantine townofPrussa(whichtoday isBursa,Turkey),describedayoungmanwith recurrent iritis,oralandgenitalulcers,andarthritis inAthens in1930.However, the rivalriesbetweenGreeceandTurkeyduring thisperiodprobably contributed to Adamantiades not getting proper recognition. We hope this note will clear the confusion surrounding the similar surnames of 2 of the 20th century’s great dermatologists so that each is recognized appropriately—Behcet for describing the mucocutaneous ocular syndrome and Bechet for identifying hypertrophic LE.

Double‐balloon enteroscopy in the diagnosis of suspected isolated Crohn's disease of the small bowel

Diagnosis of Crohn's disease (CD) with isolated involvement of the small bowel remains a major challenge. Diagnostic procedures allowing direct insight into the midgut have become available with the introduction of double-balloon enteroscopy (DBE) and video-capsule endoscopy (VCE). The aim of the present study was to evaluate the role of DBE in the diagnosis of isolated CD of the small bowel. Sixteen patients (seven males) with suspected CD of the small bowel after exclusion of abnormal changes in the upper gastrointestinal tract and colon by esophagogastroduodenoscopy and colonoscopy including histology underwent DBE and high-resolution transabdominal ultrasound. In seven of 16 (44%) patients, abnormal macroscopic findings were detected by DBE (e.g. ileitis terminalis, inflammatory stenosis, aphthous lesions or jejunal ulcers). In one of thecases, histology confirmed pathognomonic findings consistent with CD. However, the diagnosis of CD was confirmed in 11/16 (69%) patients taking into account the clinical presentation and course of the disease as well as the endoscopic and imaging results. In cases of CD with clinical expression limited to the small bowel, DBE is a helpful tool. Nevertheless, in these patients, the combination of clinical findings and additional imaging modalities is required for confirmation of the diagnosis. A suggestive medical history, high-resolution ultrasound, EGD and colonoscopy lead the algorithm before the assessment with DBE.

Incidence, Clinical Characteristics, Long-Term Course, and Comparison of Progressive and Nonprogressive Cases of Aphthous-Type Crohn's Disease: A Single-Center Cohort Study

Background/Aims: In Japan, aphthous-type Crohn's disease (type A CD) is thought to represent an early phase of Crohn's disease (CD), and diagnosis of type A CD is possible in the diagnostic criteria for CD in Japan. However, the details of type A CD are not well understood. Methods: Subjects comprised 649 CD patients diagnosed between 1985 and 2011. The incidence of type A CD over time was clarified in two periods (1985-2004 and 2005-2011). The course of type A CD was also investigated, and cases that did and did not progress to typical CD were compared. Results: No significant difference was seen in the incidence of type A CD between the two periods (5.2 vs. 8.5%, p = 0.125). Type A CD patients followed at our hospital progressed to typical CD at a rate of 59.3%. In comparing progressive and nonprogressive cases, the frequency of large, densely distributed aphthous lesions in the small intestine was higher among progressive cases (p = 0.018). Conclusion: Type A CD is an early phase of CD, and CD diagnostic criteria including early cases are valid in Japan.

Pinworm Infestation Mimicking Crohns’ Disease

We here report a case of a young man who presented to his general practitioner with diarrhea. Inflammatory bowel disease was suspected and a colonoscopy showed aphthous lesions suggestive of Crohns' disease but biopsies revealed eggs of Enterobius vermicularis. When treated for this parasite, his symptoms were alleviated and a followup colonoscopy revealed a normal colon and distal ileum. Enterobius vermicularis is the most common parasite worldwide and has been attributed with many different presentations and pathologies. It is therefore necessary to maintain vigilance, even in high-income countries, in order to diagnose patients with one of the many atypical presentations of pinworms.

Oral mucosal fixed drug eruption: Characteristics and differential diagnosis

Little is known about the characteristic features of oral mucosal fixed drug eruption (FDE). To present the clinical highlights and the differential diagnosis of oral mucosal FDE in a relatively large group of patients from Turkey. This was a methodological, retrospective, cross-sectional study of 61 patients with oral mucosal FDE. The causative drug was established mainly by oral provocation test. The age range of 61 patients (38 females, 23 males) was 7 to 62 years. Naproxen and cotrimoxazole were the main inducers. Fourteen patients (23%) had a solitary oral lesion predominantly located on the dorsum of the tongue, or on the hard palate, the former statistically significantly associated with cotrimoxazole. Bullous/erosive (n = 47), aphthous (n = 12), and erythematous (n = 2) morphology were observed. A considerable number of patients were referred with a prior clinical diagnosis of herpes simplex and Behçet's disease; some of them were already receiving long-term treatment with acyclovir and colchicine, respectively. The main limitation of the present study resides in its retrospective design. Isolated oral lesions, aphthous lesions, severe bullous/erosive lesions, and the absence of residual pigmentation are the main features that may cause difficulties in the differential diagnosis. It is important to differentiate dysmenorrhea-related monthly attacks of oral FDE in female patients caused by nonsteroidal anti-inflammatory drugs from menstruation-triggered attacks of herpes simplex infection, and isolated orogenital aphthous FDE from Behçet's disease, especially in countries with a high frequency of the disease in order to prevent irrelevant therapies.

Nipple trauma in infants? Bednar aphthae

IntroductionBednar aphthae are infected wounds caused by trauma, localized to the hard palate in infants. They do not require specific treatment because they regress spontaneously in a few days. They often remain undiagnosed; other times, because of the pain they caused, they may worsen the nursing. Case reportWe describe the clinical case of a healthy infant of 2 months, fed with formula, who has 2 aphthous lesions in the oral cavity associated with irritability and inconsolable crying during feeding. We excluded the influence of infectious factors or underlying diseases. The hypothesis of a traumatic factor was supported by the anatomical features of aphthae and then confirmed by the gradual resolution of lesions after some advices on breastfeeding. ConclusionsOur intent is to provide a photographic record of Bednar aphthae, which are quite common but often misdiagnosed also because of lacking of photographic material. Improved knowledge of this condition helps physicians in the differential diagnosis of a traumatic condition that is not as unusual as it seems in newborns.

Impact of Helicobacter pylori on the clinical course of recurrent aphthous stomatitis

Recurrent aphthous stomatitis is one of the most common lesions of oral mucosa. Helicobacter pylori is suggested as one of the etiological agents of recurrent aphthous stomatitis. Here, we conduct a study for evaluating the impact of H. pylori eradication on clinical course of recurrent aphthous stomatitis. Forty-six patients with minor aphthous lesions were enrolled. The number of RAS lesions at last 6 months and vitamin B(12) levels were recorded. All patients were detected for H. pylori with endoscopic biopsy. H. pylori was positive in 30 patients and negative in 16 patients. H. pylori-positive 30 patients received eradication therapy. Three months after therapy, patients were re-evaluated with urea breath test; 18 patients were negative (eradicated), and the remainders (12 patients) were positive (non-eradicated) for H. pylori. 6 months after eradication, vitamin B(12) levels and number of aphthous lesions at 6 months were recorded. Vitamin B(12) levels were significantly increased in H. pylori-eradicated group (P = 0.001), whereas no significant change was found in non-eradicated group (P = 0.638). Mean number of aphthous lesions (per 6 months) of H. pylori-eradicated group was significantly decreased after eradication (P = 0.0001); in the non-eradicated group, no significant change was found (P = 0.677). In Hp-positive group, number of RAS lesions and vitamin B(12) levels were negatively correlated when evaluated both before and after eradication. This study provides evidence to support the beneficial effect of H. pylori eradication in patients with recurrent aphthous stomatitis. The underlying mechanism might be the increase in vitamin B(12) levels after eradication.

Delayed Diagnosis of Squamous Cell Carcinoma of the Scrotum in a Patient with Behçet's Disease

Dear Editor: Behcet's disease (BD), a chronic inflammatory disease, is characterized by oral apthae and genital ulcer, arthritis, cutaneous lesions, such as non-bacterial folliculitis, and erythema nodosum, as well as ocular, gastrointestinal and neurological manifestations. Although genital ulcers are histologically similar to oral aphthae, they are deeper than oral aphthous ulcers and are a major criterion for diagnosis. We present a case of scrotal squamous cell carcinoma (SCC) in a patient diagnosed with BD. A 39-year-old male patient was admitted to our clinic with recurrent oral aphthae and a deep ulcer, covering the scrotum and perineal regions. The patient had been suffering from recurrent oral aphthous lesions for almost 11 years, and he had been medicated with 1.5 mg/day of colchicine, with a diagnosis of BD. Over the preceding 2 years, the genital ulcer had gradually enlarged and deepened. During this period, the patient received topical treatment and systemic antibiotics therapy; however, he was lost to follow-up. Dermatological examination revealed a large and painless ulcer, extending from the scrotum to perineal region, which was approximately 8 cm in diameter (Fig. 1). This ulcer had an indurated floor and also sharp and irregular edges. No growth was observed in the bacterial and fungal cultures of the genital ulcer. No inguinal lymphadenopathy was noted and syphilis tests were negative. Fig. 1 Well-demarcated large destructive ulcers on the scrotum and perineal area. The ulcer had a foul odored heavy discharge; it was considered as a secondary bacterial infection and systemic antibiotics therapy was initiated. After the treatment of wound infection, the lesion was excised with a margin of at least 1 cm of normal skin. The surgical specimen showed neoplastic proliferation of squamous differentiated tumour cells in the epidermis (Fig. 2). The test of the tumor cells for anti-human papilloma viruses (HPVs) antibodies was negative; furthermore, HPVs DNA was not detected by direct polymerase chain reaction (PCR) method in excisional tissue specimen. In addition, abdominal, chest and pelvic computed tomography scan showed no evident distant metastasis, including the regional lymph nodes. Six months after surgical treatment, local recurrence or metastasis was not detected yet. Fig. 2 (a) Histological picture of squamous cell carcinoma: Neoplastic proliferation of squamous differentiated tumour cells with dyskeratotic and atypical keratinocytes in the epidermis (H&E, ×200). (b) Moderately differentiated tumour cells ... Genital ulcers in BD are sometimes large and deeply located; however, they heal with scarring in 4 weeks1. Our patient had been suffering from genital ulcer for two years. The ulcer had a nodulo-ulcerative appearance and its base was highly indurated. The clinical features of the ulcer and its unresponsiveness to treatment led us to investigate other possible causes of genital ulcer. We doubted whether the SCC developed on the chronic genital ulcer of BD or emerged directly as SCC, unrelated with genital ulcer. In fact, SCC was diagnosed at first by us during his first appointment at our clinic. However, we suggest that the lesion occurred directly as SCC, due to infiltration of the whole ulcer region and the rapid progression of the lesion. Skin tumours in BD are rarely observed2. In situ SCC development on a chronic vulvar ulcer of a female patient with BD was previously reported. The authors had pointed out that long-term systemic corticosteroid treatment could contribute to the development of SCC in this genital ulcer, which was resistant to treatment3. Satolli et al.4 reported a Merkel cell carcinoma in a patient with BD, and mentioned that the immunosuppresives may lead to the development of cancer. In our case, there was no predisposition for the development of SCC, such as immunosuprressive drugs, arsenic exposure, irradiation, burn scarring, and HPV infection. We suppose that the presence of SCC together with BD is coincidental. Like BD, the other dermatoses, composed of various components, should be followed carefully, and differential diagnoses of signs and symptoms should be made. Especially, all clinical features of a genital ulcer should be noted and unusual causes should not be forgotten.

Pathergy as a Cause of False-positive Tuberculin Skin Test

To the Editors: Differential diagnosis of intestinal tuberculosis (ITB) versus inflammatory bowel conditions can be challenging, especially in areas in which tuberculosis prevalence is very high or very low.1 Clinical presentation can be indistinguishable, and the extraintestinal manifestations of both conditions can be similar. Correct and prompt diagnosis is critical to avoid dire consequences, especially in the era of biologic therapies.2 The diagnosis of ITB can be very difficult, as both acid-fast bacilli staining and biopsy culture have low sensitivity, and the new polymerase chain reaction assays are not available in poor resource settings.1 Tuberculin skin test (TST) is often used in the diagnostic process. We report on a seldom considered cause of false-positive results to TST, possibly leading to a wrong diagnosis. A 15-year-old, Indian-born girl was admitted to our hospital because of a 6-month history of diarrhea and abdominal pain. She also presented recurrent painful oral ulcers, and ocular redness and pain in the left eye. One year earlier, she had received a diagnosis of tuberculosis-related erythema nodosum. A TST performed on that occasion resulted in extensive induration followed by blistering and ulceration of a 3-cm-wide skin area. Chest radiograph was negative for tuberculosis lesions, and she had been treated with a 6-month course of isoniazid. On admission to our hospital, laboratory tests showed elevated erythrocyte sedimentation rate, 78 mm/h, and fecal calprotectin (74 mg/kg of stools; normal values, <15), a marker of intestinal inflammation. Colonoscopy and video capsule endoscopy were performed to rule out ITB and inflammatory bowel disease. Sparse aphthous lesions were seen in the distal ileum. Polymerase chain reaction assay for Mycobacterium tuberculosis and staining for acid-fast bacilli were negative, as were also chest radiograph and interferon-gamma release assay results (QuantiFERON-TB Gold test; Cellestis Limited, Australia). An ophthalmology examination revealed acute anterior uveitis in the left eye. The patient's findings fulfilled the criteria for Behçet disease (BD) according to the International Study Group (recurrent oral ulceration, uveitis, erythema nodosum).3 Intestinal involvement in the form of ileal mucosal ulcers is also commonly reported in BD.4 She was treated with a 2-month course of oral prednisone and made a full recovery. The strongly positive TST can be explained as a pathergic reaction. Pathergy is characterized by the triggering of a cutaneous inflammatory response to minor trauma like needle prick. It is mainly described in BD and in some other neutrophilic dermatoses like pyoderma gangrenosum.5 Therefore, in presence of clinical and endoscopic finding compatible with both ITB and BD, the possibility of a false-positive TST caused by a pathergic reaction should be considered. On the other hand, BD and tuberculosis can coexist, so caution is mandatory. Serena Pastore, MD Samuele Naviglio, MD Alessandro Ventura, MD Institute for Maternal and Child Health IRCCS “Burlo Garofolo” University of Trieste Trieste, Italy

Aphthous Lesions Throughout the Small Bowel on Capsule Enteroscopy: A Call for Validated Criteria for Crohnʼs Disease

Aphthous Lesions Throughout the Small Bowel on Capsule Enteroscopy: A Call for Validated Criteria for Crohnʼs Disease

Diagnostic yield of upper endoscopy in paediatric patients with Crohn's disease and ulcerative colitis. Subanalysis of the HUPIR registry

Background, aims According to Porto Criteria upper gastrointestinal (UGI) endoscopy is recommended in patients with suspected inflammatory bowel disease (IBD). Nevertheless, previous studies revealed frequent involvement of UGI tract even in patients with ulcerative colitis (UC). The aim of the present study was to determine the diagnostic role of esophagogastroduodenoscopy (EGD) and assess the prevalence and different aspects of UGI involvement in children registered in the Hungarian Pediatric IBD Registry (HUPIR) from 1st of January 2007 to 31th of December 2009. Methods Twenty seven institutes provided prospective follow-up data about newly diagnosed IBD patients to HUPIR. The registry was based on detailed questionnaire (76 parameters) involving anamnestic data, laboratory findings, activity indexes, diagnostic procedures, endoscopic examinations (EGD and ileocolonoscopy), and histological data. Localization and phenotype of disease were based on the Montreal classification criteria. Results During the 3-year period 420 children were diagnosed with IBD, 265 (63%) of them had Crohn's disease (CD), 130 (31%) UC, and 25 (6%) IBD-unclassified (IBD-U). The mean age at diagnosis was 13.2 years (range: 1.2–18 years). EGD was performed in 237 patients (56%), in most cases in patients suffering from CD. Macroscopic lesions on EGD were noted in 64% of patients with CD and 40% of children with UC. Characteristic lesions for CD (ulcer, erosion, aphthous lesion, and granuloma) were noted in 31% of CD patients, however, EGD helped to establish the final diagnosis in 9% of CD patients (diagnostic yield, 9%). Conclusions There was a high frequency of UGI involvement in children with CD and UC. One third of CD patients showed significant lesions at upper endoscopy and one patient out of ten had real diagnostic help from EGD.

Queratose liquenoide crônica: relato de caso

Keratosis lichenoides chronica or Nekam's disease is a rare mucocutaneous dermatosis characterized by keratinization. It is chronic and progressive usually affecting individuals aged 20-40 years. Around 70 cases have been reported in the literature. Due to the rarity of this condition and the lack of effective treatment, it is a difficult disease to manage. In the case described below we present a 42-year old patient with violaceous and hyperkeratotic papules in linear, reticular or plaque form, located on the trunk and limbs for five years. Aphthous lesions in the oral cavity and shallow ulcers on the genitalia also formed part of the clinical manifestation. Pathologic examination was suggestive of keratosis lichenoides chronica. Acitretin and dapsone was introduced and the lesions partially improved.

Anticoagulant and Fibrinolytic Disorders in Patients with Behçet's Disease and Recurrent Aphthous Ulcer

Behçet's disease (BD) is a chronic multisystemic inflammatory disorder characterized by recurrent oral and genital aphthous ulcers, uveitis and skin lesions. Recurrent aphthous ulcer (RAU) is the most prevalent oral mucosal disease in humans. The pathogenesis and thrombopoiesis of BD and RAU have not been fully clarified. To reveal the haemostatic dysfunctions in the patients with BD and RAU, we evaluated the levels of coagulant, anticoagulant and fibrinolytic parameters in these patients.Factor VIII clotting activity (FVIII:c), protein C antigen (PC:Ag), total protein S antigen (TPS: Ag), tissue-type plasminogen activator antigen (t-PA:Ag), plasminogen activator inhibitor-1 antigen (PAI-1:Ag) and D-dimer were detected in 24 BD, 58 RAU patients and 50 controls. Results showed that levels of PC:Ag, TPS:Ag, PAI-1:Ag and D-dimer were significantly elevated in both BD and RAU patients compared with controls (P<0.01). PAI-1:Ag was even higher in BD patients than in RAU patients (74.99±12.28 vs. 69.57±13.11, P<0.05), whereas the level of t-PA:Ag was significantly reduced in patients with BD and RAU (P<0.01). In patients with RAU, PC:Ag was lower in major aphthous ulcer (MjAU) group than in minor aphthous ulcer (MiAU) group (P<0.05). The expression of FVIII:c was significantly elevated in MiAU patients compared with controls (P<0.01), while no difference was observed between MjAU patients and controls (P>0.05). Our studies showed that there were anticoagulant and fibrinolytic disorders in BD patients, which may be responsible for diminished fibrinolysis in BD. Some haemostatic parameters may be correlated with the severity of RAU.

Expression of Th1/Th2/Th3/Th17-Related Genes in Recurrent Aphthous Ulcers

The pathogenesis of recurrent aphthous ulceration (RAU) is unknown, although an abnormal immune reaction appears to be involved. RAU may result from oral epithelium damage caused by T cell-mediated immune response. To improve understanding of the role of T cells in RAU, the present study analyzed. the expression of T cell-related genes in oral ulcers from patients with RAU, as well as in healthy non-keratinized oral mucosa from aphthae-free volunteers. Biopsies from RAU patients and healthy individuals were analyzed using Human Th1-Th2-Th3 RT(2) Profiler PCR Array and qRT-PCR that allowed to quantify the transcript levels of 86 genes related to T cell activation. We found that cells present in aphthous ulcers express a characteristic Th1-like gene profile. The majority of genes up-regulated in aphthous lesions such as IFN-γ, TNF, IL-15, IRF1, STAT-1 and STAT-4 were Th1-associated. Th2-realated genes were not overexpressed in RAU tissues, with the exception for CCR3. Th3- and Th17-related gene expression patterns were not demonstrated in RAU. These findings clearly reveal that aphthous ulcer formation is predominantly dependent on the activation of the Th1-type immune response.

Endoscopic diagnosis of acute intestinal GVHD following allogeneic hematopoietic SCT: a retrospective analysis in 175 patients

Diagnosis of acute intestinal GVHD (aGVHD) following allogeneic hematopoietic cell transplantation is based on clinical symptoms and histological lesions. This retrospective analysis aimed to validate the ‘Freiburg Criteria' for the endoscopic grading of intestinal aGVHD. Grade 1: no clear-cut criteria; grade 2: spotted erythema; grade 3: aphthous lesions; and grade 4: confluent defects, ulcers, denudation of the mucosa. Having excluded patients with infectious diarrhea, we evaluated 175 consecutive patients between January 2001 and June 2009. Setting a cutoff between grade 1 (no change in therapy) and grade 2 (intensification of immunosuppression), macroscopy had a sensitivity of 89.2% (95% confidence interval (CI): 80.4–94.9%), a specificity of 79.4% (95% CI: 69.6–87.1%), a positive-predictive value of 79.6% (95% CI: 70.0–87.2%) and a negative-predictive value of 89.0% (95% CI: 80.2–94.9%). In all, 20% of patients with aGVHD in the lower gastrointestinal tract (GIT) had lesions only in the terminal ileum. In all patients with aGVHD ⩾2 of the upper GIT, typical lesions were also found in the lower GIT. Ileo-colonoscopy showed the highest diagnostic yield for aGVHD. In conclusion, the ‘Freiburg Criteria' for macroscopic diagnosis of intestinal aGVHD provide high accuracy for identifying aGVHD ⩾2.

Autoinflammatorische Erkrankungen als Ursache von Wundheilungsstörungen

Ulcerations of the skin and mucosal membranes are a common feature of autoinflammatory diseases. They can give raise to chronic wound healing defects and should be considered in the differential diagnosis of chronic skin ulcers. The increased activation of the innate immune system in the absence of an apparent provocation for inflammation is a hallmark of autoinflammatory diseases. Mutations and alterations of signaling pathways regulating the innate immune response to physical trauma/tissue damage result into an unrestrained activation of the inflammasome, which leads to increased activation of Interleukin-1. Uncontrolled recruitment and activation of myeloid effector cells within the wound site lead to the release of potent proteases that cause the degradation of structural components of the skin. The majority of these diseases respond well to immunosuppressive and immunomodulatory treatment regimes. Therapeutic resistance converts the acute inflammatory response into a chronic and non-resolving inflammatory process that leads to tissue degeneration. In this article we will focus on the review of those autoinflammatory diseases that often display ulcerative cutaneous and aphthous lesions including pyoderma gangrenosum, Behçet disease, PAPA syndrome and hyperimmunoglobulinemia D with periodic fever syndrome (HIDS). Furthermore, the article will be complemented by an overview of those inflammatory diseases that are associated with non-ulcerative cutaneous manifestations.