Interstitial Granulomatous Dermatitis: Another Clinical Variant

A 70-year-old female patient presented with an eruption consisting of symmetrically distributed erythematous papules around the umbilicus 1 month after the cessation of adalimumab for the treatment of rheumatoid arthritis. Biopsy of a papule showed an interstitial granulomatous infiltrate in the dermis, without deposition of mucin. The lesions cleared only after re-initiation of treatment 2 months later. Interstitial granulomatous dermatitis is thought to be a distinct histopathological pattern, either drug induced or associated with rheumatoid arthritis or autoimmune collagen diseases. In our case, there was a distinct clinical presentation of interstitial granulomatous dermatitis, composed of symmetrically distributed indurated papules around the umbilicus as well as a mild granulomatous reaction pattern

Detection of IgG autoantibodies against desmocollin–3 in Greek patients with pemphigus

Pemphigus is an autoimmune bullous disorder caused by autoantibodies against desmosomal cadherins. The most common clinical forms are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Among the numerous proteins that are considered responsible for the cohesion of keratinocytes in epidermis, desmocollin-3 (Dsc-3) has been initially reported to participate in epidermal blistering in mice. There have been reports in which autoantibodies against Dsc-3 have been detected. In PV, a limited number of studies found no presence of IgG or IgA autoantibodies against Dsc-3. In this study we examined sera from Greek patients with PV and PF for the presence of IgG autoantibodies against Dsc-3. Immunoblotting for the detection of autoantibodies against Dsc-3 was performed in sera from all cases. Dsc-3 autoantibodies were not detected in either group (PV and PF). Our results confirm the hypothesis that the pathogenic role of Dsc-3 in epidermal blistering in PV and PF remains controversial. </p

Spectrum of autoimmune bullous diseases in northern Greece. A 4-year retrospective study and review of the literature

Bullous Diseases Unit at the 2nd Department of Dermatology and Venereology, Aristotle University of Thessaloniki was founded with the aim to provide the optimal diagnostic approach and treatment of patients with autoimmune bullous diseases (AΙBD).  We processed all AIBD files of patients diagnosed from 2011 to 2014 in order to record all epidemiological data and therapeutic manipulations during monitoring. 57 patients were diagnosed with intraepidermal and 62 with subepidermal bullous diseases. There were 51 cases (89%) of pemphigus vulgaris (PV) and 6 (11%) of pemphigus foliaceus (PF), whereas 45 (73%) patients were diagnosed with bullous pemphigoid (BP), 9 (14%) with mucous membrane pemphigoid (MMP), 3 (5%) with pemphigoid gestationis (PG), 3 (5%) with linear IgA dermatosis (LAD), 1 (2%) with epidermolysis bullosa aquisita (EBA), and 1 patient with an undefined subepidermal AIBD. The mean age of patients within the pemphigus spectrum was 57 years. In the pemphigoid spectrum, the mean age was 72 years. Comorbidities were reported with increasing frequency, as well as treatment options other than systemic corticosteroids, such as adjuvant immunosuppressive agents, which were used to achieve complete remission. This is a report from a tertiary AIBD Referral Center in northern Greece. Our data from a 4-year period contribute to the completion of the global geographic incidence map of AIBD.  </p

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).

BACKGROUND Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. OBJECTIVES These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. RESULTS Chronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. CONCLUSIONS These are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies

Detection of autoantibodies against BP180 and BP230 antigens as a method for diagnosis and follow up of patients with bullous pemphigoid

The aim of this study is to investigate the possible relation of circulating autoantibodies against BP180 and BP230 with the activity of the disease and to evaluate the usefulness of the immunoenzymic method ELISA for diagnosis and follow up of patients with BP. Forty patients with newly diagnosed BP were enrolled. Fifteen healthy volunteers and fifteen patients with pemphigur vulgaris served blister fluids of other etiology were measured also. In our study, detection of anti BP180 and anti BP230 autoantibodies in serum and in blister fluid proved to be a sensitive diagnostic method. The most important conclusion is that BP180 ELISA could be a method with prognostic value, able to contribute significantly to the follow up of patients with bullous pemphigoid.Η μελέτη αυτή αποσκοπεί στη διερεύνηση της σχέσης των κυκλοφορούντων αυτοαντισωμάτων έναντι των αντιγόνων ΒΡ180 και ΒΡ230 του πομφολογώδους πεμφιγοειδούς(ΠΠ) με τη δραστηριότητα της νόσου και στην αξιολόγηση της χρησιμότητας της ανοσοενζυμικής μεθόδου ELISA στη διάγνωση και παρακολούθηση του ΠΠ. Μελετήθηκαν συνολικά 40 ασθενείς με πρωτοεμφανιζόμενο ΠΠ, 15 υγιείς μάρτυρες και 15 ασθενείς με κοινή πέμφιγα. Επίσης μελετήθηκε υγρό από 13 πομφόλυγες ασθενών με ΠΠ και από 7 πομφόλυγες άλλης αιτιολογίας. Από τα αποτελέσματα των μετρήσεων, πιστοποιείται η ευαισθησία και η χρησιμότητα της ανίχνευσης των κυκλοφορούντων αυτοαντισωμάτων με ELISA στη διάγνωση του ΠΠ, υπογραμμίζεται η σημασία του ελέγχου του υγρού πομφόλυγας με την ίδια μέθοδο και επισημαίνεται η προγνωστική αξία της μεθόδου. Με την παρακολούθηση των επιπέδων των κυκλοφορούντων αυτοανοσημάτων μπορούμε να ελέγξουμε τις υποτροπές της νόσου, δυνατότητα που δεν μας παρέχουν οι έως τώρα εφαρμοζόμενες διαγνωστικές τεχνικές

Serum Levels of TNF-, IL-12/23 p40, and IL-17 in Psoriatic Patients with and without Nail Psoriasis: A Cross-Sectional Study

Nail involvement has started playing a major role in the overall assessment and management of psoriatic disease. Biologics indicated for moderate to severe chronic plaque psoriasis are shown to be beneficial in nail disease. This study aimed to assess and compare the serum levels of TNF-, IL-12/23 p40, and IL-17 in psoriatic patients with and without nail involvement. 52 consecutively selected patients with chronic plaque psoriasis were included in this cross-sectional study. Patients were studied and analyzed after they had been divided into 2 groups regarding the presence (n = 24) or not (n = 28) of nail psoriasis. The mean serum levels of TNF-were significantly higher in the group of psoriatic patients with nail lesions compared to those without (t-test; 5.40 ± 1.17 versus 3.80 ± 1.63, P = 0.026). However, the median serum levels of both IL-12/23 p40 , P = 0.297) and ), P = 0.714) did not significantly differ between the 2 groups. These results confirm the important role of TNF-in the pathogenesis of nail psoriasis and may suggest that anti-TNF agents could be more beneficial in psoriatic nail disease than agents targeting IL-12/23 p40 or IL-17 and its receptors

Association of Autoantibodies to BP180 with Disease Activity in Greek Patients with Bullous Pemphigoid

39 bullous pemphigoid (BP) patients were studied to assess the clinical significance of anti-BP180 and anti-BP230 circulating autoantibodies of BP and correlate their titers with the clinical scores of the BP Disease Area Index (BPDAI) and the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) as well as with the intensity of pruritus measured by the BPDAI pruritus component. All parameters were evaluated by the time of diagnosis (baseline), month 3, and month 6. Titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (, ) and ABSIS (, ) values, as well as with BPDAI component for the intensity of pruritus (, ) at baseline. At month 3, titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (, ) and ABSIS (, ) values, as well as with the BPDAI component for the intensity of pruritus (, ). At month 6, titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (, ) and ABSIS (, ) values, as well as with the BPDAI component for the intensity of pruritus (, ). There was no statistically significant correlation between titers of anti-BP230 autoantibodies and the BPDAI, ABSIS, and BPDAI component for the intensity of pruritus at the same time points

Serum Levels of TNF-α, IL-12/23p40, and IL-17 in Plaque Psoriasis and Their Correlation with Disease Severity

A case-control study was performed to assess the serum levels of TNF-α, IL-12/23p40, and IL-17 in patients with plaque psoriasis, compare them with healthy controls, and correlate them with disease severity, as represented by Psoriasis Area Severity Index (PASI). 32 consecutively selected, untreated patients with active, chronic plaque psoriasis were recruited and compared to 32 age- and sex-matched healthy controls. Serum cytokine levels were determined by solid phase sandwich enzyme linked immunosorbent assay (R&D Systems Europe, Ltd.). The mean serum levels of TNF-α were significantly higher in psoriatic patients compared to those of controls (Mann-Whitney U test; P=0.000). However, the median serum levels of neither IL-12/23p40 nor IL-17 differ significantly between the 2 groups (Mann-Whitney U test; P=0.968 and P=0.311, resp.). No significant correlations were found between PASI and any of the cytokine serum levels (Spearman’s rank test; P>0.05). Despite the well-evidenced therapeutic efficacy of biologic agents targeting TNF-α, IL-12/23p40, and IL-17, serum levels of TNF-α, IL-12/23p40, and IL-17 do not seem to correlate with the severity of psoriatic skin disease in untreated patients, as represented by PASI. Further investigation may add more data on the pathogenetic cascade of psoriasis