Adamantiades-Behçet disease: Between dermatology and ophthalmology.

Adamantiades-Behçet disease is an inflammatory, vascular disease of unknown etiology. The disease is named after two physicians, Benediktos Adamantiades and Hulȗsi Behçet, who both made significant contributions to the study of the disease. It was probably first described by Hippocrates in 500 BCE. Adamantiades-Behçet disease is most common in the region encompassing the ancient trade route known as the Silk Road. In Turkey, the disease is estimated to affect 80 to 370 people per 100,000 inhabitants, and it is also the country with the highest incidence rate. The frequency of the disease associated with the clinical picture differs from the origin of the onset. The disease is characterized by recurrent aphthous ulcers of the mouth, genitals, skin lesions, and eye lesions. The disease process can also involve other organs, including the joints, nervous system, large vessels, heart, and gastrointestinal tract. Aphthous oral ulcers appear as the first harbinger of the disease and affect almost all patients (97%-99%). The scientific interest in Adamantiades-Behçet disease has increased exponentially in the past decade.

Documentation of psoriasis in routine care - expert consensus on a German data set.

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.

[Persistent, unilateral eczema of the nipple in a man]. / Persistierendes unilaterales Ekzem der Mamille bei einem Mann.

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.

[Enlarged mediastinal lymph nodes of a patient with malignant melanoma stage IV under pembrolizumab treatment]. / Mediastinale Lymphknotenvergrößerung bei einer Patientin mit malignem Melanom Stadium IV unter Therapie mit Pembrolizumab.

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) and male infertility.

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.

Schistosomiasis (Bilharziasis) ova: An incidental finding in testicular tissue of an obstructive azoospermic man.

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.

Multiple HPV-Induced Squamous Cell Carcinomas on the Fingers of a Patient with Systemic Lupus Erythematosus: A Case and Review.

Human papilloma virus (HPV) infection is documented to be involved in the development of epithelial malignancies, mostly in cervical cancer. Systemic lupus erythematosus (SLE) patients have an increased prevalence of such an infection. We report the case of a 55-year-old female SLE patient who developed multiple in situ squamous cell carcinomas on her fingers, after chronic HPV infection. HPV-33 DNA was isolated from the lesions. The purpose of this case presentation is to raise awareness about HPV-induced malignancies for this high-risk group and propose an early HPV vaccination to efficiently prevent such comorbidities.

Gender-specific differences in Adamantiades-Behçet's disease manifestations: an analysis of the German registry and meta-analysis of data from the literature.

OBJECTIVE: We investigated the effect of gender on the clinical Adamantiades-Behçet'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.

The treatment of chronic recurrent oral aphthous ulcers.

BACKGROUND: Chronic recurrent oral aphthous ulcers are the most common type of inflammatory efflorescence of the oral mucosa, with a prevalence of 2% to 10% in Caucasian populations. To treat them properly, physicians should know their clinical appearance and course, conditioning factors, underlying causes, and differential diagnosis. METHOD: This review is based on pertinent articles that were retrieved by a selective search in PubMed and in the Cochrane Central Register of Controlled Trials. RESULTS: Hard, acidic, and salty foods and toothpastes containing sodium lauryl sulfate should be avoided, along with alcohol and carbonated drinks. In Germany, the only drugs that have been approved to treat oral aphthous ulcers are corticosteroids, topical antiseptic/anti-inflammatory agents such as triclosan and diclofenac, and local anesthetics such as lidocaine. Antiseptic agents and local anesthetics should be tried first; if these are ineffective, topical cortico steroids should be used. In severe cases, local measures can be combined with systemic drugs, e.g., colchicine, pentoxifylline, or prednisolone. The efficacy of systemic treatment is debated. Other immunosuppressive agents should be given systemically only for refractory or particularly severe oral aphthous ulcers due to Adamantiades-Behçet disease. CONCLUSION: The treatment of chronic recurrent oral aphthous ulcers is symptomatic, mainly with topically applied agents. It is tailored to the severity of the problem in the individual case, i.e., the frequency of ulcers, the intensity of pain, and the responsiveness of the lesions to treatment. Effective treatment relieves pain, lessens functional impairment, and lowers the frequency and severity of recurrences.

Non-infectious ulcerating oral mucous membrane diseases.

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-Behçet 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.

Ocular involvement in Adamantiades-Behçet's disease in Berlin, Germany.

BACKGROUND: Epidemiological studies demonstrate a prevalence of Adamantiades-Behçet'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. METHODS: We retrospectively analyzed all ABD patients seen in our department since 1982. All patients fulfilled the criteria of the International Study Group for Behçet's Disease. We found 140 patients (63 female, 77 male), with a mean follow-up of 6.4 years (0.5-22 years). RESULTS: 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. CONCLUSIONS: 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.

Medium-dose prednisolone pulse therapy in alopecia areata.

BACKGROUND: 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. OBJECTIVE: Evaluation of efficacy and saftety of a short-term medium-dose pulse prednisolone treatment in alopecia areata. RESULTS: 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. METHODS: 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. CONCLUSION: 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.

Longterm visual prognosis of patients with ocular Adamantiades-Behçet's disease treated with interferon-alpha-2a.

OBJECTIVE: 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. METHODS: 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). RESULTS: 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. CONCLUSION: Longterm remission of ocular inflammation can be achieved with the combination of IFN-alpha and low-dose corticosteroids.