S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology.

INTRODUCTION: Linear IgA dermatosis (LAD) is a rare subepidermal autoimmune bullous disease (AIBD) defined by predominant or exclusive immune deposits of immunoglobulin A at the basement membrane zone of skin or mucous membranes. This disorder is a rare, clinically and immunologically heterogeneous disease occurring both in children and in adults. The aim of this project is to present the main clinical features of LAD, to propose a diagnostic algorithm and provide management guidelines based primarily on experts' opinion because of the lack of large methodologically sound clinical studies. METHODS: These guidelines were initiated by the European Academy of Dermatology and Venereology (EADV) Task Force Autoimmune Bullous Diseases (AIBD). To achieve a broad consensus for these S2k consensus-based guidelines, a total of 29 experts from different countries, both European and non-European, including dermatologists, paediatric dermatologists and paediatricians were invited. All members of the guidelines committee agreed to develop consensus-based (S2k) guidelines. Prior to a first virtual consensus meeting, each of the invited authors elaborated a section of the present guidelines focusing on a selected topic, based on the relevant literature. All drafts were circulated among members of the writing group, and recommendations were discussed and voted during two hybrid consensus meetings. RESULTS: The guidelines summarizes evidence-based and expert opinion-based recommendations (S2 level) on the diagnosis and treatment of LAD. CONCLUSION: These guidelines will support dermatologists to improve their knowledge on the diagnosis and management of LAD.

Response to adalimumab in Caucasian and Asian patients with hidradenitis suppurativa: A retrospective cohort study of an Australian cohort stratified by patient-reported ethnicity.

BACKGROUND/OBJECTIVES: Caucasian and Asian patients with hidradenitis suppurativa demonstrate significant differences with regard to age, gender and body mass index. Demographic characteristics are known to influence the efficacy and drug survival of hidradenitis suppurativa therapeutics including biologic therapies. What remains unknown is the impact of ethnicity upon the efficacy of therapeutics once demographic and disease characteristics have been taken into account. This is an important question given the expansion of biologic therapies for HS into the global patient community. METHODS: We assessed 170 patients from a single HS specialist centre in Australia stratified by patient-identified ethnicity including those identifying as either Caucasian or Asian. RESULTS: Asian patients demonstrated lower BMI, higher rates of smoking and greater odds of Hurley stage 3 disease with tunnels than Caucasian patients in line with the reported literature. There was no significant difference between percentage of individuals achieving HiSCR50 or IHS4-55 at Week 16. Significant differences were seen in median time to secondary loss of response, and Kaplan-Meier curve analysis showed a significant difference between curves when stratified by patient-reported ethnicity. Cox regression analysis demonstrated after accounting for age, gender, BMI, smoking and Hurley stage, the significance of ethnicity in influencing time to secondary loss of response disappears. CONCLUSIONS: Caucasian or Asian ethnicity does not influence response to adalimumab treatment on patients with hidradenitis suppurativa.

Infectious Disease Screening Prior to Systemic Immunomodulatory Therapy in Hidradenitis Suppurativa: Consensus Guidelines from the Asia-Pacific Hidradenitis Suppurativa Foundation.

BACKGROUND: Current infectious disease screening recommendations for hidradenitis suppurativa (HS) are adopted from recommendations in chronic plaque psoriasis. No HS-specific guidelines for infectious disease screening prior to immunomodulatory therapy have been developed. OBJECTIVES: To establish an expert Delphi consensus of recommendations regarding infectious disease screening prior to systemic immunomodulatory therapy in HS. METHODS: Participants were identified via recent publications in the field and were sent a questionnaire regarding infectious diseases encountered in the setting of HS, and opinions regarding infectious disease screening prior to various systemic immunomodulatory therapies. All questions were informed by a systematic literature review regarding infections exacerbated or precipitated by immunomodulatory therapy. Questionnaire responses were followed by round-table discussion with a core group of 8 experts followed by a final round of questionnaires resulting in achievement of consensus. RESULTS: 44 expert HS physicians from 12 countries on 5 continents participated in the development of the expert consensus recommendations. Consensus recommendations include screening for hepatitis B, hepatitis C and tuberculosis in all individuals with HS prior to therapy. All immunomodulatory therapies (biologic and systemic immunosuppressant therapy) should be preceded by infectious disease screening including patient and location specific considerations for endemic local diseases and high-risk activities and occupations. Clinical assessment has a significant role in determining the need for laboratory screening in the setting of many uncommon or tropical diseases such as leprosy, leishmaniasis and strongyloidiasis. CONCLUSIONS: The presented consensus recommendations are the first specifically developed for pre-treatment infectious disease screening in Hidradenitis Suppurativa.

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.

Malignancy-associated generalised exfoliative dermatitis: A retrospective study in a single-centre Asian cohort.

BACKGROUND: Erythroderma is an inflammatory skin condition that causes extensive erythema and skin scaling amounting ≥90% of the body surface area. This retrospective cohort study describes the prevalence of malignancy-associated erythroderma in a single centre where there was concerted effort to systematically offer malignancy screens to all adult erythroderma patients above the age of 65 years. METHODS: Clinical charts were reviewed for all adult inpatients and outpatients with erythroderma who attended the National University Hospital (NUH) from 1 July 2019 to 31 December 2021. Data collected included patient demographics, clinical findings, laboratory investigations, disease-specific investigations such as endoscopic procedures and biopsies, follow-up duration and mortality data. RESULTS: Seventy-four patients were analysed. The median age of the patients was 73 years old (interquartile range: 59-81 years old). An underlying dermatosis was the most common cause of erythroderma-63 patients having atopic dermatitis/asteatotic eczema or psoriasis. Three patients had erythroderma from drug eruptions, and 1 patient had chronic actinic dermatitis. Four patients had associated malignancies (5.4%). Half of our patients completed further evaluation for malignancy (52.7%). The rest had either declined or were eventually unable to complete the investigations. There was a higher prevalence of associated malignancy (7.8%) in elderly patients above 65 years old. CONCLUSION: When compared to existing literature, our cohort reflects a higher observed occurrence of malignancy in association with erythroderma. As delays in evaluation for underlying malignancy could result in potentially deleterious outcomes, it is prudent to consider systematic screening for malignancy in high-risk populations such as elderly erythroderma patients.

Teledermatology in an emergency department: benefits and gaps.

BACKGROUND: Teledermatology has provided new avenues through which dermatologists can provide healthcare. Teledermatology was introduced to the Emergency Department (ED) to enable immediate dermatological consult. We aimed to assess the impact of teledermatology on the management of dermatological conditions by emergency medicine physicians and subsequent health resource utilization. METHODS: We conducted a retrospective review of teledermatology referrals from the ED of our tertiary hospital in Singapore from June 2015 to December 2019. The dermatological conditions, the triaging and treatment recommendations were analyzed. Follow-up plans were recorded. RESULTS: Between June 2015 and December 2019, 147 patients were referred from the ED via teledermatology; 11 (7.5%) were admitted, and 136 (92.5%) were recommended to be discharged with a dermatological diagnosis and management plan. If required, a follow-up appointment in the dermatology specialist clinic was arranged. Of the 136 patients who were discharged, 129 (94.9%) patients were provided with outpatient appointment in the dermatology clinic, out of which 110 patients returned for follow-up. 90 (81.8%) patients retained the initial teledermatology diagnoses and 20 (18.2%) patients had their teledermatology diagnoses revised after in-person review. CONCLUSIONS: Teledermatology allows for more efficient triaging of patients with dermatological conditions. Reliability between teledermatology and clinic-based examination is good. Patients may be managed mainly in the outpatient setting with appropriate specialty-directed treatment, return advice, and appropriately-triaged follow-up outpatient appointment.

Hidradenitis suppurativa in pediatric patients.

Interest in and research on hidradenitis suppurativa (HS) have increased during the past decade, resulting in expanded knowledge about associated comorbidities and treatment efficacy. Knowledge about pediatric-onset HS is still limited, based on case studies, small case series, and extrapolation from adult studies. With increasing prevalence of childhood obesity, decreasing pubertal age, and increasing awareness of HS, physicians may start encountering younger HS patients. This review presents an updated discussion on the epidemiology, pathophysiology, and associated comorbidities in HS, with a focus on pediatric and adolescent patients. It also suggests recommendations for investigation and treatment based on current evidence.

Hidradenitis suppurativa in South-East Asia and East Asia.

Hidradenitis suppurativa (HS) in South-East Asia and East Asia shows distinct clinical, environmental, physiological and likely genetic differences compared with the West. A male predominance is present, which may be due to differences in smoking habits. Involvement of the buttocks is common in East Asian patients, while the axillae are most commonly affected in South-East Asian patients. Metabolic comorbidities are prevalent in South-East Asian and East Asian HS patients. A family history of HS is less common than noted in Western populations. Asian ethnic subgroups deserve further study.

Efficacy of targeted education in reducing topical steroid phobia: A randomized clinical trial.

BACKGROUND: Fear of adverse effects of corticosteroids is common in dermatology and results in medication nonadherence. OBJECTIVE: To study the efficacy of targeted education in reducing topical steroid phobia. METHODS: In this double-blinded, randomized controlled trial, participants in the intervention arm were presented with an educational video and patient information leaflet targeting common misconceptions of topical corticosteroids. Steroid phobia was assessed with the topical corticosteroid phobia (TOPICOP) scale, medication adherence with the Elaboration d'un outil d'evaluation de l'observance des traitements medicamenteux (ECOB) score, and quality of life with the Dermatology Life Quality Index (DLQI). RESULTS: The study randomized 275 patients. The mean TOPICOP score in the intervention arm decreased (improved) from 41.9 (SD, 17.4) to 37.1 (SD, 20.0) and to 33.8 (SD, 19.0) at 1 month and 3 months, respectively, with the reduction arising from the knowledge domain but not the fears and behaviors domain. This remained statistically significant after adjusting for demographic confounding with an expected reduction of 4.22 points (P = .031). After accounting for demographic factors, there was no statistical difference in medication adherence and quality of life. Limitations include the exclusion of non-English-speaking patients. CONCLUSION: Targeted education at a single time point improved the TOPICOP score primarily in the knowledge domain but not in the fear domain.

Limited efficacy of diphenylcyclopropenone in the treatment of alopecia areata: Experience from a Tertiary Healthcare Institution in Singapore.

Alopecia areata (AA) is a common cause of nonscarring hair loss. Diphenylcyclopropenone (DPCP) is a form of contact immunotherapy used in the treatment of AA. We retrospectively reviewed all patients who were diagnosed with AA over a 4-year period (1st January 2012 to 31st December 2015) and who have received DPCP. Forty patients were studied in total. The mean duration of disease prior to the study was 195 days. Patients received a mean number of 14.91 sessions (range: 1-65). The mean number of sessions required before clinical response was seen was 2.33 sessions, corresponding to 0.001% DPCP. Based on the modified Global Assessment Grading System, 33.5% (n = 11) of the patients experienced less than 25% improvement, 48.5% (n = 16) experienced 25%-74% improvement and 18.3% (n = 6) experienced more than 75% improvement. One patient had severe sensitisation reaction amounting to near erythroderma which resolved completely upon cessation of DPCP therapy. No other adverse reactions were noted in the cohort. DPCP remains a valuable tool in a dermatologist's armamentarium in treating alopecia areata as it is safe, well-tolerated, and shows limited efficacy.

Radiofrequency ablation-induced thermal cutaneous injury in a neonate: A potential complication after intrauterine radiofrequency ablation.

Over the years, the application and complications of radiofrequency ablation (RFA) in selective fetal reduction for complex pregnancies have been increasingly documented. Despite its rising use in the field of obstetrics and gynecology, the cutaneous complications of RFA have not been commonly reported. Here, we present a case of cutaneous thermal injury to the fetus likely secondary to intrauterine RFA for fetal reduction.

Retrospective Study on Autoimmune Blistering Disease in Paediatric Patients.

BACKGROUND: Autoimmune blistering diseases (AIBDs) are rare in children and their prevalence in Singapore is unclear. We aimed to investigate the clinical and immunopathologic characteristics of children diagnosed with AIBDs in Singapore. MATERIALS AND METHODS: The clinical and histology databases at the National Skin Centre in Singapore were searched to identify patients younger than 18 years old diagnosed with an AIBD from January 1, 1998, through December 31, 2012. Patient demographic characteristics, presentation, triggers, investigations, treatments, and disease course were analyzed. RESULTS: Twelve patients with AIBDs were identified; five (41.7%) had linear immunoglobulin A disease (LAD); two (16.7%) each had pemphigus vulgaris (PV), bullous pemphigoid, and bullous systemic lupus erythematosus; and one (8.3%) had pemphigus foliaceus. Four (33.3%) were female and eight (66.7%) male. The mean age of onset was 8.7 years (range 2-17 years). Most patients were treated with steroids and adjuvant immunosuppressants. Intravenous rituximab was used effectively in a patient with recalcitrant PV. The mean follow-up was 2.35 years (range 0.17-7.33 years). As of the last follow-up, four (33.3%) patients were in complete remission off therapy, two (16.7%) were in complete remission on therapy, four (33.3%) were in partial remission on therapy, and two (16.7%) were lost to follow-up. CONCLUSION: Consistent with the existing literature, our study shows that LAD is the most common cause of AIBDs in children. Although common in the West, dermatitis herpetiformis was not identified in the current study. Intravenous rituximab may be considered in recalcitrant childhood PV, but vigilant monitoring for side effects is crucial. Immunohistopathologic evaluation is important and repeat biopsies may be of value in patients with atypical disease courses.

Lichen nitidus presenting with nail changes--case report and review of the literature.

Lichen nitidus of the nail is rare and can precede the onset of skin lesions. Delayed diagnosis is common. We present an unusual case of lichen nitidus-associated nail changes that preceded the onset of skin lesions in a 4-year-old Indian girl. We also conduct a review of six other cases of lichen nitidus with nail involvement from the English-language literature. Clues to the diagnosis of lichen nitidus include violaceous or pigmentary changes of the nail fold and subtle lichenoid papules on the affected digits. Lichen nitidus of the nails appears to be less severe than nail changes of lichen planus and is generally self-limiting. Understanding the natural history of lichen nitidus of the nails will help physicians better counsel patients and their families.