Successful full clinical remission of bullous pemphigoid with intravenous pulse methylprednisolone and cyclophosphamide: a case report

We report a case of a 73 year old man with generalized bullous pemphigoid (BP). Oral steroid therapy was contr-indicated due to his personal medical history. The patient was treated with intravenous methylprednisolone pulse therapy (I.M.P.T.) followed by oral cyclophosphamide with satisfactory results. The therapeutic challenges of BP are being discussed with emphasis given on intravenous methylprednisolone pulse therap

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

Analysis of dermoscopic findings of basal cell carcinomas (BCCs) and their contribution in the diagnostic accuracy

Background: There is limited data on dermoscopic features of basal cell carcinomas (BCCs). We evaluated the presence of dermoscopic features in superficial (sBCCs), nodular (nBCCs), pigmented and non-pigmented BCCs in order to evaluate the role of dermoscopy in the diagnosis of different subtypes of BCCs. Methods: We conducted a retrospective study to evaluate the presence of dermoscopic features in superficial, nodular, pigmented and non-pigmented BCCs. One hundred thirty eight lesions (42 superficial, 96 nodular, 102 pigmented and 36 non-pigmented) were assessed by dermoscopy. Results: The most significant features in all categories, were a scattered vascular pattern, featureless areas, atypical red vessels, arborizing vessels, comma vessels, background of white-red structureless areas and telangiectasias. Haemorrhage-ulceration, hypopigmented areas and blue-grey ovoid nests were all more likely to be observed in sBCCs, than in nBCCs (p<0.0001). Arborizing and atypical red vessels in addition to featureless areas, were more frequent in nodular than in sBCCs (p<0.0001). Telangectasias, white-red structureless areas, red dots and red globules were more common in non-pigmented than in pigmented BCCs (p<0.0001). In addition, a significant difference of arborizing vessels was detected in pigmented lesions in comparison to non-pigmented (p<0.0001). Conclusions: Dermoscopic hallmarks of all BCCs may be the scattered atypical vessels, featureless areas and the white-red structureless background. Superficial BCCs are also characterized by comma vessels, haemorrhage, small ulcerations, hypopigmented areas, telangiectasias and blue-gray ovoid nests while nBCCs by arborizing vessels. Dermoscopy was found to be a valuable tool for the diagnosis of specific subtypes of BCCs.Το βασικοκυτταρικό καρκίνωμα (ΒΚΚ) είναι η συχνότερη μορφή δερματικού καρκίνου. Είναι συχνή πάθηση, εμφανιζόμενη σε ηλικία, συνήθως άνω των 40 ετών. Εντοπίζεται σχεδόν αποκλειστικά σε έντριχα, φωτοεκτεθειμένα μέρη, κυρίως πρόσωπο, ώτα, λαιμό καταδεικνύοντας τη σχέση εμφάνισής του με την αθροιστική δράση της ηλιακής ακτινοβολίας στα εκτεθειμένα μέρη του σώματος, σε άτομα με ανοιχτόχρωμο δέρμα. Το ΒΚΚ εμφανίζεται συνήθως ως μονήρης βλάβη. Σε ποσοστό περίπου 40%, άτομα που είχαν ένα ΒΚΚ, θα εμφανίσουν στην επόμενη δεκαετία μία ή περισσότερες νέες βλάβες. Το ΒΚΚ μεθίσταται πολύ σπάνια, παρόλα αυτά, χωρίς θεραπευτική αντιμετώπιση, ο όγκος αυτός μπορεί να εξελιχθεί σε ιδιαίτερα τοπικά καταστρεπτικό και διεισδυτικό όγκο του δέρματος. Το βασικοκυτταρικό καρκίνωμα ανήκει στο ευρύ φάσμα των κακοηθών επιδερμιδικών νεοπλασιών ή των μη μελανινοκυτταρικών όγκων. Η διάγνωσή του βασίζεται στην κλινική εξέταση, το ιστορικό του ασθενούς και την ιστοπαθολογική μελέτη. Τα τελευταία χρόνια αναπτύχθηκε μια νέα τεχνική αναίμακτη και εύκολη η οποία φαίνεται να συμβάλλει σημαντικά στην έγκαιρη διάγνωση κακοήθων βλαβών δέρματος: η δερματοσκόπηση. Η εξέταση δηλαδή με μικροσκόπιο επιφάνειας δέρματος, η οποία προσφέρει ένα είδος ενδιάμεσης εικόνας μεταξύ κλασσικής κλινικής εικόνας και ιστολογικής. Η μέθοδος αυτή επιτρέπει την υπομακροσκοπική εκτίμηση των αλλοιώσεων του δέρματος, από την επιδερμίδα έως το χόριο. Στην παρούσα μελέτη έγινε μια προσπάθεια να συνεκτιμηθούν παλιά με καινούργια δεδομένα, να αναλυθούν τα δερματοσκοπικά ευρήματα των ΒΚΚ. και να αξιολογηθεί η χρησιμότητα της μεθόδου της δερματοσκόπησης στην διάγνωση και παρακολούθηση των ΒΚΚ, με απώτερο σκοπό να χρησιμοποιηθεί ως εξέταση ρουτίνας για τη διάγνωση αυτών των όγκων. Μελετήθηκαν συνολικά 138 βλάβες ΒΚΚ, σε 96 ασθενείς, οι οποίοι επισκέφτηκαν το νοσοκομείο μας στο χρονικό διάστημα μεταξύ του Ιανουάριου 2007 έως και τον Δεκέμβριο 2008 και αυτές αξιολογήθηκαν δερματοσκοπικά (42 επιφανειακά, 96 οζώδη, 102 μελαγχρωματικά και 36 μη-μελαγχρωματικά ΒΚΚ). Η αξιολόγηση των βλαβών διενεργήθηκε με ψηφιακό δερματοσκόπιο (Mole Max II, 30 φορές μεγέθυνση). Όλα τα δερματοσκοπικά κριτήρια αναλύθηκαν σε σχέση με την ύπαρξη ή την απουσία οζώδους βλάβης και μελάγχρωσης με σκοπό να εξεταστεί κατά πόσον υπήρχε σημαντικώς στατιστική διαφορά μεταξύ των κατηγοριών οζώδη - επιφανειακά ΒΚΚ, και μελαγχρωματικά - μη μελαγχρωματικά ΒΚΚ. Για τον σκοπό αυτό εφαρμόστηκε η στατιστική δοκιμασία x² για τετράπτυχο πίνακα (Fisher’s exact test). Τα σημαντικότερα χαρακτηριστικά σε όλες τις κατηγορίες, ήταν το διάσπαρτο αγγειακό πρότυπο, οι περιοχές άνευ ειδικών κριτηρίων, τα άτυπα ερυθρά αγγεία, τα διακλαδιζόμενα αγγεία, τα αγγεία με μορφή κόμματος, το λευκο-ρόδινο υπόστρωμα και οι τηλεαγγειεκτασίες. Αιμορραγία, εξέλκωση, περιοχές υπομελάγχρωσης και γκρι-μπλε ωοειδές φωλιές παρατηρήθηκαν πιο συχνά στα επιφανειακώς επεκτεινόμενα ΒΚΚ , σε σχέση με τα οζώδη (p<0,0001). Διακλαδιζόμενα και άτυπα αγγεία επιπρόσθετα με περιοχές άνευ ειδικών δερματοσκοπικών κριτηρίων, καταγράφηκαν πιο συχνά στα οζώδη ΒΚΚ (p<0,0001). Τηλεαγγειεκτασίες, λευκό-ρόδινο άνευ ειδικών δερματοσκοπικών δομών υπόστρωμα, στικτά αγγεία και ερυθρά σφαιρίδια ήταν πιο συχνά σε μη μελαγχρωματικά από ό, τι σε μελαγχρωματικά ΒΚΚ (p<0,0001). Επιπλέον, διακλαδιζόμενα αγγεία με στατιστικώς σημαντική διαφορά ανιχνεύθηκαν στις μελαγχρωματικές βλάβες όταν αυτές συγκρίθηκαν με μη-μελαγχρωματικές (p<0,0001). Η καθιέρωση των συγκεκριμένων δερματοσκοπικών κριτηρίων (δερματοσκοπικού αλγόριθμου) στα οποία καταλήξαμε, είναι ουσιαστικής σημασίας για την έγκαιρη και ακριβή διάγνωση διαφόρων υποτύπων ΒΚΚ. Ιδιαίτερα σημαντικό συμπέρασμα από τη μελέτη μας, είναι η ορθή κατάταξη των επιμέρους υποτύπων του ΒΚΚ, ώστε αυτή να οδηγήσει σε αποφυγή περιττών ιατρικών πράξεων, χειρουργικών επεμβάσεων, αποφυγή λανθασμένης κατηγοριοποίησης η οποία μπορεί να οδηγήσει σε αυξημένη νοσηρότητα και υποτροπές. Με τον τρόπο αυτό αντιμετωπίζεται ο ασθενής με ΒΚΚ όσο το δυνατόν ορθότερα. Στα πλαίσια αυτά, η κλινική εικόνα σε συνδυασμό με τον δερματοσκοπικό αλγόριθμο που περιγράφηκε στη παρούσα μελέτη, μπορούν να ενισχύσουν τη διαγνωστική ακρίβεια του σύγχρονου δερματολόγου

Eccrine Poroma: Pathogenesis, New Diagnostic Tools and Association with Porocarcinoma—A Review

Eccrine poroma (EP) is a relatively rare benign adnexal neoplasm that usually affects elderly patients. Its pathogenesis is still under investigation, but recent gene studies have revealed gene fusions as key incidences resulting in oncogenetic pathways. It often presents as a solitary, firm papule, mostly asymptomatic, located on the soles or palms. Due to its clinical and dermoscopic variability, it is characterized as the great imitator. We performed a literature review, aiming to summarize current data on the pathogenetic mechanisms, new dermoscopic features, and novel diagnostic tools that may aid in early diagnosis and proper management of this rare adnexal tumor. Furthermore, we reviewed the possible pathogenetic associations between EP and its malignant counterpart, namely eccrine porocarcinoma. This systematic approach may aid in understanding the pathogenetic mechanisms and how to use novel histopathologic markers and imaging methods to overcome the diagnostic dilemma of this rare tumor

The efficacy and tolerability of tetracyclines and clindamycin plus rifampicin for the treatment of hidradenitis suppurativa : Results of a prospective European cohort study

Background: Tetracyclines and clindamycin plus rifampicin combination therapy are both considered first-line therapy in current hidradenitis suppurativa guidelines. However, evidence for their efficacy is drawn from small studies, often without validated outcomes. Objective: To assess the 12-week efficacy of oral tetracyclines and a combination of clindamycin and rifampicin. Methods: A prospective, international cohort study performed between October 2018 and August 2019. Results: In total, 63.6% of the included 283 patients received oral tetracyclines, and 36.4% were treated with clindamycin and rifampicin. Both groups showed a significant decrease in International Hidradenitis Suppurativa Severity Score System from baseline (both P <.001). The Hidradenitis Suppurativa Clinical Response (HiSCR) was achieved in 40.1% and 48.2% of patients, respectively (P =.26). Patient characteristics or disease severity were not associated with the attainment of HiSCR or the minimal clinically important differences for the Dermatology Life Quality Index and pain. Limitations: Cohort study. Respectively, 23.9% and 19.4% of patients had to be excluded from the HiSCR analysis for the tetracycline and combination therapy group because of a low abscess and nodule count at baseline. Conclusion: This study shows significant efficacy of both tetracycline treatment and clindamycin and rifampicin combination therapy after 12 weeks in patients with hidradenitis suppurativa. No significant differences in efficacy were observed between the 2 treatments, regardless of disease severity

The efficacy and tolerability of tetracyclines and clindamycin plus rifampicin for the treatment of hidradenitis suppurativa: Results of a prospective European cohort study

Background: Tetracyclines and clindamycin plus rifampicin combination therapy are both considered first-line therapy in current hidradenitis suppurativa guidelines. However, evidence for their efficacy is drawn from small studies, often without validated outcomes. Objective: To assess the 12-week efficacy of oral tetracyclines and a combination of clindamycin and rifampicin. Methods: A prospective, international cohort study performed between October 2018 and August 2019. Results: In total, 63.6% of the included 283 patients received oral tetracyclines, and 36.4% were treated with clindamycin and rifampicin. Both groups showed a significant decrease in International Hidradenitis Suppurativa Severity Score System from baseline (both P &lt; .001). The Hidradenitis Suppurativa Clinical Response (HiSCR) was achieved in 40.1% and 48.2% of patients, respectively (P = .26). Patient characteristics or disease severity were not associated with the attainment of HiSCR or the minimal clinically important differences for the Dermatology Life Quality Index and pain. Limitations: Cohort study. Respectively, 23.9% and 19.4% of patients had to be excluded from the HiSCR analysis for the tetracycline and combination therapy group because of a low abscess and nodule count at baseline. Conclusion: This study shows significant efficacy of both tetracycline treatment and clindamycin and rifampicin combination therapy after 12 weeks in patients with hidradenitis suppurativa. No significant differences in efficacy were observed between the 2 treatments, regardless of disease severity

The efficacy and tolerability of tetracyclines and clindamycin plus rifampicin for the treatment of hidradenitis suppurativa; results of a prospective European cohort study.

Tetracyclines and clindamycin plus rifampicin combination therapy are both considered first-line therapy in current Hidradenitis Suppurativa (HS) guidelines. However, evidence for their efficacy is drawn from small studies, often without validated outcomes.info:eu-repo/semantics/publishe

External Validation of the IHS4-55 in a European Antibiotic-Treated Hidradenitis Suppurativa Cohort

Background: Previously, a new dichotomous outcome was developed, calculated as 55% reduction in the International Hidradenitis Suppurativa 4 (IHS4-55) score. It was validated in datasets of adalimumab and placebo-treated HS patients. External validation is an important aspect of clinical outcomes. Objectives: We aimed to externally validate the novel dichotomous IHS4-55 in a non-biologic treated dataset of HS patients. Methods: Data from a previously published European-wide prospective clinical study of antibiotic treatment of HS patients were used to assess the association of IHS4-55 achievement with individual reduction in inflammatory nodules, abscesses, and draining tunnels. Moreover, the associations between IHS4-55 positivity and achievement of the minimal clinically important differences (MCIDs) for Dermatology Life Quality Index (DLQI), Numerical Rating Scale (NRS) Pain, and NRS Pruritus were analyzed. Results: Data were obtained from 283 individual patients, of which 36.4% (103/283) were treated with clindamycin and rifampicin and 63.6% (180/283) with tetracyclines for 12 weeks. Achievers of the IHS4-55 demonstrated a significant reduction the counts of inflammatory nodules, abscesses, and draining tunnels (all p &lt; 0.001). Additionally, IHS4-55 achievers had an odds ratio for achieving the MCID of DLQI, NRS Pain, and NRS Pruritus of 2.16 (95% CI 1.28-3.65, p &lt; 0.01), 1.79 (95% CI 1.10-2.91, p &lt; 0.05), and 1.95 (95% CI 1.18-3.22, p &lt; 0.01), respectively. Conclusions: This study shows the external validity of the novel IHS4-55 by demonstrating a significant association between IHS4-55 achievement and a reduction in inflammatory lesion counts as well as achievement of MCIDs for DLQI, NRS Pain, and NRS Pruritus in an antibiotic-treated cohort. These findings support the use of the IHS4-55 as a novel primary outcome measure in clinical trials.</p