Hospitalization and mortality in Asian autoimmune bullous dermatosis patients: A 17-year retrospective study.

Limited data exist on the factors associated with hospitalization and mortality in Asian inpatients with autoimmune bullous dermatoses (AIBDs). This study aimed to elucidate the risk factors affecting hospitalization and mortality rates in Asian patients with AIBDs. A retrospective analysis of patients with AIBDs treated at Siriraj Hospital during a 17-year period was performed using the International Classification of Diseases 10th revision codes. The characteristics of inpatients and outpatients were compared, and mortality rates and associated factors were identified. The study included 360 AIBD patients (180 inpatients, 180 outpatients). Inpatients were significantly younger than outpatients. The identified risk factors for hospitalization were malignancy (odds ratio [OR] 2.83, 95% confidence interval [CI] 1.13-8.04; p = 0.034), moderate to severe disease (OR 2.52, 95% CI 1.49-4.34; p < 0.001), systemic corticosteroid use ≥15 mg/day (OR 2.27, 95% CI 1.21-4.41; p = 0.013) and oral cyclophosphamide treatment (OR 9.88, 95% CI 3.82-33.7; p < 0.001). Kaplan-Meier analysis revealed mortality rates of 26%, 36% and 39% for inpatients with pemphigus at 1, 3 and 5 years, respectively. For inpatients with pemphigoid, the corresponding rates were 28%, 38% and 47%. Infections, particularly pneumonia, were the predominant cause of death in both conditions. This study confirmed that both Asian ethnicity and healthcare disparities may be correlated with adverse outcomes in patients with AIBDs. Pemphigus mortality rates were substantially greater in Asian patients than in Caucasian patients. Continuous monitoring of factors contributing to hospitalization and mortality is imperative to improve treatment outcomes.

Comparative analyses of various IgE-mediated and non-IgE-mediated inducers of mast cell degranulation for in vitro study.

In vitro investigations of mast cell (MC) degranulation are essential for studying many diseases, particularly allergy and urticaria. Many MC-degranulation inducers are currently available. However, there is no previous systematic comparative analysis of these available inducers in term of their efficacies to induce MC degranulation. Herein, we performed systematic comparisons of efficacies of five well-known and commonly used MC-degranulation inducers. RBL-2H3 cells were sensitized with 50 ng/ml anti-DNP IgE or biotinylated IgE followed by stimulation with 100 ng/ml DNP-BSA or streptavidin, respectively. For non-IgE-mediated inducers, the cells were treated with 5 µg/ml substance P, compound 48/80, or A23187. At 15-, 30-, 45- and 60-min post-induction, several common MC-degranulation markers (including intracellular [Ca2+], ß-hexosaminidase release, tryptase expression by immunofluorescence staining, cellular tryptase level by immunoblotting, secretory tryptase level by immunoblotting, CD63 expression by immunofluorescence staining, and CD63 expression by flow cytometry) were evaluated. The data showed that all these markers significantly increased after activation by all inducers. Among them, A23187 provided the greatest degrees of increases in intracellular [Ca2+] and ß-hexosaminidase release at all time-points and upregulation of CD63 at one time-point. These data indicate that all these IgE-mediated (anti-DNP IgE/DNP-BSA and biotinylated IgE/streptavidin) and non-IgE-mediated (substance P, compound 48/80, and A23187) inducers effectively induce MC degranulation, while A23187 seems to be the most effective inducer for MC degranulation.

Dermatologists Should Encourage and Resume Photoprotection in Patients with Photodermatoses and Melasma Following the COVID-19 Pandemic.

Background: The COVID-19 pandemic may alter individuals' perspectives and behaviors regarding sun exposure and photoprotection. Presently, there is a paucity of information about how the pandemic influences photoprotection in photodermatoses and melasma. Objective: To compare the photoprotection attitudes and behaviors of individuals with photodermatoses and melasma with those of a control group (other dermatologic patients) before and during the COVID-19 pandemic. Methods: A questionnaire-based, cross-sectional study was conducted among Thai patients with photodermatoses, melasma, and other dermatological conditions between August 2021 and November 2022. Each of the three groups consisted of 35 patients. Results: Among the 105 patients, 81.9% were female, and the mean age was 45.83 years. Over 80% of individuals who used surgical masks daily for 4 to 8 hours believed that these masks provided skin protection from the sun. The duration of sun exposure and the frequency of photoprotection practices decreased significantly in all groups during the COVID-19 pandemic compared to the pre-pandemic period. Sunscreen was the most prevalent form of photoprotection, but its usage declined considerably during the pandemic. Upon physical examination, the photodermatoses and control groups exhibited unchanged skin conditions, while the patients with melasma demonstrated improved skin conditions during the pandemic. Conclusion: Owing to increased indoor activities and the perception that face masks could block sunlight, the COVID-19 pandemic led to notable reductions in sun exposure and photoprotective practices. Despite the absence of photodermatoses exacerbation and the observed improvements in patients with melasma, consistent and effective photoprotection must continue to be promoted.

Effect of Platelet-Rich Plasma and other Key Factors on Hair Follicle Preservation.

Background: Hair graft preservation is an important factor that influences graft survival in hair transplantation. Objective: To investigate the benefits of adding platelet-rich plasma (PRP), and the effect of different storage solutions and temperatures on hair follicle preservation. Materials and Methods: This randomized-controlled study included 10 androgenetic alopecia patients who underwent hair transplantation. Forty-five hair grafts were collected from each patient and then randomized to 8 different culture conditions for 7 days. Hair grafts were cultured in Williams' Medium E or Ringer's lactate solution (RLS) at either 4°C or 37°C, and with or without 10% PRP supplementation. Results: In vitro hair growth in Williams' Medium E was significantly greater than in RLS. The 37°C temperature condition was found to be significantly better than the 4°C condition. The growth of hair grafts cultured with PRP was not significance difference from those without PRP. However, immunofluorescence staining for cytokeratin 15 showed greater expression in hair graft cultured with PRP. Conclusion: PRP may have a beneficial effect for preserving the viability of hair grafts. Williams' Medium E and 37°C temperature were found to be superior to RLS and 4°C relative to hair follicle growth in organ culture.

Food-Dependent Exercise-Induced Wheals/Angioedema, Anaphylaxis, or Both: A Systematic Review of Phenotypes.

BACKGROUND: Food-dependent exercise-induced allergic reactions can manifest with wheals, angioedema, and anaphylaxis, alone or in combination. OBJECTIVE: To systematically review the clinical manifestation, culprit foods and exercise, augmenting factors, comorbidities, and treatment options of each phenotype. METHODS: Using predefined search terms, we assessed and analyzed the relevant literature until June 2021. Preferred Reporting Items for Systematic Reviews and Meta-Analysis recommendations were applied to this systematic review. RESULTS: A total of 231 studies with 722 patients were included. The most common phenotype was anaphylaxis with wheals, angioedema, or both, reported in 80% of patients. This was associated with a higher number of anaphylactic episodes, augmenting factors, and use of on-demand antihistamine compared with the least common phenotype, anaphylaxis without wheals or angioedema, reported in 4% of patients. Anaphylaxis with wheals/angioedema was also associated with distinct characteristics compared with stand-alone wheals, angioedema, or both, in 17% of patients. Patients with anaphylaxis were older at the time of disease onset, less often had a history of atopy, showed more positive results in response to food and exercise provocation tests, had a more restricted spectrum of culprit foods, and more often used on-demand epinephrine. CONCLUSIONS: The three phenotypes of allergic reactions to food and exercise differ in clinical characteristics, triggers, and response to treatment. Knowledge of these differences may help with patient education and counseling as well as disease management.

The validity and reliability of a Thai version of the Angioedema Control Test: Which recall period is preferable?

BACKGROUND: The Angioedema Control Test (AECT) is a questionnaire that monitors disease control in patients with angioedema, with a recall period of 4 weeks (AECT-4wk) or 3 months (AECT-3mo). OBJECTIVE: This study investigated the psychometric properties of a Thai version of the AECT. METHODS: Of 54 patients, 46, 5, 2, and 1 had recurrent angioedema with chronic spontaneous urticaria, hereditary angioedema, idiopathic histaminergic angioedema, and acquired angioedema due to C1 esterase inhibitor deficiency, respectively. The AECT, Angioedema Activity Score (AAS), Dermatology Life Quality Index (DLQI), Angioedema Quality of Life Questionnaire (AE-QoL), and anchors for disease control (numeric rating scale [NRS] and patient global assessment-Likert scale [PatGA-LS]) were used. The patients rated the efficacy of their treatment. RESULTS: Fifty-four and 47 patients completed the AECT-4wk and AECT-3mo, respectively. Both AECT versions showed significant correlations with disease activity (AAS, r = 0.6-0.8), disease control (NRS and PatGA-LS, r = 0.7-0.9), and quality of life impairment (DLQI and AE-QoL, r = 0.6-0.8). Higher correlations were found for the AECT-4wk than for the AECT-3mo. Excellent internal consistency (alpha = 0.98 and 0.97, respectively) and intraclass correlation (0.96 and 0.94, respectively) were found. A cutoff ≥ 10 was confirmed to identify patients with well-controlled disease for both AECT versions (AUCs = 0.89 and 0.97). CONCLUSIONS: The Thai version of the AECT is a valid and reliable tool for clinical practice. Due to the shorter recall period, the AECT-4wk may be more accurate than, and preferable to, the AECT-3mo. A cutoff ≥ 10 should be used to identify patients with well-controlled disease.

Do Antinuclear Antibodies Influence the Clinical Features of Chronic Spontaneous Urticaria?: A Retrospective Cohort Study.

Background: Antinuclear antibody (ANA) is often used as a screening test for autoimmune comorbidities in patients with chronic spontaneous urticaria (CSU). However, the relationship of ANA status and the clinical course of the disease have not been fully described. Objectives: To compare clinical features of CSU patients who are positive and negative for ANA. Methods: This was a retrospective cohort study that enrolled CSU patients attending the Urticaria Clinic at Siriraj Hospital from 2013 to 2019. Demographics, clinical characteristics, laboratory investigations, and treatments were collected until July 2021. All patients were investigated for ANA. Clinical feature data was compared between CSU patients with positive ANA and negative ANA groups using the 2-sample t-test and the Mann-Whitney U test for quantitative variables. The chi-squared test or Fisher's exact test was conducted to explore the association of qualitative variables. Disease relapse and remission were analysed via Kaplan-Meier survival analysis. Results: Of 323 CSU patients, 31% had positive ANA. There were no significant differences in disease severity or impairment in quality of life. Patients with a positive ANA test had significantly lower prevalence of allergic rhinitis (p = .048) and significantly higher level of erythrocyte sedimentation rate (p = 0.007). Kaplan-Meier survival analysis showed that 2% of ANA positive CSU patients achieved remission status after one year and 28% did so after five years. There was no statistically significant difference in time to remission and time to relapse between ANA-positive and negative groups. Conclusion: Positive ANA in CSU patients could not indicate the differences in main disease characteristics from the ANA-negative CSU patients. Investigation for ANA may be useful in CSU patients who are suspected of having autoimmune diseases.

Natural history and clinical course of patients with dermographism in a tropical country: a questionnaire-based survey.

Background: Dermographism is the most common form of chronic inducible urticaria. However, the natural history and clinical course of patients with dermographism in tropical countries has not fully been described. Objective: To examine clinical features, natural history and clinical course of dermographism in Thai patients according to their experiences. Methods: A cross-sectional, internet-based survey was conducted in 2021. All study respondents completed a 45-item questionnaire that was circulated on social media regarding dermographism. Results: Among the 2,456 respondents who reported dermographism, 1,900 had symptomatic dermographism (SD), while 556 had simple dermographism (SimD). Of the respondents who reported SD and SimD, the female to male ratio was 2.2:1 and 2.4:1, respectively. The median age of the first episode of SD and SimD was 16 and 15 years, respectively. Older age, greater body weight, cardiovascular diseases, allergic conjunctivitis, atopic dermatitis, changes in temperature, and family history of dermographism were all factors linked to an increased probability of SD. Half of the respondents with SD reported moderate itch severity. Moreover, about half of SD and almost all of SimD respondents let the wheal resolve on its own. Second generation H1-antihistamines were most commonly prescribed while over-the-counter medicines were taken by both SD and SimD respondents. Conclusion: This survey highlights several aspects of dermographism in Thai patients which can be useful for healthcare providers. SD is troublesome and affects the quality of life of many patients, leading some to seek medication themselves.

Disease activity of patients with chronic urticaria receiving COVID-19 vaccines.

BACKGROUND: Cutaneous adverse events after receiving a COVID-19 vaccine were identified. The disease activity of urticaria after a COVID-19 vaccine has never been explored in chronic urticaria patients. OBJECTIVE: To evaluate disease activity of chronic urticaria after receiving a COVID-19 vaccine. METHODS: A prospective cross-sectional study was conducted in chronic urticaria patients aged 18 or above who visited Siriraj Hospital between July and September 2021, and received the first and second dose of COVID-19 vaccine. The status prior to vaccination, including disease activity, disease control and disease severity was assessed by a urticaria activity score over seven days, urticaria control test, and modified medication score. The disease activity after vaccination was recorded. RESULTS: A total of 130 patients with a mean age of 45.9 ± 14.7 were enrolled in this study. Adenoviral and inactivated vaccines were administered to 85 (65.4%) and 45 patients (34.6%), respectively. Exacerbation was reported in 20 cases (15.4%) after the first dose and 17 cases (13.1%) after the second dose. Nine patients (45%) reported exacerbation after both the first and second dose. The majority of patients only had wheal, while three patients reported wheal with angioedema. No anaphylaxis was reported. Factor predicting exacerbation was concurrent thyroid disease (aRR 2.78, p < 0.01). CONCLUSIONS: Approximately 15% of chronic urticaria patients reported exacerbation after receiving a COVID-19 vaccination. No serious events were observed. Chronic urticaria patients should be vaccinated against COVID-19 after a discussion of the risk of disease flare-up.

Prevalence, Clinical Manifestations, Treatment, and Clinical Course of Chronic Urticaria in Elderly: A Systematic Review.

Purpose: Data specific to the epidemiology, clinical features, and management of chronic urticaria (CU) in the geriatric population remain limited and not well understood. We aim to systematically review the prevalence, clinical manifestations, treatment, and clinical course of elderly patients with CU. Patients and methods: Original articles that included data of elderly (aged >60 years) with CU that were published until February 2021 were searched in PubMed, Scopus, and Embase using predfefined search terms. Related articles were evaluated according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses recommendations. Results: Among the included 85 studies and 1,112,066 elderly CU patients, most (57.4%) were women. The prevalence of elderly CU in the general population ranged from 0.2-2.8%, and from 0.7-33.3% among all CU patients. Compared to adult CU, elderly CU patients had a higher percentage of wheal alone (73.9%), and lower rate of positive autologous serum skin test and atopy. Gastrointestinal diseases were the most common comorbidity (71.9%), and there was a high rate of malignancies and autoimmune diseases. Second generation H1-antihistamines were commonly used, and achievement of complete control was most often reported. Omalizumab was prescribed in 59 refractory patients, and a significant response to treatment was reported in most patients. The treatment of comorbidities also yielded significant improvement in CU. Conclusion: Elderly CU was found to be different from adult CU in both clinical and laboratory aspects. H1- antihistamines are effective as first-line therapy with minimal side-effects at licensed doses. Treatment of secondary causes is important since the elderly usually have age-related comorbidities.

Systematic comparisons of various markers for mast cell activation in RBL-2H3 cells.

Mast cell activation plays a key role in various allergic diseases and anaphylaxis. Several methods/techniques can be used for detection of mast cell activation. However, there was no previous systematic evaluation to compare the efficacy of each method/technique. The present study thus systematically compared various markers for mast cell activation induced by IgE cross-linking. The widely used RBL-2H3 mast cells were sensitized with anti-DNP (dinitrophenyl) IgE overnight and activated with DNP-BSA (bovine serum albumin) for up to 4 h. The untreated cells and those with anti-DNP IgE sensitization but without DNP-BSA activation served as the controls. Intracellular calcium level gradually increased to ~2-fold at 1 h, reached its peak (~5-fold) at 2 h, and returned to the basal level at 3-h post-activation. The increases in cellular tryptase level (by Western blotting) (~0.3- to 0.4-fold) and average cell size (~2.5-fold) and decrease of nucleus/cytoplasm ratio (~0.4- to 0.5-fold) were marginal at all time-points. By contrast, ß-hexosaminidase release and CD63 expression (by both flow cytometry and immunofluorescence detection/localization), secreted tryptase level (by Western blotting), and tryptase expression (by immunofluorescence detection/localization) stably and obviously increased (~10-fold as compared with the untreated control and sensitized-only cells or detectable only after activation). Based on these data, the stably obvious increases (by ≥ 10-fold) in ß-hexosaminidase release, CD63 expression (by both flow cytometry and immunofluorescence staining), secreted tryptase level (by Western blotting), and tryptase expression (by immunofluorescence staining) are recommended as the markers of choice for the in vitro study of mast cell activation using RBL-2H3 cells.

Evidence for histamine release in chronic inducible urticaria - A systematic review.

Background: Chronic inducible urticaria (CIndU) constitutes a group of nine different CIndUs in which pruritic wheals and/or angioedema occur after exposure to specific and definite triggers. Histamine released from activated and degranulating skin mast cells is held to play a key role in the pathogenesis of CIndU, but evidence to support this has, as of yet, not been reviewed systematically or in detail. We aim to characterize the role and relevance of histamine in CIndU. Methods: We systematically searched 3 electronic databases (PubMed, Scopus, and Embase) for studies that reported increased serum or skin histamine concentration (direct evidence) or in vitro or ex vivo histamine release (indirect evidence) following trigger exposure. Results: An initial total of 3,882 articles was narrowed down to 107 relevant studies of which 52 were in cold urticaria, 19 in cholinergic urticaria, 14 in heat urticaria, 10 in contact urticaria, 7 each in solar urticaria and vibratory angioedema, 4 each in symptomatic dermographism and aquagenic urticaria, and 3 in delayed pressure urticaria. The results of our review support that histamine has a key pathogenic role in the pathogenesis of all CIndUs, but it is not the sole mediator as evidenced by the often poor relationship between the level of histamine and severity of symptoms and the variable clinical efficacy of H1-antihistamines. Conclusions: Histamine released from skin mast cells is a key driver of the development of signs and symptoms and a promising therapeutic target in CIndU.

Food-Dependent Exercise-Induced Wheals, Angioedema, and Anaphylaxis: A Systematic Review.

BACKGROUND: Food-dependent exercise-induced wheals, angioedema, and anaphylaxis remain insufficiently characterized. OBJECTIVE: We systematically reviewed the literature on clinical manifestations, laboratory investigations, culprit foods, triggering exercise, comorbidities, and treatment outcomes. METHODS: Using predefined search terms and Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) recommendations, we searched 3 electronic databases to identify relevant literature published before July 2021. RESULTS: Of 722 patients (median age 25 years; 55.4% male) from 231 studies (43 cohort studies, 15 cases series, and 173 case reports), 79.6% and 3.7% had anaphylaxis with and without wheals and/or angioedema, respectively. The remaining 16.6% had wheals and/or angioedema without anaphylaxis. The duration from eating to exercising and from exercising to symptom onset ranged from 5 minutes to 6 hours (median 1 hour) and from 5 minutes to 5 hours (median 30 minutes), respectively, and virtually all patients exercised within 4 hours after eating and developed symptoms within 1 hour after exercising. Wheat was the most common culprit food. Running was the most common trigger exercise. Most patients were atopic, and 1 in 3 had a history of urticaria. Aspirin and wheat-based products were the most frequent augmenting factors. On-demand antihistamines, corticosteroids, and epinephrine were commonly used and reported to be effective. Patients who stopped eating culprit foods before exercise no longer developed food-dependent exercise-induced allergic reactions. CONCLUSIONS: Food-dependent exercise-induced allergic reactions are heterogeneous in their clinical manifestations, triggers, and response to treatment. Patients benefit from avoidance of culprit foods before exercise, which highlights the need for allergological diagnostic workup and guidance.

A Systematic Review of Aquagenic Urticaria-Subgroups and Treatment Options.

BACKGROUND: Aquagenic urticaria (AquaU) is a rare variant of chronic inducible urticaria where wheals occur after skin contact with water. Information on clinical manifestations and treatment outcomes is limited, which makes the management of AquaU challenging. OBJECTIVE: To systematically review disease features and relevant triggers of AquaU and patients' response to treatment. METHODS: Related articles were searched by use of the terms "aquagenic urticaria" and "aquagenic angioedema" until June 2021 and reviewed according to Preferred Reporting Items for Systematic Reviews and Meta-Analysis recommendations. RESULTS: A total of 77 patients with AquaU were investigated in 59 studies including 47 case reports and 12 case series. AquaU predominantly presented in women (47 patients, 61%), and the mean age of onset of the disease was 19.6 ± 10.8 years (range: 0-54 years). Wheals commonly occurred in localized areas and regardless of the water temperature. Based on the reviewed evidence, AquaU can be classified as familial AquaU (FAquaU, 18.2%) and acquired AquaU (AAquaU, 81.8%). Although many treatments were used in both subtypes of AquaU, the use of second-generation H1 antihistamines (2ndAH1) was reported most often to achieve marked improvement in both subtypes. The use of topical therapies in AquaU, which most commonly use hydrophobic vehicles, is poorly documented and of controversial efficiency. CONCLUSIONS: AquaU is proposed to be classified into 2 subtypes, FAquaU and the more common AAquaU. Treatment with a 2ndAH1 is recommended as the first-line treatment for both types of AquaU. Further studies are required to fill knowledge gaps.

A web-based survey on the behavior of moisturizer used in adults with and without atopic dermatitis in Thailand.

BACKGROUND: Moisturizers play an important role in restoring the skin barrier. They should be used to treat and prevent eczema, especially in atopic dermatitis (AD). OBJECTIVE: To evaluate the factors that influence selection of moisturizers in adult patients with AD and without it. Usage behavior between the two groups was also determined. METHODS: A cross-sectional web-based survey was performed. RESULTS: A total of 1,195 participants with mean age of 46.5 ± 14.5 were enrolled. Fifty participants (4.2%) met the William's criteria for AD diagnosis. Most participants reported using moisturizer every day or two times per day. A non-sticky moisturizer, followed by pleasant odor were considered important properties. For choosing a moisturizer, personal satisfaction was the most common answer given by participants. The most common locations that participants applied moisturizer were the extremities (85.1%) and face (84.9%). Physicians' suggestion was also a significant factor that led to moisturizer use by AD patients but it was not significant in the non-AD group (29.2% vs 14.7%, p = 0.007, OR 2.4). A pH of 5.5 and the anti-inflammatory property were important factors in choosing a moisturizer in the AD group. Both AD and non-AD participants preferred liquid soap over bar soap in daily life. CONCLUSIONS: Our results showed that most participants have basic knowledge of how to use a moisturizer. Physicians' suggestion influenced the selection of moisturizer in AD patients. Thus, physicians should continue to educate in order to achieve good clinical outcomes.

The validity and reliability of the Thai-version of 5-D itch scale.

BACKGROUND: Pruritus is commonly associated with skin disorders. The 5-D itch scale was developed as a specific questionnaire for pruritus. OBJECTIVE: This study aimed to evaluate the validity, reliability, and sensitivity to change of the Thai 5-D itch scale in Thai patients. METHODS: The Thai Dermatology Life Quality Index (DLQI), patient's global assessment of disease severity (PatGA-VAS), Chronic Urticaria Quality of Life Questionnaire (CU-Q2oL), and seven-day urticaria activity score (UAS7) were evaluated as correlation with Thai 5-D itch scale. Seventy-five stable patients (42 chronic urticaria patients and 33 eczema patients), who had no change in disease severity after 4-weeks were assessed for test-retest reliability. RESULTS: Of 130 pruritus patients who were treated at Department of Dermatology, Siriraj Hospital, 65 patients were diagnosed with chronic urticaria. The others were diagnosed with eczema. The validity of Thai 5-D itch scale correlated strongly with Thai DLQI total score (r = 0.76, p < 0.0001) and PatGA-VAS (r = 0.79, p < 0.0001). The strong reliability of Thai 5-D itch scale was demonstrated as intraclass correlation coefficient of 0.90. The changes in Thai 5-D itch scale was correlated with the changes in PatGA-VAS and UAS7 which indicated that the Thai 5-D itch scale had good sensitivity to change (r = 0.66) and (r = 0.67), respectively. CONCLUSIONS: The Thai 5-D itch scale is a questionnaire with good validity, reliability and sensitivity to change to evaluate pruritus in Thai patients. This will support the use of 5-D itch scale in practice, in other languages.

Livedoid vasculopathy: Clinical course and long-term outcome in Asian patients with a review of the literature.

Livedoid vasculopathy (LV) is an uncommon, chronic, and recurrent thrombo-occlusive vascular disorder. Data specific to LV in Thai population remains scarce. This study aimed to evaluate the clinical course and treatment outcomes of LV in Thai patients, and to perform a literature review for studies that reported on anticoagulant treatment in LV. Seventy-four patients with a mean age of 37.6 ± 14.7 years were included. The female to male ratio was 5.2:1, and the median follow-up was 10.5 months. Most patients had primary LV disorder. Forty-eight patients were improved with treatments, with a median duration of 11.4 months. Combination treatments were commonly used, including anti-inflammatories, antiplatelets, and immunosuppressants. Add-on therapy with anticoagulant or psoralen plus ultraviolet-A (PUVA) led to disease improvement in a majority of the patients treated. Kaplan-Meier analysis demonstrated that 38.5%, 53.7%, and 57.9% would have disease improvement at 1, 2, and 3 years, respectively. Of 39 studies (n = 219) that reported on anticoagulant treatment in LV, anticoagulant drug was used as monotherapy in 104 patients. The mean duration of anticoagulant treatment was 7.2 ± 3.8 months, which led to disease improvement in 97 patients (93.3%). Bleeding side effect was found in 9 patients (8.7%). The highest incidence of LV was found among females aged 30 to 40 years. Combination therapy with anti-inflammatory drugs, antiplatelet drugs, and immunosuppressants led to disease improvement. The observed efficacy of add-on PUVA or anticoagulant is promising and should be further investigated. Further studies are needed to guide the development of an LV management guideline.