Pigmentation of lower limbs: Contribution of haemosiderin and melanin in chronic venous insufficiency and related disorders.

BACKGROUND: To determine the composition of skin pigmentation in chronic venous insufficiency (CVI) and other less common vascular conditions of lower limbs. METHODS: Forty-five skin biopsies were obtained from 17 patients. Samples were taken from pigmented regions and compared with control non-lesional samples from the same patient. Perl's Prussian Blue was used to identify haemosiderin and Schmorl's for melanin. RESULTS: Seven patients presented with CVI, one with concurrent livedo vasculopathy (LV). One patient had LV only. Two patients had acroangiodermatitis (AAD). Six patients had post-sclerotherapy pigmentation (PSP), one with concurrent post-inflammatory hyperpigmentation (PIH). One patient had PIH only. The predominant pigment in CVI samples was haemosiderin. C5-C6 patients showed increased epidermal melanin. LV, AAD, and PSP samples showed dermal haemosiderin but no increase in epidermal melanin. PIH samples showed prominent epidermal melanin whilst no haemosiderin was detected. CONCLUSION: The predominant pigment in CVI and other vascular conditions was haemosiderin. Melanin was present in later stages of CVI (C5-C6) and in PIH.

Academic dermatology in Australia and New Zealand between 2017 and 2022: A cross-sectional bibliometric analysis.

INTRODUCTION: Academic dermatologists in Australia and New Zealand provide high-quality and meaningful contributions to the understanding of disease and therapeutic translational research. Concerns have been raised by the Australian Medical Association regarding the decline of clinical academics in Australia as a whole, however, such trends in scholarly output have not previously been analysed for Australasian dermatologists. METHODS: A bibliometric analysis of dermatologists in Australia and New Zealand was conducted in January and February 2023. Available Scopus profiles for all dermatologists were used to measure lifetime H index, scholarly output, citation counts and field-weighted citation impact (FWCI) in the last 5 years (2017-2022). Trends in output over time were measured using non-parametric tests. Differences in output between subgroups stratified by gender and academic leadership positions (associate professor or professor) were measured using Wilcoxon rank-sum and one-way ANOVA tests. The scholarly output of recent College graduates was also analysed as a subgroup, comparing the same bibliographic variables in the 5 years preceding and 5 years following awarding of their fellowships. RESULTS: From the 463 practising dermatologists in Australia and New Zealand, 372 (80%) were successfully matched to Scopus researcher profiles. Of these dermatologists, 167 were male (45%) and 205 (55%) were female, and 31 (8%) held academic leadership positions. Most dermatologists (67%) published at least one paper in the last 5 years. The median lifetime H index was 4, and between 2017 and 2022 median scholarly output was 3, the median citations were 14 and the median FWCI was 0.64. There was a non-significant trend towards fewer publications per year, however, citation count and FWCI decreased significantly. By subgroups, female dermatologists published significantly more papers between 2017 and 2022, and other bibliographic variables were comparable to male dermatologists. However, women were underrepresented in positions of academic leadership-comprising only 32% of this cohort despite representing 55% of dermatologists. Professors were also significantly more likely to have higher bibliographic outcomes than associate professors. Finally, analysis of recent College graduates highlighted a significant decline in bibliometric outcomes pre- and post-fellowship. CONCLUSION: Overall, our analysis identifies a trend towards decreased research output by dermatologists in Australia and New Zealand in the last 5 years. Strategies to support dermatologists in research endeavours, particularly women and recent graduates, will be essential in maintaining strong scholarly output among Australasian dermatologists and thereby sustaining optimal evidence-based patient care.

Sclerotherapy of lower limb veins: Indications, contraindications and treatment strategies to prevent complications - A consensus document of the International Union of Phlebology-2023.

BACKGROUND: Sclerotherapy is a non-invasive procedure commonly used to treat superficial venous disease, vascular malformations and other ectatic vascular lesions. While extremely rare, sclerotherapy may be complicated by serious adverse events. OBJECTIVES: To categorise contraindications to sclerotherapy based on the available scientific evidence. METHODS: An international, multi-disciplinary panel of phlebologists reviewed the available scientific evidence and developed consensus where evidence was lacking or limited. RESULTS: Absolute Contraindications to sclerotherapy where the risk of harm would outweigh any benefits include known hypersensitivity to sclerosing agents; acute venous thromboembolism (VTE); severe neurological or cardiac adverse events complicating a previous sclerotherapy treatment; severe acute systemic illness or infection; and critical limb ischaemia. Relative Contraindications to sclerotherapy where the potential benefits of the proposed treatment would outweigh the risk of harm or the risks may be mitigated by other measures include pregnancy, postpartum and breastfeeding; hypercoagulable states with risk of VTE; risk of neurological adverse events; risk of cardiac adverse events and poorly controlled chronic systemic illness. Conditions and circumstances where Warnings and Precautions should be considered before proceeding with sclerotherapy include risk of cutaneous necrosis or cosmetic complications such as pigmentation and telangiectatic matting; intake of medications such as the oral contraceptive and other exogenous oestrogens, disulfiram and minocycline; and psychosocial factors and psychiatric comorbidities that may increase the risk of adverse events or compromise optimal treatment outcomes. CONCLUSIONS: Sclerotherapy can achieve safe clinical outcomes provided that (1) patient-related risk factors and in particular all material risks are (1a) adequately identified and the risk benefit ratio is clearly and openly discussed with treatment candidates within a reasonable timeframe prior to the actual procedure; (1b) when an individual is not a suitable candidate for the proposed intervention, conservative treatment options including the option of 'no intervention as a treatment option' are discussed; (1c) complex cases are referred for treatment in controlled and standardised settings and by practitioners with more expertise in the field; (1d) only suitable individuals with no absolute contraindications or those with relative contraindications where the benefits outweigh the risks are offered intervention; (1e) if proceeding with intervention, appropriate prophylactic measures and other risk-mitigating strategies are adopted and appropriate follow-up is organised; and (2) procedure-related risk factors are minimised by ensuring the treating physicians (2a) have adequate training in general phlebology with additional training in duplex ultrasound, procedural phlebology and in particular sclerotherapy; (2b) maintain their knowledge and competency over time and (2c) review and optimise their treatment strategies and techniques on a regular basis to keep up with the ongoing progress in medical technology and contemporary scientific evidence.

Skin necrosis following sclerotherapy. Part 2: Risk minimisation and management strategies.

Tissue necrosis is a serious but rare complication of sclerotherapy. Early detection and targeted management are essential to prevent progression and minimise serious complications. In the first instalment of this paper, we reviewed the pathogenic mechanisms of post-sclerotherapy necrosis. Here, we describe risk minimisation and management strategies.Risk factors must be addressed to reduce the chance of necrosis following sclerotherapy. These may be treatment-related including poor choice of sclerosant type, concentration, volume or format, poor injection technique, suboptimal ultrasound visualisation and treatment of vessels in high-risk anatomical areas. Risk factors specific to individual patients should be identified and optimised pre-operatively.Tissue necrosis is more likely to occur with extravasation of irritant sclerosants such as absolute alcohol, sodium iodide, bleomycin and hypertonic saline, whereas extravasation of foam detergent sclerosants rarely results in tissue loss. Proposed treatments for extravasation of irritant sclerosants include infiltration of an isotonic fluid and hyaluronidase. Management of inadvertent intra-arterial injections may require admission for neurovascular observation and monitoring for ischaemia, intravenous systemic steroids, anticoagulation, thrombolysis and prostanoids infusion when required. Treatment of veno-arteriolar reflex vasospasm (VAR-VAS) necrosis follows the same protocol involving systemic steroids but rarely requires hospital admission and may not require anticoagulation.In general, treatment of post-sclerotherapy necrosis is challenging and most proposed treatment measures are not evidence-based and only supported by anecdotal personal experience of clinicians. Despite all measures, once the necrosis has set in, it is very difficult to reverse the process and all measures described here may only be useful in prevention of progression and extension of the ulceration.Mid to long-term measures include addressing exacerbating factors, management of medical and psychosocial comorbidities, treatment of secondary infections and referrals to relevant specialists. All ulcers should be managed with compression and prescribed dressing regimes in line with the healing stage of the ulcer.

Skin necrosis following sclerotherapy. Part 1: Differential diagnosis based on classification of pathogenic mechanisms.

Background: Tissue necrosis is a significant but uncommon complication of sclerotherapy. The pathogenic mechanisms of this often-debilitating complication have been poorly described in the literature.Purpose: To elucidate the pathological mechanisms, we propose a morphological approach to classify sclerotherapy-induced skin necrosis into two categories of round and stellate (star-like) necrosis.Research Design: Comprehensive literature review was conducted.Results: Round necrosis is typically caused by extravasation of sclerosants. It typically presents as an ulcer with smooth and non-geographic borders. Historically, extravasation has been cited as the main cause of sclerotherapy-related necrosis. While this may be the case with osmotic or irritant sclerosants, it is far less likely with the use of detergent agents particularly in the foam format.The more commonly encountered pattern of stellate necrosis is an ischaemic ulcer secondary to arterial/arteriolar occlusion. In contrast to round necrosis, stellate necrosis follows an intra-vascular injection of sclerosants such as an inadvertent intra-arterial injection. But more frequently, stellate necrosis may follow a perfectly executed intra-venous or intra-telangiectatic delivery of sclerosants. Several pathogenic pathways can be considered. The physiologic response of veno-arteriolar reflex vasospasm (VAR-VAS) is possibly the most frequent pathway. It follows a high-pressure injection of the sclerosant in a target vein resulting in a rapid rise of intravenous pressures which in-turn would trigger a sympathetic neuronal reflex vasospasm of the pre-capillary sphincters and a corresponding opening of the normally closed arterio-venous anastomoses (AVAs). This communication would allow entry of the sclerosing agent into the arteriolar side of the circulation resulting in arteriolar occlusion and infarction of the corresponding skin. Similarly, an intravenous administration of sclerosants in the vicinity of defective boundary valves or persistently open AVAs can result in the entry of detergent agents into the arteriolar side of the microvasculature causing an ischemic stellate ulcer.Conclusions: In this first instalment of these two-part series, we review the pathogenic mechanisms of post-sclerotherapy necrosis. In the second instalment, we describe risk minimisation and management strategies.

An analysis of the prevalence of osteoporosis and osteopenia in patients with epidermolysis bullosa: A cross-sectional study.

BACKGROUND: Epidermolysis bullosa (EB) patients have multiple risk factors for osteoporosis. There is limited literature describing the prevalence of bone health in EB, particularly in adults and less severe EB types. OBJECTIVES: To investigate the prevalence of osteopenia or osteoporosis in EB patients from the Australasian Epidermolysis Bullosa Registry (AEBR). METHODS: Of 417 AEBR patients, 72 underwent a dual energy X-ray absorptiometry scan. Bone mineral density (BMD) T and Z-scores, EB Disease Activity and Scarring Index (EBDASI), and Quality of Life in EB (QOLEB) scores were obtained. RESULTS: T-scores of RDEB patients were significantly lower than the diagnostic cut-off value for osteopenia. EBDASI and QOLEB scores were inversely correlated with Z-scores. The prevalence of osteoporosis in adults was 75% in severe EB types (RDEB and JEB). In adults with less severe types (EBS and DDEB), the prevalence of osteopenia was 50% and 33%, respectively. CONCLUSIONS: This is the largest study of osteoporosis in EB to date and the first to include adult patients with EBS. The high prevalence of osteoporosis and osteopenia identified in these patients warrants larger, collaborative international studies. Nevertheless, EB patients with high disease severity and QOL scores, irrespective of type, should receive early osteoporosis screening and prophylaxis.

Outcomes and Predictors for Re-stenosis of Esophageal Stricture in Epidermolysis Bullosa: A Multicenter Cohort Study.

BACKGROUND: Esophageal strictures are the common gastrointestinal complications in patients with epidermolysis bullosa (EB) requiring dilation. There is limited information on the best type of intervention, outcomes, and predictors for re-stenosis. OBJECTIVES: We aimed to investigate the frequency, clinical presentation of esophageal strictures in EB patients, and to ascertain the predictors of re-stenosis. METHODS: We conducted a retrospective, multicenter cohort study involving 7 specialized, international EB centers on patients who were 0 to 50 years of age. Descriptive statistics and hazard risks for re-stenosis were calculated. RESULTS: We identified 125 patients with 497 esophageal stricture episodes over a mean period of observation of 17 (standard deviation [SD] = 11.91) years. Dilations were attempted in 90.74% of episodes, using guided fluoroscopy 45.23%, retrograde endoscopy 33.04%, and antegrade endoscopy 19.07%. Successful dilation was accomplished in 99.33% of attempts. Patients experienced a median of 2 (interquartile range [IQR]: 1-7) stricture episodes with a median interval between dilations of 7 (IQR: 4-12) months. Predictors for re-stenosis included: number of strictures (2 vs 1 stricture: χ = 4.293, P = 0.038, hazard ratio [HR] = 1.294 (95% confidence interval [CI]: 1.014--1.652 and 3 vs 1 stricture:χ = 7.986, P = 0.005, HR = 1.785 [95% CI: 1.194, 2.667]) and a long (≥1 cm) segment stricture (χ = 4.599, P = 0.032, HR = 1.347 (95% CI: 1.026--1.769). Complications were more common with the endoscopic approach (8/86, antegrade endoscopy; 2 /149, retrograde endoscopy vs 2/204, fluoroscopy; χ = 17.39, P-value <0.000). CONCLUSIONS: We found excellent dilation outcomes irrespective of the dilation procedure; however, with higher complications in the endoscopic approach. Long (>1 cm) segment involvement and multiple locations were predictive of stricture reoccurrence.

Deep vein sclerosis following sclerotherapy: Ultrasonic and d-dimer criteria.

OBJECTIVES: The aim of sclerotherapy is to induce fibrosclerosis of superficial veins. We postulated that inadvertent entry of sclerosants into deep veins can result in sclerotic occlusion, deep vein sclerosis, a non-thrombotic process distinct from spontaneous deep vein thrombosis. The aim of this study was to assess the role of d-dimer in differentiating between deep vein sclerosis and deep vein thrombosis. METHODS: Proximal trunks of great and small saphenous veins were treated with endovenous laser ablation. Venous tributaries and perforators were treated with foam ultrasound guided sclerotherapy. Ultrasound studies of lower limb deep veins were performed before and one week after the procedures, to detect deep vein occlusions (DVOs). d-dimer levels were measured for DVOs and long-term ultrasound studies monitored the recanalisation rates. RESULTS: In a six-year period, 9143 procedures were performed in 1325 patients for bilateral varicose veins. This included 1124 endovenous laser ablation and 8019 foam ultrasound guided sclerotherapy procedures. A total of 259 DVOs (2.83%) were identified on ultrasound which included 251 deep vein sclerosis (2.74%), seven deep vein thrombosis (0.07%) and one endovenous heat-induced thrombosis (EHIT, 0.08%). d-dimer values <0.5 µg/mL excluded deep vein thrombosis s, 0.5-1.0 µg/mL were more likely to be associated with deep vein sclerosis and >1.0 µg/mL were a more likely to be associated with deep vein thrombosis. Lower sclerosant concentrations and higher foam volumes were associated with increased risk of DVO (p < .0001). No significant relationship was found between DVO and gender or thrombophilia. Deep vein thrombosis and EHIT cases but not deep vein sclerosis patients were anticoagulated. None had thromboembolic complications. Patients were followed up for a median of 299 days (37-1994 days). Recanalisation rates were 71.1% for deep vein sclerosis (92.3% competent) and 71.4% for deep vein thrombosis (60.0% competent). CONCLUSIONS: Deep vein sclerosis is a relatively benign clinical entity distinct from deep vein thrombosis and does not require anticoagulation. Majority of affected veins on long-term follow-up regain patency and competence. d-dimer can be used to assist in differentiating deep vein sclerosis from deep vein thrombosis.

Granuloma formation following cyanoacrylate glue injection in peripheral veins and arteriovenous malformation.

Background: Cyanoacrylate adhesive closure is a technically simple alternative to endothermal ablation of peripheral veins. N-butyl cyanoacrylate is delivered via catheters or by percutaneous injection resulting in occlusion of target veins. The local tissue reaction or the systemic immune response that may follow have not been characterised. Aim: To characterise the late local tissue reaction to N-butyl cyanoacrylate glue injected in peripheral vessels. Methods: Biopsies were obtained from two patients. In patient one, distal tributaries of the great saphenous vein were injected with VenaBlock™ glue under ultrasound guidance. Ultrasound-guided incisional biopsies were performed at one week, six weeks and 12 months. In patient two, a peripheral arterio-venous malformation was injected with Venablock™ and biopsy was performed 12 months later. Histological analysis was performed using haematoxylin and eosin and immunofixation with CD-4, CD-31, CD-34, CD-68 and D2-40. Results: Echogenic material with a strong shadow artefact consistent with the injected N-butyl cyanoacrylate was observed on ultrasound on all follow-up occasions. Biopsies taken at one week showed intravascular glue without histiocytes. Biopsies at six weeks showed isolated foreign body histiocytes coating intravascular fibrillary glue spicules but no granuloma formation. The one-year biopsies showed extravascular changes including fibrosis, lymphoid aggregates and multiple extravascular foreign body cavitated granulomas. Some vessel lumens contained residual spicules of glue but no intravascular granulomas. The extravascular granulomas were deeply located, asymptomatic and not complicated by clinical ulceration. Histologically, there was no evidence of transepidermal elimination. Conclusion: Extravascular foreign body cavitated granulomas containing spicules of glue with fibrosis and lymphoid aggregates occur as a delayed finding following the use of N-butyl cyanoacrylate.

Atypical Disseminated Variant of Galli-Galli Disease: A Review of the Literature.

Galli-Galli disease (GGD) is a rare genodermatoses within the group of reticulated pigmentary disorders of the skin. Traditionally, its clinical presentation is identical to that of Dowling-Degos disease (DDD), with the additional feature of acantholysis on histopathological examination. We have reviewed the published cases of GGD to provide further support for the hypothesis that in fact, 2 phenotypes of GGD exist: the characteristic flexural GGD associated with KRT5 mutations and a disseminated variant with no mutation identified to date. A review of the literature revealed 53 reported cases of GGD. Fifteen atypical phenotype cases are described, and no KRT5 mutation has yet been identified. There is growing evidence that acantholysis is an underreported feature of DDD and that GGD and DDD are variations of the same disease, or in fact the same entity. This theory is supported by the identification of the c.418dupA missense mutation in both GGD and DDD. This review highlights that there is growing evidence that there are likely 2 clinical phenotypes of GGD with an associated genotypic correlation.

Folliculitis decalvans-like pustular plaques on the limbs sparing the scalp.

Folliculitis decalvans is a neutrophilic cicatricial alopecia characterised by progressive pustular folliculitis. Folliculitis decalvans is seen as a condition usually limited exclusively to the scalp and rarely affects the limbs. We present a case of a 63-year-old man with a 3-year history of progressive pustular folliculitis with inflammatory patches and central scarring alopecia on both forearms and a circumscribed patch on his right lower leg. His presentation, clinical course and isolation of Staphylococcus aureus together with the histopathological findings all supported a folliculitis decalvans-like pustular folliculitis limited to the limbs. Biopsies revealed follicular pustules, gross interfollicular fibrosis with plasma cells and concentric perifollicular fibrosis with lymphocytes, all features seen with folliculitis decalvans. The positive response to antibiotics combined with topical corticosteroids mirrored the response seen with scalp folliculitis decalvans. In contrast to the previously reported cases, the patient had no evidence of folliculitis decalvans on the scalp.

Angioscopy: Direct visualization of chronic venous occlusion, May-Thurner syndrome, and other applications in phlebology.

BACKGROUND: Angioscopy has been widely used in the diagnosis and management of vascular disorders and in particular in coronary artery disease. However, few applications have been developed in the diagnosis or management of venous disease. METHODS: Endovenous angioscopy was performed to explore applications of this modality in phlebology. Procedures were performed in a sterile setting. Access was obtained by ultrasound guidance and a 9F introducer sheath. An 8.5F videoscope was used to visualize target veins. Continuous saline irrigation was used to displace blood and to clear the visual field. RESULTS: Fifteen procedures were performed. We describe diagnostic or interventional applications of endovenous angioscopy that include diagnosis and characterization of chronic venous occlusion, deployment of venous stents, angioscopy-guided thrombectomy, foam sclerotherapy, and endovenous laser ablation. Chronic venous occlusion was observed to be fibrotic rather than thrombotic. CONCLUSIONS: Endoscopic imaging of the venous system has great potential to improve access and to guide endovenous interventions. Chronic venous occlusion in post-thrombotic syndrome is a fibrotic process, and chronic venous fibrosis is a better description of the type of occlusion and should replace chronic venous thrombosis.

A new indirect immunofluorescence BIOCHIP method for the serological diagnosis of bullous pemphigoid: A review of literature.

The BIOCHIP (Dermatology Mosaic 7, EUROIMMUN, Lubeck, Germany) is a novel multiplex indirect immunofluorescence technique used in the serological diagnosis of bullous pemphigoid. The BIOCHIP method combines the screening of several autoantibodies and target antigen-specific substrates in a single miniature incubation field to allow for simultaneous processing of the most common autoimmune bullous diseases autoantibodies using a single investigation. This manuscript reviews the literature on the validity of the BIOCHIP in the diagnosis of bullous pemphigoid. A systematic search for journal articles comparing the sensitivity and specificity of diagnostic tests for bullous pemphigoid patients was conducted in online databases via the Ovid SP search interfaces. The literature search generated 745 articles of which 189 were deemed relevant. Among the relevant articles, seven studies investigated the validity of the BIOCHIP indirect immunofluorescent test in the diagnosis of bullous pemphigoid. The BIOCHIP has demonstrated itself to be a specific test for BP180 (96.5-100%) and BP230 (98.3-100%), with a high sensitivity for BP180 (83.3-100%) but poor sensitivity for BP230 (24.3-66.7%). The BIOCHIP mosaic-based immunofluorescence test is potentially a simple, time- and effort-saving test that can aid in the diagnosis and screening of bullous pemphigoid. Further studies should report on the inter-rater reliability of the BIOCHIP to further validate the tool.

Merkel cell carcinoma in situ: New insights into the cells of origin.

We report a case of a 71-year-old male who presented with a small skin-coloured plaque on his cheek. Histopathology demonstrated an intraepidermal carcinoma with follicular involvement. No evidence of dermal invasion was seen. Immunohistochemical studies showed areas of positive staining for CK20, EMA and synaptophysin. Histopathology findings were found to be most consistent with a diagnosis of intraepidermal carcinoma with features of Merkel cell carcinoma in situ, in combination with a squamous cell carcinoma in situ, with follicular involvement. Recent advances and findings suggest Merkel cell polyomavirus MCPyV-positive Merkel cell carcinoma and MCPyV-negative Merkel cell carcinoma have different cells of origin from different germ layers.

Prevalence and pathogenesis of osteopenia and osteoporosis in epidermolysis bullosa: An evidence-based review.

BACKGROUND: Osteopenia or osteoporosis is one of the many comorbidities in patients with Epidermolysis Bullosa (EB). Current literature on the prevalence of osteoporosis in EB is scarce. OBJECTIVE: This review will analyse the current literature in the field of patients with compromised bone health in EB and any articles on the prevalence of such diseases in EB groups. METHODS: A systematic search for articles related to bone health and epidermolysis bullosa (EB) (1946-2017) was performed on seven databases: MEDLINE, EMBASE and EBM, PubMed, ProQuest, Scopus and Web of Science. Search terms: epidermolysis bullosa, osteop*, bone mass, bone mineral*, fracture, dual X-ray absorptiometry, vitamin D, calcium, nutrition, exercise and physical activity. Abstracts from all search results were screened, and reference scanning of the search results was performed. Eighty-three articles met the selection criteria and were considered for review. Letters to the editor and abstract-only articles were excluded. Articles were favoured based on citation count, impact factor of their journal and study sample size. The search included all languages. RESULTS: The searches yielded a total of 1309 articles including 717 duplicates. The remaining 592 articles were screened by title and abstracts. Eighty-three full-text articles were analysed. Twenty-one articles directly relating to bone health in EB were included. Three descriptive studies and one case-control study were found, indicating a need for research of larger scale. CONCLUSION: Further investigations into osteoporosis in EB, especially the milder forms of EB, are valuable in providing evidence to support guideline developments for EB bone health management.

Pemphigoid gestationis and intravenous immunoglobulin therapy.

Pemphigoid gestationis, which is also known as herpes gestationis, is a rare, pregnancy-associated, autoimmune bullous disease. Treatment depends on the severity of the disease for each patient and the safety and use of these drugs during pregnancy and breastfeeding must be taken into consideration to guide their use. We describe the therapeutic response of two cases of pemphigoid gestationis that did not respond to conventional immunosuppressive therapy or adverse effects limited their use. Both patients eventually received treatment with intravenous immunoglobulin therapy, which resulted in clinical remission. This clinical improvement with disappearance of lesions and a reduction in pruritus was paralleled in a decline in Bullous Pemphigoid Disease Activity Index activity scores, which is a validated scoring system to measure the related condition, bullous pemphigoid.

New biochip immunofluorescence test for the serological diagnosis of pemphigus vulgaris and foliaceus: A review of the literature.

The immunoassays that are available for the serological diagnosis of the more common subtypes of autoimmune blistering diseases such as pemphigus vulgaris (PV) and pemphigus foliaceus (PF) include enzyme-linked immunosorbent assay (ELISA) testing to specific antigens desmoglein (Dsg)1 and Dsg3, direct immunofluorescence (DIF), indirect immunofluorescence (IIF), and immunoblotting. A review of the literature on the biochip assay was conducted. Six studies investigated the validity of a new biochip, mosaic-based, IIF test in patients with pemphigus and demonstrated its relatively high sensitivity and specificity (Dsg3: 97.62-100%, 99.6-100%; Dsg1: 90%, 100%) in comparison with ELISA (Dsg3: 81-100%, 94-100%; Dsg1: 69-100%, 61.1-100%), and/or IIF (PV: 75-100%, 91.8-100%; PF: 67-100%) using suitable substrates. So far, validation studies of the biochip have been conducted in four countries (Germany, Italy, Turkey, and Poland) but none in the southern hemisphere. Caucasian patients were recruited as normal controls for these studies; thus, the diagnostic value of the biochip remains uncertain in population groups of other ethnicities. A range of disease control patients were recruited including patients with linear immunoglobulin A dermatosis, psoriasis, discoid lupus erythematosus, lichen planus, and noninflammatory skin diseases (e.g., squamous cell carcinoma, basal cell carcinoma, and vascular leg ulcers). Prospective studies with control patients from a diverse range of ethnicities are needed to better validate the biochip.