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Background and objectives: Documenting patient data in psoriasis clinical practice can improve care, but standardized and transparent documentation is rare. The current project aimed to develop a data set for the documentation of psoriasis in daily practice. Material and methods: In four online Delphi rounds and one in-person meeting, 27 psoriasis experts allocated variables to a standard, an optimal and an optional data set. Most of the questions were standardized. Open questions were included to allow for the provision of reasons and to enlarge the data sets. Furthermore, in the in-person meeting we considered a) patients' attitudes and b) dermatologists' information on the current usage and acceptability in Germany. Results: The consensus approach resulted in a data set with 69 variables. The standard data set includes 20, the optimal data set 31 and the optional data set 18 variables. In summary, the data set can mainly be grouped into master data, general status and medical history data, medical history of psoriasis, status of psoriasis, diagnostics and comorbidity, therapies and patient-reported outcomes. Conclusions: The consensus recommendation of a standard, an optimal and an optional data set for routine care of psoriasis intends to be a decision-making aid and an orientation for both daily practice and further development of documentation systems.

A 69-year-old man presented with a 12-month history of unilateral eczematous changes of the left nipple and areola without any signs of systemic inflammation. His main complaints were mild, intermittent itching, and occasionally local pain or burning. An external initial treatment with topical corticosteroids combined with disinfectants/antibiotics did not lead to an improvement. Triggering factors or any other conditions that could affect the diagnosis could not be detected. Histopathological examination of a biopsy from the left nipple performed at admission confirmed the suspected diagnosis of Paget disease. Radical left breast mastectomy was performed and the patient is under regular follow-up.Ein 69-jähriger Patient stellte sich mit einer 12-monatigen Anamnese von einseitigen ekzematösen Veränderungen der linken Brustwarze und des Warzenhofs ohne systemische Entzündungszeichen vor. Seine Hauptsymptome waren milder, zeitweiliger Juckreiz und gelegentlich lokale Schmerzen oder Brennen. Eine externe Erstbehandlung mit topischen Kortikosteroiden in Kombination mit Desinfektionsmitteln/Antibiotika führte zu keiner Besserung. Auslösende Faktoren oder andere Zustände, die die Diagnose beeinflussen könnten, konnten nicht eruiert werden. Die histologische Untersuchung der bei der Aufnahme durchgeführten Biopsie aus der linken Brustwarze bestätigte die Verdachtsdiagnose eines Morbus Paget. Eine radikale linke Mastektomie wurde durchgeführt, und der Patient befindet sich in regelmäßiger Nachsorge.

BACKGROUND: Tufted angioma/kaposiform hemangioendothelioma represents a spectrum of rare benign skin vascular neoplasms that differ in the extent and prognosis. Tufted angioma affects primarily children, is localized to skin, shows no tendency for ulceration or tissue invasion and has a favorable prognosis. CASE REPORT: In this case report, tufted angioma involved the lower eyelid resulting in its destruction and compromised its function, hence necessitating rapid surgical excision to preserve the function of eyelid. CONCLUSION: TA/KHE is a rare vascular benign neoplasm usually asymptomatic but may affect delicate structures, such as the eyelid resulting in destruction and hence may require rapid surgical intervention. corresponding eye.

Die Einfuhrung der Biologika hat die Therapie der mittelschweren bis schweren Plaquepsoriasis bahnbrechend verandert. Indem die biologischen Therapien der Psoriasis kontinuierlich erweitert werden, ist es besonders wichtig, die Wirksamkeit und Sicherheit der Substanzen nicht nur in klinischen Studien, sondern auch in langfristigen Anwendungsbeobachtungen im Rahmen von Registern zu ermitteln. Es erfolgt die Darstellung typischer Nebenwirkungen und signifikanter Risiken der Biologikatherapien im Rahmen der Psoriasis unter der Berucksichtigung psoriatischer Kontrollpopulationen. Es wurde eine selektive Literatursuche in PubMed durchgefuhrt, und Studien zur Langzeitsicherheit im Rahmen der Psoriasisregister PsoBest, PSOLAR und BADBIR wurden ausgewertet. Zur Beurteilung der langfristigen Sicherheit der Biologika ist es von besonderer medizinischer Bedeutung, die Verlaufe an moglichst vielen Patienten in Langzeitregistern zu beobachten. Eine Tendenz zu verbesserten Nebenwirkungs- und Sicherheitsprofilen der neueren biologischen Medikamente scheint sich abzuzeichnen.

A 40-year-old patient with malignant melanoma pT4bN0M1a stage IV (AJCC classification 2017) was treated with the PD-1/PD-L1 antibody pembrolizumab. Three months after treatment initiation the patient developed enlarged mediastinal lymph nodes despite the partial response of the cutaneous metastases. An elective lymph node extirpation was performed. The histopathological result was consistent with a sarcoid-like reaction. Treatment was discontinued and a pulse therapy with systemic steroids led to a significant remission of the lymphadenopathy. This side effect can manifest both during PD-1/PD-L1 antibody and CTLA-4 antibody therapy and should be included in the differential diagnosis of mediastinal lymphadenopathy.

Schistosomiasis (bilharziasis) is an endemic parasitic disease of a major source of morbidity and mortality for developing countries. It is caused by different species of blood flukes (trematode worms) of the genus Schistosoma, the most important of which are S. haematobium which infests the genitourinary tract and S. mansoni and S. japonicum which infest the gastrointestinal tract. The incidence of schistosome-induced male infertility is not known and probably underestimated being overshadowed by the more severe and often fatal urinary and gastrointestinal complications. Several causes may contribute to schistosomiasis-induced male infertility, such as hormonal imbalance, testicular tissue damage and genital ductal system obstruction, in addition to decreased libido and erectile dysfunction due to associated hormonal imbalance and prostatic infestation. Demonstration of the schistosome ova in seminal plasma or testicular tissue confirms the diagnosis. Treatment of schistosomiasis-induced male infertility depends on clinical evaluation and includes, besides anthelmintic treatment, hormonal replacement and assisted reproduction (IVF/ICSI) in cases of severe oligozoospermia and or obstructive and nonobstructive azoospermia.

More than 1.5 million people were diagnosed with skin cancer in 2012 in Germany-of which 318,000 were malignant melanoma. The number of malignant skin tumours has increased by 60% since 2005. Epithelial skin cancers are even more common. Since 2012, 1.3 million diagnoses have been documented. This incidence represents an increase of 79% within 7 years. The number of skin cancer patients treated in German hospitals has also increased. In 2014, 99,613 patients were treated as inpatients with the diagnosis of skin cancer; in 2000 there were 57,147 patients. This was the largest growth rate among all cancer treatments in hospitalised patients. The continuously changing age pyramid leads to an expected further growth of the incidence of skin tumours. In parallel the development of molecular knowledge in tumorigenesis is also rapid. A series of cell-specific mutations have been described in recent years for various skin tumours. Mutations are found mainly in genes engaging their translation products at key positions in regulatory cell metabolism or cell division. These include oncogenes, which have greatly increased activity due to targeted mutations or tumor suppressor genes and act under physiological conditions as negative regulators that are inactivated by mutations. These findings have led to the development of a series of new promising compounds for the treatment of skin tumours.

Chronisch rezidivierende Aphthen sind die haufigsten entzundlichen Lasionen der Mundschleimhaut mit bis zu 10% in der Bevolkerung und hoherer Pravalenz bei Kindern. Anamnese, klinische Morphe, Pradilektionsstellen und ein phasenhafter Ablauf sind hilfreich, um Erkrankungen, die aphthenahnlich imponieren konnen, zu unterscheiden. Konditionierende Grunderkrankungen sollten ausgeschlossen werden. Die Therapie soll die Schmerzhaftigkeit und die funktionellen Beeintrachtigungen sowie die Frequenz und den Schweregrad der Rezidive reduzieren. Bei leichteren Verlaufsformen stehen symptomatisch wirkende Lokalpraparate zur Verfugung. Bei schweren Verlaufen einer Aphthose der Majorform oder bei Systembeteiligung kommen ebenfalls lokale Masnahmen neben einer systemischen Therapie in Betracht, z. B. mit Colchicin, Pentoxifyllin oder Prednisolon. Im Rahmen des Morbus Admantiades-Behcet kann durch systemische Applikation immunmodulatorisch wirksamer Medikamente das Auftreten neuer Aphthen vermieden werden. Die Arbeit gibt einen Uberblick uber Erscheinungs- und Verlaufsformen, Differenzialdiagnosen und an Studien orientierten Therapiemoglichkeiten der Aphthen.

Epidemiological studies demonstrate a prevalence of Adamantiades-Behcets disease (MAB) in the range of 0.12-420 per 100,000 inhabitants with the highest rates in Istanbul, Turkey and the lowest rates in the USA. Ophthalmological data on the prevalence of ocular involvement are limited for MAB in Germany because most epidemiological studies are based on rheumatological or dermatological data. Berlin is the city with the highest number of non-native German inhabitants and its multiethnic character renders it uniquely appropriate for epidemiological studies on MAB. This article summarizes the most important epidemiological data of 140 patients (63 female and 77 male) with a mean follow-up of 6.4 years (range 0.5-22 years) which we have recently published. The mean age was 23 years at the first manifestation and 32 years when the fully developed disease was recorded. The mean age at onset of ocular involvement was 30 years, 56% of patients developed ocular involvement, which was the first manifestation in 8.6% and the second manifestation in 19.3%. More than half the patients developed ocular involvement. The calculated prevalence of ocular involvement in MAB is 1.77/100,000 inhabitants for the population of Berlin.

Of the 721 documented patients in the German Registry for Adamantiades-Behcet disease (registered charity), 258 were of German and 308 of Turkish descent, along with 30 other countries of origin. The prevalence in Germany is 0.9:100,000. Manifestation of the disease was predominantly in the third decade of life (median age: 26.5 years) and in 10.7% the disease onset was under 16 years of age. The full clinical picture developed on average in 2.9 years (median 3 months). Patients of Turkish descent showed androtopism in contrast to those of German descent (female:male 1.9:1), which was also detected in the whole collective in a ratio of 1.4:1. In 12.4% there was a family history with differences between German and Turkish patients (3.8% versus 14.6%) as well as in patients with disease onset in young and adult age (25.0% versus 7.3%). Most frequent features included oral aphthae (98.5%), cutaneous lesions (81%), genital ulcers (64.7%), ocular manifestations (51.6%), arthritis (52.4%) and positive pathergy test (30.8%). Turkish patients suffered significantly more often from eye manifestations compared with Germans, while in German patients prostatitis/epididymitis and gastrointestinal involvement were more frequently documented. As serious complications arose blindness 6.8%, meningoencephalitis 4.0%, severe arthritis 2.6%, fatal outcome 1.2%, hemoptysis 1.1% and gastrointestinal perforation 0.5%. The HLA-B5 antigen was positive in 58.1% and showed an association with eye manifestations. The relative risk of HLA-B5 positive individuals is high in both Germans (6.57) and Turks (5.81).

Congenital bilateral absence of vas deferens (CBAVD) results in obstructive azoospermia in which testicular function, such as spermatogenesis, is preserved. Bilateral testicular biopsy is not only diagnostic but also therapeutic as retrieved spermatozoa are usually cryopreserved for assisted reproduction. In this case report, CBAVD was confirmed in a 24-year-old azoospermic man on the basis of persistent azoospermia associated with low semen volume, absent fructose and normal hormonal levels, ultrasonographically (absent seminal vesicles) and histologically (intact spermatogenesis). Interestingly and incidentally, only the right testicular biopsy showed ova of two parasitic species of Schistosoma, namely Schistosoma haematobium which infests the genitourinary tract and Schistosoma mansoni which infests the gastrointestinal tract. Both species are rare causes of azoospermia, and the case should be further managed as CBAVD in which the left testicle is considered the preferred site of sperm retrieval for assisted reproduction.

Although the exact etiology of alopecia areata is still unknown, systemic prednisolone treatment seem to be effective in early stages but significant side effects may occur leading to discontinuation of treatment.Evaluation of efficacy and saftety of a short-term medium-dose pulse prednisolone treatment in alopecia areata.84% of the patients with multifocal alopecia areata markedly improved after the completion of the three courses. A patient with the ophiasis type only responded after the third course, but relapsed 7 months later. The patients with alopecia areata totalis and universalis did not respond to the treatment. No major side effects were observed.Monocenter prospective study of intravenous 100 mg intravenous prednisolone pulse therapy on 3 consecutive days at 1-month-intervals for three courses in 23 patients with active alopecia areata rapidly evolving and/or resistant to topical therapies and no contraindication for systemic steroids.A series of three monthly courses of medium-dose prednisolone pulse therapy is effective and well tolerated in most patients with active, multifocal alopecia areata. The results are rather disappointing in patients with alopecia areara totalis/universalis.

To evaluate the association of HLA-B51 and ocular involvement in Adamantiades–Behcet's disease. We retrospectively analysed all patients with Adamantiades–Behcet's disease examined in our Department of Ophthalmology since 1982. All patients fulfilled the criteria of the International Study Group for Behcet's disease. We included 140 patients (63 female and 77 male) with a mean follow-up of 6.4 years. The mean age at the first manifestation was 23 years; full disease was noted at 32 years. The mean age at the time of eye involvement was 30 years. Most of the patients were of Turkish (n=73) or German (n=34) origin. A total of 56% patients developed eye involvement. Forty-nine out of 76 HLA-B51-positive patients (64.5%) and 26 out of 60 HLA-B51-negative patients (43.3%; P=0.014) developed ocular involvement. More than the half of the patients with Adamantiades–Behcet's disease evaluated in our department developed ocular involvement. There was a statistically significantly higher frequency of HLA-B51 in these patients.

© Blackwell Verlag GmbH • www.blackwell.de • 1610-0379/2006/0401-0049 JDDG | 1 2006 (Band 4) Introduction Historical background and nomenclature In his third book of “Epidimion” Hippocrates of Kos (460–377 BC) described a disease with symptoms comparable to the cardinal symptoms of Adamantiades-Behcet disease (ABD): “Many patients had aphthous ulcerations in the mouth. There were numerous purulent genital ulcers, as well as furuncles, which were external and internal, as well as in the groins. There was a watery inflammation of the eye that was chronic and painful as well as growths on the eyelids – external and internal – which impaired eyesight in many patients. (...) There were also larger ecthymata and large herpetic lesions in many patients. “ In 1930 Benediktos Adamantiades (1875–1965), a Greek ophthalmologist, presented a lecture describing findings in an 18-year-old patient, for the first time grouping ocular symptoms, oral aphthae, and genital ulcers together as symptoms of a single clinical entity. He later added vascular involvement as a fourth cardinal symptom and in a review article presented the first classification of the disease by describing its ocular, mucocutaneous, and systemic forms [1]. The chief symptom, which according to Adamantiades was hypopyon, appears at various intervals, causing more or less lasting permanent damage and ultimately resulting in blindness. Hulusi Behcet (1889–1948), a Turkish dermatologist, first described in 1937 patients with recurrent lesions affecting the mouth, genitals, and eyes, adding a catalogue of symptoms including periodontitis, jaw cysts, acneiform skin changes, and erythema nodosum as well as arthralgia. Behcet’s intense work on the disease and his conviction about its autonomous character finally brought this puzzling clinical picture to the attention of the scientific community. CME

Oral aphthous ulcers typically present as painful, sharply circumscribed fibrin-covered mucosal defects with a hyperemic border. Chronic recurrent oral aphthous ulcers occur in three different clinical morphological variants and with two different time courses. Small ulcers of the minor-type (Mikulicz) are less than 1 cm in diameter (usually 2–5 mm) and heal spontaneously in 4–14 days. They account for 80–90% of all recurrent oral aphthous ulcers (1, e1). Scarring occurs in around 8% of cases (1, e2) (Figure 1a). Large ulcers of the major-type (Sutton ulcers) are usually 1–3 cm in diameter, deeply indurated and can last for 10 days to 6 weeks or occasionally even longer (1, e3) (Figure 1b). They account for around 10% of recurrent benign oral ulcers. About 64% of Sutton ulcers heal with scarring. Herpetiform aphthous ulcers are very small (1–2 mm) grouped lesions (1, e4) (Figure 1c). They account for around 5% of recurrent oral aphthous ulcers, are extremely painful and persist for 7–10 days. As many as a 100 ulcers can be present; they may coalesce into larger erosive plagues and about 32% heal with scarring. The three morphologic variants can occasionally appear simultaneously (2). Figure 1 (a) Minor-type oral aphthous ulcers, (b) major-type oral aphthous ulcer, (c) herpetiform oral aphthous ulcers Another classification is based on the time course. The simple chronic recurrent oral aphthous ulcers present with a limited number of small, quickly healing, minimally painful ulcers limited to the oral mucosa and recurring with 3–6 episodes annually. In complex aphthosis, there are a few or many slowly healing intensely painful ulcers on the oral and perhaps genital mucosa (3). The latter may also be perigenital, affecting the scrotum, vulva, anus, perineum and inguinal region. Complex aphthosis features frequently appearing ulcers with either short lesion-free periods or even repeatedly recurrent ulcers, severe pain and even systemic effects such as interference with eating and the resultant problems of inadequate nutrition (3).

Objective We investigated the effect of gender on the clinical Adamantiades-Behcet's disease (ABD) phenotype with data from the German ABD registry and a meta-analysis from a systematic literature review. Methods Using the German ABD registry data, we compared 36 clinical variables by gender (with women as the reference category) and investigated potential effect modification by HLA-B5 or ethnic background. The registry data were combined with those from a literature search to calculate pooled relative risks (RRs) for variables with data from ≥10 relevant datasets. Results The German ABD registry provided information for 747 subjects (58.1% males) and the systematic literature review identified another 52 datasets informing on 16 variables. Both analyses consistently revealed the association of male gender with ocular involvement (RR 1.28 and 1.34 from the ABD registry and meta-analysis, respectively), folliculitis (RR 1.30 and 1.26), papulopustular lesions (RR 1.23 and 1.25), vascular involvement (RR 2.31 and 2.27), superficial (RR 2.96 and 1.63) and deep venous thromboses (RR 2.56 and 2.16) and female gender with genital ulcers (RR 0.78 and 0.92) and joint involvement (RR 0.79 and 0.89). The ABD registry data additionally showed male gender associated with heart involvement (RR 10.60), whereas the meta-analyses revealed male gender associated with the pathergy test (RR 1.14) and female gender associated with erythema nodosum (RR 0.86). HLA-B5 and Turkish or German origin did not affect the observed associations. Conclusion These analyses support gender-associated clinical variations in ABD and in particular a clinically meaningful risk of cardiovascular involvement for men.

Cellular immunity plays a major role in controlling human papilloma virus infection and development of cervical carcinoma. Mononuclear cell infiltration possibly due to the action of chemokines becomes prominent in the tumor tissue. In fact, the macrophage chemoattractant protein-1, MCP-1, was detected in cervical squamous cell carcinoma in situ, whereas absent in cultured cells. From this, unknown environmental factors were postulated regulating chemokine expression in vivo. In this study, we show high CD40 expression on cervical carcinoma cells and CD40 ligand (CD40L) staining on attracted T cells in tumor tissue, suggesting a paracrine stimulation mechanism via CD40L-CD40 interactions. We therefore investigated chemokine synthesis in nonmalignant and malignant human papilloma virus-positive cell lines after CD40L exposure. Constitutive expression of MCP-1, MCP-3, RANTES, and IFN-γ-inducible protein-10 was almost undetectable in all cell lines tested. CD40L was able to induce MCP-1 production; however, despite much higher CD40 expression in malignant cells, MCP-1 induction was significantly lower compared with nontumorigenic cells. After sensitization with IFN-γ, another T cell-derived cytokine showing minimal effects on CD40 expression levels, CD40 ligation led to a more than 20-fold MCP-1 induction in carcinoma cell lines. An even stronger effect was observed for IFN-γ-inducible protein-10. Our study highlights the synergism of T cell-derived mediators such as CD40L and IFN-γ for chemokine responses in cervical carcinoma cells, helping to understand the chemokine expression patterns observed in vivo.

Non-infectious ulcerative oral mucous membrane diseases are difficult to separate at first glance: they can appear as aphthous, bullous, lichenoid, drug-induced or toxic-irritative reactions. The overall considerations of history, localization of lesions, clinical and histological features, as well as direct and indirect immunofluorescence examination are required for the correct diagnosis. Some disorders start preferably at the oral mucosa, like pemphigus vulgaris and Adamantiades-Behcet disease, while others, such as cicatricial pemphigoid and habitual aphthosis generally are confined to the mucous membranes. This overview summarizes clinical and diagnostic features, differential diagnoses and current therapeutic possibilities of non-infectious inflammatory stomatopathies, which possess a specific position among skin diseases in distinction to infectious or neoplastic oral ulcers. This group of diseases includes aphthous lesions, lichen planus mucosae, lupus erythematosus, disorders with intraepidermal or subepidermal formation of blisters including pemphigus, bullous pemphigoid, erythema multiforme and variants as well as allergic or toxic contact stomatitis.

Relapsing ocular involvement is one of the major manifestations in Adamantiades-Behçet's disease (ABD). Combining systemic corticosteroids with cyclosporin A is currently the treatment of choice. Interferon-alpha (IFN-alpha) has proven to be effective in mucocutaneous ABD and has been reported to improve ocular lesions. We examined the longterm effects of IFN-alpha-2a in a case series of 45 patients with ocular involvement.Since 1988, 45 patients (79 eyes of 90 eyes) with ocular involvement in ABD have been treated with IFN-alpha (3 x 6-9 Mio IU per wk). In the initial acute phase of the disease, patients additionally received short-term corticosteroids (oral prednisolone 100 mg/day), tapered to a maintenance dose of 10 mg/day within 2 weeks. IFN-alpha-2a was administered as longterm therapy with a mean duration of 30 months (range 1.1-101 mo).IFN-alpha-2a/prednisolone treatment was effective against vasculitis, optic nerve neuropathy, and iritis. Sixty-four eyes had no recurrence under therapy. To date, recurrences have been seen in 26 eyes under IFN-alpha treatment. Flu-like symptoms were recorded in nearly all patients (n = 43). Further side effects were dose-dependent reversible thrombocytopenia (n = 1), psychosis (n = 3), depression (n = 13), thyroiditis (n = 1), and reversible diffuse alopecia (n = 7). In our series, 92% of all eyes showed stable or improved visual acuity in longterm followup.Longterm remission of ocular inflammation can be achieved with the combination of IFN-alpha and low-dose corticosteroids.

The oral cavity contains many organs and tissues compressed in a small area. Accordingly oral tumors have a wide variety of appearances. Reactive hyper-plastic lesions include epulis,morsicatio,traumatic ulcer or palatal hyperplasia. These benign lesions must be separated clinically and histologically from precancerous and neoplastic lesions. In leukoplakia,the individual risk can be estimated by clinical signs. Nevertheless histopathology is mandatory because precancerous lesions usually precede or accompany most oral cancers. Amalgam tattoo,oral nevi and melanoacanthoma have to be considered as differential diagnoses of oral melanoma. Accurate clinico-pathological diagnosis is mandatory to insure appropriate therapy. Oral soft tissue tumors such as Kaposi sarcoma and multiple mucosal neuromas in MEN 2b require interdisciplinary management. Diseases affecting the minor salivary glands which may be encountered by dermatologists include mucocele, necrotizing sialometa-plasia,and tumors such as pleomorphic adenoma.

Epidemiological studies demonstrate a prevalence of Adamantiades-Behcet’s disease (ABD) in the range of 0.12–420 per 100,000 inhabitants, with the highest rates in Istanbul, Turkey, and the lowest rates in the USA. Ophthalmological data on the prevalence of ocular involvement are limited for ABD in Germany, because most epidemiological studies are based on rheumatological or dermatological data. Berlin is the city with the highest number of non-native German inhabitants, and its multi-ethnic character renders it uniquely appropriate for epidemiological studies on ABD. We retrospectively analyzed all ABD patients seen in our department since 1982. All patients fulfilled the criteria of the International Study Group for Behcet’s Disease. We found 140 patients (63 female, 77 male), with a mean follow-up of 6.4 years (0.5–22 years). The mean age was 23 at the first manifestation and 32 when the full-blown disease was noted. The mean age at onset of ocular involvement was 30. Most of the patients were of Turkish (n = 73) or German (n = 34) origin. Fifty-six percent developed ocular involvement, which was the first manifestation in 8.6% and the second manifestation in 19.3% of cases. More than half the patients developed ocular involvement. The calculated prevalence of ocular involvement in ABD is 1.77/100,000 inhabitants for the municipality of Berlin.

Treatment of recurrent aphthous stomatitis (RAS) remains, to date, empirical and non-specific. The main goals of therapy are to minimize pain and functional disabilities as well as decrease inflammatory reactions and frequency of recurrences. Locally, symptomatically acting modalities are the standard treatment in simple cases of RAS. Examples include topical anaesthetics and analgesics, antiseptic and anti-phlogistic preparations, topical steroids as cream, paste or lotions, antacids like sucralfate, chemically stable tetracycline suspension, medicated toothpaste containing the enzymes amyloglucosidase and glucoseoxidase in addition to the well-known silver nitrate application. Dietary management supports the treatment. In more severe cases, topical therapies are again very useful in decreasing the healing time but fail to decrease the interval between attacks. Systemic immunomodulatory agents, like colchicine, pentoxifylline, prednisolone, dapsone, levamisol, thalidomide, azathioprine, methotrexate, cyclosporin A, interferon alpha and tumour necrosis factor (TNF) antagonists, are helpful in resistant cases of major RAS or aphthosis with systemic involvement.

Adamantiades-Behcet’s disease (ABD) is a multi-system disorder with recurrent oral and/or genital ulcerations, skin lesions and ocular involvement. Eye involvement is a common manifestation that affects the patients’ quality of life more than any other. Left untreated, it leads to blindness and often to loss of the eye through secondary complications like phthisis or painful glaucoma. In some cases, such as tractional retinal detachment, secondary glaucoma or secondary cataract formation, intraocular surgery is required. A prospective study of patients with ocular ABD who underwent intraocular surgery under systemic treatment with interferon-alpha was conducted. From 1999 to 2005, we treated eight eyes from five patients with pars plana vitrectomy (n = 1), phacoemulsification (n = 6) and trabeculectomy (n = 1). The mean follow-up was 49 months (range 5–94 months). Seven out of eight eyes had better visual acuity following surgery. One eye did not benefit from cataract surgery because of optic nerve atrophy. Only one eye showed prolonged inflammation following phacoemulsification. None of the patients showed a recurrence during follow-up. The failure of conservative glaucoma treatment necessitated trabeculectomy in one eye; the patient has had no further recurrence for 6 years under monotherapy with interferon-alpha. Interferon-alpha is a potent therapy for Adamantiades-Behcet’s disease with ocular involvement. It also provides a basis for safe and reliable surgical interventions. In seven of eight eyes, there was no intra- or peri-operative recurrence of inflammation, which is a common complication after these procedures. Only one eye showed acute and prolonged inflammation following cataract surgery.