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Introduction: Immunomodulation using TNF-α inhibitors (anti-TNF-a), especially adalimumab, is highly effective in the treatment of hidradenitis suppurativa (HS) in cases that are poorly controlled by conventional treatments. However, paradoxical psoriasis represents a peculiar type of psoriasis that may occur de novo or as the worsening of pre-existent psoriatic lesions during treatment with adalimumab. Case Presentation: We reported 4 cases of patients suffering from HS, who developed paradoxical psoriasis after treatment with adalimumab for their HS, analyzing their clinical and epidemiological characteristics. All 4 cases were middle aged, smokers, overweight or obese. Half of the patients were males (50%). All of them were classified as Hurley stage III, with a mean duration of HS of 20 years. Two patients had a family history of psoriasis. All 4 patients had been on at least 5 months of successful treatment with adalimumab before the onset of the lesions. Conclusions: Paradoxical psoriasis emerged in 4 patients who received at least 5-month regimen of adalimumab for long-lasting HS. Although different mechanisms have been hypothesized for such events, the exact underlying pathogenetic pathway remains unclear. Consistent reporting of such rare cases, and on a larger scale, is encouraged in order to enrich the available literature.

Background: Previously, a new dichotomous outcome was developed, calculated as 55% reduction in the International Hidradenitis Suppurativa 4 score (IHS4-55). 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 was 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 (MCID) for Dermatology Life Quality Index (DLQI), numerical rating scale (NRS) Pain, and NRS Pruritus were analyzed. Results: Data was 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

BACKGROUND Recent trends in the sensitization of construction workers show a decrease in potassium dichromate and an increase in epoxy resin sensitization. OBJECTIVES To present the trends of occupational contact allergy of construction workers in Greece from 2009 to 2018. METHODS We retrospectively reviewed the files of patients with eczema patch tested in our Contact Dermatitis Clinic who were construction workers. RESULTS A total of 191 construction workers initially reported contact dermatitis. Of these, 138 had occupation-relevant allergic contact dermatitis (ACD) or irritant contact dermatitis (ICD). All patients were men. After being patch tested, 98 (71.0%) were diagnosed with ACD and 40 with ICD. Median duration of occupation till onset of ACD was 2 years (interquartile range [IQR] 0.8-7). The hands were the most common location for ACD (73.5%), followed by the trunk (39.8%), the legs (38.8%), and the face (11.2%). Of the patients, 74.6% had lesions affecting multiple body sites. Potassium dichromate (67%) was the most frequent allergen, followed by thiuram mix (37.4%) and cobalt chloride (31.8%). Sensitization to epoxy resins was lower (12.1%). CONCLUSION The sensitization pattern of Greek construction workers does not follow the trends in Central or Northern Europe, rather sharing attributes with less industrialized countries.

BACKGROUND: Hidradenitis suppurativa (HS), a chronic, recurrent, debilitating skin disease, is characterized by painful, inflammatory, subcutaneous lesions of the axilla, inguinal and anogenital regions. Overall prevalence of HS is ˜1%, and the impact of disease on patient quality of life (QoL) and healthcare resource utilization (HRU) is high. OBJECTIVES: To estimate the real-world effectiveness of adalimumab (Humira®) treatment in patients with moderate-to-severe HS on disease severity, pain, QoL, work productivity and HRU. METHODS: HARMONY (Effectiveness of Adalimumab in Moderate to Severe HidrAdenitis SuppuRativa Patients - a Multi-cOuNtry studY in Real Life Setting) is a multicentre, postmarketing observational study in adult patients with moderate-to-severe HS. Disease severity and QoL parameters were evaluated using validated measures at 12-week intervals over 52 weeks of treatment. The primary endpoint was the proportion of patients achieving a Hidradenitis Suppurativa Clinical Response (HiSCR: ≥50% reduction in abscess and inflammatory nodule count, with no increase in abscess and draining fistula counts relative to baseline) at 12 weeks. Secondary endpoints were HiSCR at 24, 36 and 52 weeks and changes in QoL parameters and work productivity assessments. Analyses were conducted using as-observed data. RESULTS: The proportion of patients reaching the primary HiSCR endpoint was 70.2% (n = 132/188 enrolled) and remained ≥70% until study completion. There were statistically significant (P < 0.0001) reductions in worst and average skin pain. All of the QoL measures evaluated improved significantly (P < 0.0001) by 12 weeks of adalimumab treatment, as did work productivity assessments (P < 0.05), and there was a ˜50% decrease in HRU between baseline and week 52. Adalimumab was well tolerated. CONCLUSIONS: In this real-world setting, adalimumab treatment of moderate-to-severe HS resulted in decreased disease severity and improvements in QoL and productivity. Response to adalimumab was rapid (within 12 weeks) and sustained (52 weeks). No unexpected safety signals were reported.

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Background: Hidradenitis suppurativa (HS) is a chronic, recurrent, auto-inflammatory disease that affects the pilosebaceous unit in apocrine gland-bearing areas. Bacteria are thought to play a role in the development and progression of disease. In addition, antibiotics are frequently used as first-line management for HS. We sought to determine the carriage status of Staphylococcus aureus and its resistance to antibiotics among patients with HS in a tertiary referral hospital in Athens, Greece. Methods: In this observational cohort study, 68 consecutive patients attending the HS clinic of “Attikon” General University Hospital in Athens, Greece, during a 9-month period were enrolled. All patients had not received any antibiotic therapy for any reason during the previous 3 months before enrollment. Nasal and oropharyngeal samplingwere obtained, and specimens were tested for the presence of S. aureus.Antibiotic susceptibility testing was performed using the VITEK 2 system. Standard statistical tests, descriptive statistics tests, and χ2 and Pearson correlation tests were performed, using IBM SPSS Statistics 25.The level of significance was set at a pvalue Results: Sixty-eight patients with HS were studied. There were 44 females (64.7%) and 24 males (35.3%). The mean age was 36.63 ± 13.0 (IQR = 21), and the mean age at onset of disease was 23.90 ± 11.53 (IQR = 14). The mean duration of disease was 12.74 ± 10.20 years (IQR = 15). Fifteen (22.1%) of the patients were Hurley stage I, 22 (32.4%) were Hurley stage II, and 31 (45.6%) were Hurley stage III. S. aureus carriage was detected in 17 patients (25%). Six of them (35.3%) had MRSA strains. There was an increased prevalence of S. aureus colonization (p = 0.058) and MRSA (p = 0.101) in Hurley stage III patients, but this result was not statistically significant. Conclusions: We found a 25% prevalence of S. aureus colonization (17/68 patients) and a 35.3% prevalence of MRSA (6/17) among our HS patients. There was an increased prevalence of S. aureusand MRSA positivity in HS patients with Hurley stage III. Further studies are needed to clarify the possible clinical significance of S. aureus carriage in the disease development and progression as well as in the treatment outcome.

Introduction: Hidradenitis suppurativa (HS) is a chronic inflammatory skin disorder of the follicular epithelium. The aim of the study was to investigate the effectiveness of colchicine on the clinical outcomes of HS patients, and to evaluate wither colchicine as monotherapy or in combination with doxycycline would provide better outcomes. Methods: A retrospective study was conducted including 44 patients with established HS, divided into three groups. The first group (n = 15 patients) received colchicine as monotherapy, the second group (n = 14 patients) received colchicine and doxycycline 100 mg/d, while the third group (n = 15 patients) received colchicine and doxycycline 40 mg/d. Disease severity during treatment was assessed at baseline and follow-up, using the Hurley Scoring System and the International Hidradenitis Suppurativa Severity Score System (IHS4). All patients were also asked to complete a Dermatology Life Quality Index (DLQI) questionnaire. These scores were compared among the study groups. Results: The DLQI and IHS4 scores significantly improved after treatment with colchicine (p &lt, 0.001) in all groups. All colchicine regimes, including the single colchicine regime, colchicine plus doxycycline 100 mg regime, and colchicine plus doxycycline 40 mg regime, resulted in significant improvements in the DLQI and IHS4 scores (p &lt, 0.001). Clinical improvement based on DLQI and IHS4 scores was similar in all groups. None of the patients had to discontinue the treatment due to adverse events. Discussion: In conclusion, our findings suggest that colchicine may improve clinical severity and quality of life in HS patients, either as monotherapy or in combination with doxycycline, both at antimicrobial (100 mg) and sub-antimicrobial (40 mg) doses.

Background: Squamous cell carcinoma of the lip accounts for 20% of all oral carcinomas. Its diagnosis may be challenging because it clinically resembles actinic cheilitis and inflammatory lesions of the lips. Objectives: To determine clinical and dermatoscopic predictors of squamous cell carcinoma of the lip vs. other lip lesions. Methods: Multicentre retrospective morphological study, including histologically confirmed cases of squamous cell carcinoma of the lip and controls consisting of actinic cheilitis and inflammatory lesions of the lips. Clinical and dermatoscopic images were evaluated for the presence of predefined criteria. Crude and adjusted odds ratios and corresponding 95% confidence intervals were calculated by univariate and multivariate logistic regression respectively. Results: A total of 177 lip lesions were evaluated, 107 (60.5%) were squamous cell carcinomas and 70 (39.5%) were controls. The most frequent dermatoscopic criteria of lip squamous cell carcinoma were scales (100%), white halos (87.3%) and ulceration (79.4%). The majority of squamous cell carcinomas displayed polymorphic vessels (60.8%), with linear (68.6%) and hairpin (67.6%) being the most frequent types. Multivariate logistic regression analysis showed that clinical predictors of lip squamous cell carcinoma were exophytic appearance and clinical hyperkeratosis, with 43-fold and 6-fold higher probability respectively. White clods and ulceration in dermoscopy presented a 6-fold and 4-fold increased risk for squamous cell carcinoma respectively. Conclusions: A scaly lesion with exophytic growth, dermatoscopically displaying white clods, ulceration and linear and hairpin vessels is very likely a squamous cell carcinoma of the lip.

For the second year in a row the European Academy of Dermatology and Venereology (EADV) School on Hidradenitis suppurativa/acne inversa (HS), which took place on February 3-4, 2020 in Athens with the participation of 4 specialists and 20 residents (17 female, 7 male; Table 1) systematically assessed its effect on the participants knowledge. The global immediate learning efficacy outcome of the two-day theoretical and practical course was evaluated in a voluntary, anonymous, prospective interventional study. A questionnaire with 50 course-relevant questions was distributed to all participants at the beginning and the end of the course for the purpose of self-assessment.

Background: Thin nodular melanoma (NM) often lacks conspicuous melanoma-specific dermatoscopic criteria and escapes clinical detection until it progresses to a thicker and more advanced tumour. Objective: To investigate the dermatoscopic morphology of thin (≤2mm Breslow thickness) vs. thick (>2mm) NM and to identify dermatoscopic predictors of its differential diagnosis from other nodular tumours. Methods: Retrospective, morphological case–control study, conducted on behalf of the International Dermoscopy Society. Dermatoscopic images of NM and other nodular tumours from 19 skin cancer centres worldwide were collected and analysed. Results: Overall, 254 tumours were collected (69 NM of Breslow thickness ≤2mm, 96 NM >2mm and 89 non-melanoma nodular lesions). Light brown coloration (50.7%) and irregular brown dots/globules (42.0%) were most frequently observed in ≤2mm NMs. Multivariate analysis revealed that dotted vessels (3.4-fold), white shiny streaks (2.9-fold) and irregular blue structureless area (2.4-fold) were predictors for thinner NM compared to non-melanoma nodular tumours. Overall, irregular blue structureless area (3.4-fold), dotted vessels (4.6-fold) and serpentine vessels (1.9-fold) were predictors of all NM compared to non-melanoma nodular lesions. Limitations: Absence of a centralized, consensus pathology review and cases selected form tertiary centres maybe not reflecting the broader community. Conclusions: Our study sheds light into the dermatoscopic morphology of thin NM in comparison to thicker NM and could provide useful clues for its differential diagnosis from other non-melanoma nodular tumours.

Background: Hidradenitis suppurativa (HS), also referred to as acne inversa, is a debilitating skin disease characterized by inflammatory nodules, chronic abscesses and tunnels (fistulae and sinuses). The association with pilonidal sinus disease (PSD) is frequently reported but not well documented. Objectives: To determine the prevalence and characteristics of inflammatory skin lesions located in the intergluteal fold (IGF) of patients with HS. Methods: This was an international multicentre retrospective cross-sectional study based on data collection from a large cohort of patients with HS with and without histopathology. Results From a total of 2465 patients with HS included in the study, 661 (27%) reported lesions in the IGF. These patients were significantly more often smokers and had more severe HS. Of the 238 patients with an available clinical diagnosis, intergluteal-HS (IG-HS) was diagnosed in 52 patients (22%) and PSD was diagnosed in 186 patients (78%). IG-HS was associated with the localization of HS in the proximity of the IGF, including the buttocks, genitals and the anus. There was a possibility of misclassification bias in this study as a clinical/image-based diagnosis or histopathology of the IGF lesions was not always available. Conclusions: The high prevalence of PSD suggests a strong link between both entities. Therefore, it may be useful to identify common pathophysiological mechanisms and develop common therapeutic strategies., SCOPUS: ar.j, info:eu-repo/semantics/published

Summary Background Chronic hand eczema is a common dermatological disorder of multifactorial aetiology. It affects physical, material, social and psychological aspects of life, thereby impairing health-related quality of life. Objectives The aim of the present study was to assess quality of life, anxiety, depression and obsessive-compulsive tendencies in patients with chronic hand eczema. Materials and methods Seventy-one patients with chronic hand eczema were included in the study. Quality of life was evaluated according to the Dermatology Life Quality Index (DLQI). Patients were also assessed for anxiety and depression with the Hospital Anxiety and Depression Scale (HADS), and for compulsive behaviour with the Leyton Trait Scale. Results The DLQI score was 11.11 ± 1.81 in patients with chronic hand eczema. Scores on the Leyton Trait Scale were significantly higher than those of healthy controls (p

Zusammenfassung Hintergrund Die Alopezie ist eine haufige dermatologische Erkrankung mit zumeist kosmetischen Folgen, jedoch einem wesentlichen psychologischen und psychosozialen Einfluss. Ziel Vergleichende Erhebung des Einflusses einer vernarbenden und einer nicht-vernarbenden Alopezie auf die Lebensqualitat und verschiedene psychologische Bereiche bei erwachsenen Griechinnen. Patientinnen und Methoden Vierundvierzig Frauen im Alter zwischen 18 und 70 Jahren mit vernarbender (n = 19) oder nicht-vernarbender (n = 25) Alopezie wurden eingeschlossen. Bei allen Patientinnen wurden der Dermatologische Lebensqualitatsindex (Dermatology Life Quality Index, DLQI), die Hospital Anxiety and Depression Scale (HADS), die Rosenberg Self-esteem Scale (RSES) und die UCLA Loneliness Scale (UCLA-LS) erhoben. Ergebnisse Frauen mit vernarbender Alopezie hatten hohere DLQI-, HADS- und UCLA-LS-Werte und niedrigere RSES-Werte im Vergleich zu Frauen mit nicht-vernarbender Alopezie. Statistisch signifikante Unterschiede zwischen den Gruppen bestanden bei DLQI (p = 0,0067), HADS (p = 0,0008) und den HADS-Angst (HADS-A) (p < 0,05) sowie HADS-Depression (HADS-D) (p < 0,01) – Subskalen. Schlussfolgerungen Bei vernarbender Alopezie ist die psychologische Belastung der Patientinnen schwerer und die Lebensqualitat starker eingeschrankt als bei nicht-vernarbender Alopezie, was wahrscheinlich die schlechtere Prognose der vernarbenden Alopezie widerspiegelt.

Growing evidence identifies histamine as a key player in allergic responses, but the reports relating serum histamine to tolerance are scarce and inconclusive. This study investigated the relationship of circulating histamine to the tolerant phenotype in allergic beekeepers. The results showed a positive correlation between the serum levels of histamine and specific immunoglobulin G4 (sIgG4) to honeybee venom, but not with total IgE or sIgE. Interestingly, both sIgG4 and histamine levels were negatively correlated with the time since the last bee sting. In contrast to total IgE and sIgE, serum sIgG4 and histamine levels followed comparable patterns, being higher in tolerant/recently stung individuals and lower in the least frequently stung subgroup. The data obtained in this pilot study associated, for the first time, serum histamine levels with allergen tolerance in allergic individuals and provided the lead for further considering the putative immunomodulatory properties of histamine in allergic responses.

Summary Background Alopecia is a common dermatological condition with mostly cosmetic consequences that, nevertheless, has significant psychological and psychosocial impact. Objective To assess the impact of alopecia on quality of life and certain psychological domains and to compare it between scarring and non-scarring alopecia in Greek adult women. Patients and methods Forty-four women, aged 18–70 years, with scarring (n = 19) or non-scarring alopecia (n = 25) were recruited. All patients were evaluated by Dermatology Life Quality Index (DLQI), Hospital Anxiety and Depression Scale (HADS), Rosenberg Self-esteem Scale (RSES) and UCLA Loneliness Scale (UCLA-LS). Results Women with scarring alopecia had higher scores in DLQI, HADS and UCLA- LS and lower scores in RSES, compared to women with non-scarring alopecia. A statistically significant difference between the two groups was documented for DLQI (p = 0.0067), HADS (p = 0.0008), and HADS-Anxiety (HADS-A) (p < 0.05) and HADS-Depression (HADS-D) (p < 0.01) subscales. Conclusions The psychological burden is heavier and quality of life is more severely impaired among women with scarring alopecia compared with non-scarring alopecia, probably depicting the poorer prognosis of the former.

We report a case of a 5-year-old girl with physical and psychomotor retardation, acquired microcephaly, and history of recurrent infections. Dermoscopic and microscopic hair examination revealed a “road-dividing line”-like pigmentation of hair shafts. The combination of history, clinical findings, and hair examination led to the diagnosis of Griscelli syndrome type II. The picture of “road-dividing line” on hair microscopic and dermoscopic examination is highly characteristic for Griscelli syndrome, and in this framework, dermatologic examination may be of high importance for the diagnosis.

Introduction: Androgenetic alopecia (AGA) in females is a difficult to treat skin disorder. A novel topical botanical lotion has been approved for its treatment. It acts by increasing Bcl-2, perifollicular Langerhans and mast cells, and perifollicular collagen. The aim of this study was to evaluate the efficacy and safety of this lotion in females. Methods: Forty women with AGA were randomized to apply the active lotion or placebo, twice daily for 24 weeks. Subjects were evaluated at 0, 12, and 24 weeks by clinical examination, photographic documentation, quality of life evaluation (DLQI), and trichogram (anagen to telogen ratio). Results: The clinical evaluation demonstrated an increased hair density in the intervention group (great improvement in 7.7%, moderate in 88.5%, and stable in 3.8%). The self-assessment score increased from 4.5 to 6.0 (24 weeks), and the DLQI improved from 4 to 3 in the intervention group (p < 0.001). The mean anagen to telogen ratio in the intervention group was 2.1, 3.9, and 6.0 at 0, 12, and 24 weeks, respectively, whereas in the control group it was 2.2, 3.8, and 3.3, respectively. Conclusion: The new topical botanical lotion showed remarkable efficacy, with a high degree of patient satisfaction and improvement of their quality of life.

Background: Bullous lichen planus (BLP) is a rare variant of lichen planus, characterized by the development of vesicular and bullous lesions, of skin, nails, hair and/or mucosa. Main observations: We present a case of 63-year-old woman with BLP, unresponsive to previous therapies with topical corticosteroids, topical calcipotriol, antihistamines and oral cyclosporine (4 mg/kg/day for 4 months). She was already receiving treatment for arterial hypertension, hyperlipidemia, atrial fibrillation and uncontrolled diabetes mellitus. Acitretin was administered for 5 months with complete remission of BLP lesions and no major side effects. Conclusions: This is probably the first reported case of BLP treated with acitretin monotherapy. In this case acitretin was an efficacious and well-tolerated therapeutic option for BLP. ( J Dermatol Case Rep . 2016; 10(4): 62-64)

Background/Objective: Hidradenitis suppurativa (HS) is a chronic, inflammatory skin disease that remarkably affects the patients' quality of life. A prospective study of 152 HS patients was conducted in order to depict the clinico-epidemiological characteristics of HS patients in Greece. Materials and Methods: From February 2015 to August 2016, 152 patients attended the Hidradenitis Suppurativa Outpatient Clinic of our department. The most important clinico-epidemiological data of the patients were registered. Descriptive statistics and an ordered logistic regression analysis were performed. Results: Of the 152 patients, 60.5% were females. Their median age was 37 years. A total of 26.3% of the patients suffered from Hurley stage I, 44.7% from Hurley stage II, and 29.0% from Hurley stage III. The median duration of disease was 9 years. The most common site of involvement was the groins. The mean Dermatology Life Quality Index (DLQI) was higher for Hurley stage III. In multivariable analysis, only the body mass index was found to independently predict the disease severity. Conclusion: Among the factors examined, the body mass index appears to independently predict the disease severity. Quality of life evaluation revealed a stronger impact compared to the international experience. To our knowledge, this is the first descriptive epidemiological study among HS patients in Greece.

Background Few published data, concerning the electron microscopy findings of idiopathic guttate hypomelanosis have been published so far. Objectives To reveal the electron microscopic findings of idiopathic guttate hypomelanosis and their aetiopathogenetic associations. Methods Punch biopsy specimens from four patients with idiopathic guttate hypomelanosis, after being properly processed, were observed under the electron microscope. Results In the epidermis, melanocytes and melanosomes were normal in structure. In some areas, there was a reduced uptake of melanosomes by the keratinocytes. In the dermis, fibroblasts were structurally normal. Also, most elastic and collagen fibres were normal, but there were focal elastotic changes. Conclusions No significant structural abnormality of the melanocytes was observed, but rather a functional defect in the transfer of melanosomes from the melanocytes to the keratinocytes.

The specific anatomy of the glabrous skin, characterized by marked orthokeratosis and the presence of furrows and ridges, results in peculiar dermoscopic patterns of acral melanocytic lesions. Most frequently, acral nevi are typified by a parallel furrow pattern and acral melanoma (AM) by a parallel ridge pattern (PRP). Although the dermoscopic patterns of AM have been extensively investigated, little attention has been paid to site-related differences between palmar and plantar AM. The current study aimed to compare patients' characteristics, melanoma thickness, and the morphologic variability of AM depending on the localization on palms or soles. Patients demographics and dermoscopic images of 118 AM, including 99 (83.9%) plantar and 19 (16.1%) palmar lesions (mean thickness, 2.1 mm), were extracted from the databases of seven pigmented skin lesion clinics and were evaluated for the presence of predefined criteria. Palmar melanomas were remarkably more frequent in women (male-to-female ratio, 1/3.8) and thinner than plantar melanomas (1.3 vs. 2.2 mm). Dermoscopically, no significant differences were found between plantar and palmar melanomas, with PRP scored in 64.6 and 68.4% of plantar and palmar lesions, respectively. Non-site-specific melanoma criteria were detected in 83.9% of lesions and, among melanomas not exhibiting a PRP, 95.1% showed at least one non-site-specific melanoma criterion. In conclusion, plantar and palmar AMs show sex-related and thickness-related differences, but do not differ with respect to their dermoscopic features. For cases lacking the PRP, non-site-specific melanoma criteria may be considered as helpful additional clues for the correct diagnosis.

Aims: To assess the efficacy and safety of rituximab in refractory pemphigus and the possible benefit of an additional prophylactic infusion at 6 months. Methods: Seventeen patients with pemphigus vulgaris, 1 with pemphigus foliaceus and 1 with pemphigus vegetans were treated with 4 weekly infusions of rituximab (375 mg/m2). Nine patients received an additional prophylactic infusion after 6 months while the rest received no maintenance therapy. In case of recurrence, an additional single infusion was administered. Results: Control of the disease was obtained after 3-8 weeks. End of the consolidation phase for all patients was observed after 16 weeks. Patients remained in full remission for 7-42 months. All immunosuppressive agents, including prednisone, were discontinued after 2-12 months. The disease relapsed in 5 out of 9 patients who received the additional prophylactic infusion, and in 3 out of 10 patients among those skipping the prophylactic additional infusion. Conclusion: One course of rituximab and treatment of relapses is highly effective and well tolerated in the treatment of refractory pemphigus. In this pilot study of 19 patients, the prophylactic infusion does not appear to have provided any additional benefit to the patients receiving it.

Majocchi's granuloma (MG) is an uncommon deep fungal infection. It is usually caused by Trichophyton rubrum and may develop in any hair-bearing skin, commonly on the face and the extremities. We present a 27-year-old female with MG of the pubic area treated unsuccessfully with itraconazole capsule 100 mg for 4 weeks. The medication was discontinued and switched to terbinafine, which proved efficacious. The pubic tinea clinically presented in conjunction with fever, lymphadenopathy, inflammatory plaques, follicular lesions and subcutaneous nodules. It was considered that the fungal infection was initially transmitted from skin to skin during sexual practices with her husband. The repeated shaving of her pubic area, the misdiagnosis as bacterial infection and the use of topical corticosteroid on a preexistent tinea probably predisposed the patient to MG.

Antigen presentation in chronic skin disorders is mediated through the interleukin (IL)-12/IL-23 pathway and, hence, through the IL-12 receptor. Recent evidence suggesting dysregulated antigen presentation in skin lesions of hidradenitis suppurativa (HS) led to investigate the role of single nucleotide polymorphisms (SNPs) of the gene IL-12RB1 coding for the IL12-Rβ1 receptor subunit. Genomic DNA was isolated from 139 patients and 113 healthy controls; nine SNPs in the transcribed region of IL12RB1 were genotyped. No significant differences of genotype and allele frequencies were found between the two groups. Two common haplotypes were recognized, namely h1 and h2. Carriage of h2 related with minor frequency alleles was associated with a greater risk for the acquisition of Hurley III disease stage and with the involvement of a greater number of skin areas. Patients with the h1 haplotype presented disease at an older age. This is the genetic study enrolling the largest number of patients with HS to date. Although SNPs of IL12RB1 do not seem to convey genetic predisposition, they are associated directly with the phenotype of the disease.

Background: Nail disorders have considerable psychological impact and may limit activity by impairing functionality of both fingers and toes Objective: To evaluate the impact of nails disorders on quality of life (QoL). Methods: 1063 patients with nail disorders completed an anonymous nail-specific QoL questionnaire consisting of 24 and 16 questions, respectively, for fingernails and toenails with five possible responses to each question. A score of 1-5 was given to each response and the final score was adjusted on a percentile scale. The subjects were classified in groups according to nail disorders. Statistical analysis was carried out using T-test to compare the means between two groups and ANOVA analysis of variance to compare the impact of quality of life on the different types of nail disorders. Results: Comparison between groups showed a statistically significant higher impact for trauma, onychomycosis, other infections, structure abnormalities, psoriasis, other inflammatory diseases and paronychia and a lower impact for chromonychias and tumours. QoL was statistically significantly more affected in patients having multiple nails involved, women, and in people aged 60-79 years. There was no statistically significant difference on the QoL impact between patients having only fingernails or only toenails involved. Conclusion: Even though published literature tends to focus on the impact of nail psoriasis and onychomycosis on QoL, other nail disorders cause similar frustration to patients. A possible explanation for this is that appearance of the nail has a more significant impact on QoL than the severity of the disorder.

Summary Background Recent evidence has suggested that deranged immune responses play a role in the pathogenesis of hidradenitis suppurativa (HS). Objectives To investigate the role of single nucleotide polymorphisms (SNPs) of the tumour necrosis factor (TNF) and Toll-like receptor 4 (TLR4) genes in the physical course of HS; these genes encode for proteins implicated in the immune response of the host. Methods DNA was isolated from 190 patients with HS and 84 healthy controls. SNPs at the promoter regions −376G/A, −238G/A and −308G/A of the TNF gene and the Asp299Gly and Thr399Ile SNPs of the TLR4 gene were determined by polymerase chain reaction (PCR) and digestion of the PCR product by restriction enzymes; after electrophoresis on 2·0% agarose gel, products were visualized on under ultraviolet radiation. Results The presence of the −238 TNF gene polymorphism was associated with a predisposition to HS (P = 0·027). Susceptibility to the disease was strongly correlated with the presence of AGG/GGA/AGA/GAA TNF haplotypes in 32 (17%) patients compared with two (2%) controls (P

Frontal fibrosing alopecia (FFA) is a primary lymphocytic cicatricial alopecia characterized by a progressive band-like recession of the frontotemporal hairline and frequent loss of the eyebrows. It predominantly affects postmenopausal women. Coexistence of FFA and vitiligo is rarely reported in the literature. We retrospectively studied 20 cases diagnosed with FFA in a 14-month period in our Department. Among them, there were 2 cases, a 72-year-old woman and a 48-year-old man, who developed FFA on preexisting vitiligo of the forehead. Anatomical colocalization of the two dermatoses supports the notion that a causal link may exist and their association may not be coincidental. We suggest that interrelated immunologic events and pathologic processes may underlie both these skin conditions.

Schnitzler's syndrome is characterized by chronic urticaria and monoclonal immunoglobulin (Ig) M gammopathy, with other features including intermittent fever, joint and/or bone pain with radiologic evidence of osteosclerosis, lymphadenopathy, enlarged liver and/or spleen, leukocytosis, and elevated erythrocyte sedimentation rate. The etiology of the syndrome remains obscure, although involvement of various cytokines has been proposed. Bone metabolism and angiogenesis markers are deregulated in Waldenström macroglobulinemia and IgM monoclonal gammopathy of unknown significance, but these markers have not been assessed in Schnitzler's syndrome. Herein, we report a patient with Schnitzler's syndrome who was treated with oral pefloxacin. Serum levels of osteoclast regulators, markers of bone remodeling and angiogenesis cytokines, were measured before treatment and serially after the initiation of treatment. High bone turnover and strikingly elevated levels of angiogenic cytokines were observed at diagnosis. Treatment with pefloxacin resulted in a normalization of the bone remodeling process and a significant reduction of angiogenic cytokines, with rapid and sustained improvement of symptoms, suggesting that these factors might be implicated in the pathophysiology of this syndrome. Furthermore, pefloxacin was proven to be an effective treatment for patients with Schnitzler's syndrome.

Contains fulltext : 171452.pdf (Publisher’s version ) (Closed access) Importance: Hidradenitis suppurativa (HS) is a common skin disorder in which excessive inflammation is believed to have an important role. There is no specific therapy for HS. Objective: To investigate the safety and efficacy of the anti-inflammatory biological therapy anakinra in HS. Design, Setting, and Participants: Double-blind, randomized, placebo-controlled clinical trial with a 12-week treatment phase and a 12-week follow-up phase. The setting was Attikon University General Hospital, a tertiary care institution in Athens, Greece. Participants were 20 patients with Hurley stage II or III HS. The study and the analysis were conducted between March 1, 2012, and February 28, 2014. Interventions: Patients were randomized to receive injections from identical syringes containing placebo or anakinra subcutaneously once daily for 12 weeks. Peripheral blood mononuclear cells were isolated and stimulated for cytokine production before the beginning of treatment and at week 12 (the end of treatment) and week 24. Main Outcomes and Measures: The primary end point was the effect of anakinra on HS disease severity. Secondary end points were the time to a new exacerbation and the production of cytokines. Results: Among the 20 trial participants, 10 each were randomized to the group to receive anakinra or the placebo group. The mean (SD) ages were 42.8 (13.8) and 36 (11.3) years in the anakinra and placebo groups, respectively. The disease activity score was decreased at the end of treatment in 20% (2 of 10) of the placebo arm compared with 67% (6 of 9) of the anakinra arm (P = .04). Hidradenitis suppurativa clinical response at 12 weeks was achieved in 30% (3 of 10) of the placebo arm and in 78% (7 of 9) of the anakinra arm (P = .04). The production of interferon-gamma by peripheral blood mononuclear cells in the anakinra arm was decreased, and the production of interleukin 22 was increased. The time to a new HS exacerbation was prolonged in the anakinra arm by log-rank test (log rank, 6.137; P = .01). No serious adverse events were reported. Conclusions and Relevance: Anakinra has the potential to be an effective and well-tolerated treatment for HS. Inhibition of interleukin 1 is a promising treatment strategy. Trial Registration: clinicaltrials.gov Identifier: NCT01558375.

Background: Psoriasis is one of the most common, immune-mediated, chronic inflammatory skin diseases. Proinflammatory cytokines play an important pathogenetic role at a local level. Objective:To assess whether the proinflammatory cytokines IL-1β, IL-6, IL-17, IL-22 and TNF-α are released systemically during psoriasis. Methods:Peripheral blood mononuclear cells (PBMCs) were isolated from 30 patients with psoriasis and 30 healthy volunteers. Cytokine production was assessed in supernatants using an enzyme immunoassay after stimulation of PBMCs with microbial stimuli. In addition, flow cytometry was used to determine the subsets of monocytes involved and the intracellular TNF-α production in monocytes. Results:IL-17 levels were significantly higher in the supernatants of PBMCs from psoriatic patients after stimulation with phytohemagglutinin. TNF-α production was also significantly higher in cells from psoriatic patients after stimulation with all stimuli, as compared with health volunteers. Similar changes were not found for the other cytokines. A statistically significant difference was observed between patients and controls for inflammatory CD14+/CD16+ monocytes (p

Introduction: Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease of unclear etiology. The role of bacteria in the pathogenesis of disease remains controversial. Materials and Methods: Specimens were obtained from 22 HS patients by direct percutaneous needle aspiration. The collected material was cultured in aerobic and anaerobic conditions, and sensitivity tests were performed. Results: Of the 22 patients, 32% were culture negative and 68% were culture positive. A total of 16 isolates was obtained, 14 aerobic and 2 anaerobic. Aerobic bacteria were present in 86% of the specimens, whereas only anaerobic bacteria were isolated in 7%. The predominant aerobic species were Proteus mirabilis, Staphylococcus haemolyticus and Staphylococcus lugdunensis. The isolated anaerobic bacteria were Dermacoccus nishinomiyaensis and Propionibacteriumgranulosum. Conclusion: A variety of aerobic and anaerobic bacteria was isolated from the HS lesions of our patients. In contrast to previous studies, fewer patients were found to be culture positive, and Staphylococcus aureus was isolated in only 1 of them. More studies are necessary to elucidate the controversial role of bacteria in the pathogenesis of HS.

A number of genetic mutations is associated with cardiomyopathies. A mutation in the coding region of the phospholamban (PLN) gene (R14del) is identified in families with hereditary heart failure. Heterozygous patients exhibit left ventricular dilation and ventricular arrhythmias. Here we generate induced pluripotent stem cells (iPSCs) from a patient harbouring the PLN R14del mutation and differentiate them into cardiomyocytes (iPSC-CMs). We find that the PLN R14del mutation induces Ca2+ handling abnormalities, electrical instability, abnormal cytoplasmic distribution of PLN protein and increases expression of molecular markers of cardiac hypertrophy in iPSC-CMs. Gene correction using transcription activator-like effector nucleases (TALENs) ameliorates the R14del-associated disease phenotypes in iPSC-CMs. In addition, we show that knocking down the endogenous PLN and simultaneously expressing a codon-optimized PLN gene reverses the disease phenotype in vitro. Our findings offer novel strategies for targeting the pathogenic mutations associated with cardiomyopathies., Phospholamban (PLN) is a regulator of heart contractility. Here the authors show that cardiomyocytes derived from induced pluripotent stem cells of a cardiomyopathy patient with mutant PLN exhibit functional defects consistent with the disease, and that this mutation can be functionally corrected by genome editing and gene therapy.

Conventional therapies for human papillomavirus infection aim to remove clinically apparent lesions, while latent infection may remain, representing a threat for transmission and carcinogenesis. The use of a systemic agent may more effectively control the virus. We conducted a randomised placebo-controlled study to investigate the efficacy and safety of oral inociplex in the treatment of cervical condylomata acuminata (CA) that had been resistant to conventional therapies. Thirty-eight white European women, aged 20–43 years, with genital warts of the cervix, refractory to at least one conventional therapy, were randomly assigned to receive either inosiplex, 50 mg/kg daily peros for 12 weeks (group 1), or placebo (group 2). Of the 17 evaluable group 1 women, 4 responded to the treatment completely, 7 responded partially and 6 did not respond. Of the 19 group 2 women, none responded to the treatment completely, 3 responded partially and 16 did not respond. The therapeutic difference between women receiving active and placebo therapy was statistically significant (χ2= 6.69, P < 0.01) and remained significant when an intention-to-treat analysis was performed (χ2= 7.69, P < 0.01). None of the complete responders experienced recurrence during the 12-month follow up. Adverse effects were mild and resolved upon completion of therapy. Compared with placebo, inosiplex showed considerable efficacy with insignificant and reversible adverse effects and without recurrences. Inosiplex may represent an efficacious and safe alternative systemic form of therapy for cervical genital warts.

Summary Background Poikiloderma of the face and neck (Civatte) is a rather common, indolent, chronic dermatosis, most often affecting menopausal females. Cumulative excessive sun exposure, a phototoxic or a photoallergic reaction, hormonal changes of menopause and genetic factors have all been incriminated in its obscure aetiopathogenesis. Objectives To evaluate the role of contact sensitization and photosensitivity in the pathogenesis of poikiloderma of Civatte (PC). Methods Thirty-two patients (24 females and eight males, age range 38–74 years) with PC were patch tested with the European standard series and the fragrance series, and were photopatch tested with the photoallergens series. Additionally, photo-testing with a monochromator was performed. Results Thirteen of 32 patients (40·62%) had one or more positive reactions to allergens of the standard series. Eight patients (25%) had positive reactions to fragrance mix and/or Balsam of Peru, which are included in the standard series, or to allergens of the fragrance series. Nickel sulphate was the single most common cause of contact sensitization (18·75%) among our patients. Ninety-seven subjects, who were patch tested with the standard series for suspected allergic contact dermatitis of the face and/or neck, served as age, sex and site controls. Of these, nine (9·27%) had one or more positive reactions to fragrance compounds. Statistical analysis showed a statistically significant difference in the frequency of positive reactions to fragrances between the PC group and the control group (χ2 value = 3·91, P

Chronic hand eczema is a common dermatological disorder of multifactorial aetiology. It affects physical, material, social and psychological aspects of life, thereby impairing health-related quality of life.The aim of the present study was to assess quality of life, anxiety, depression and obsessive-compulsive tendencies in patients with chronic hand eczema.Seventy-one patients with chronic hand eczema were included in the study. Quality of life was evaluated according to the Dermatology Life Quality Index (DLQI). Patients were also assessed for anxiety and depression with the Hospital Anxiety and Depression Scale (HADS), and for compulsive behaviour with the Leyton Trait Scale.The DLQI score was 11.11 ± 1.81 in patients with chronic hand eczema. Scores on the Leyton Trait Scale were significantly higher than those of healthy controls (p 0.027). As concerns the HADS-Anxiety subscale, patients with hand dermatitis had statistically significantly higher scores than those of volunteers (p = 0.002). In contrast, no statistically significant difference was found between the two groups with regard to the HADS-Depression subscale score and total HADS score.Hand eczema treatment should address the severity of skin lesions as well as the psychological impact of hand eczema.