Comparison of Intralesional Sodium Stibogluconate versus Intralesional Meglumine Antimoniate for the Treatment of Leishmania major Cutaneous Leishmaniasis.

Israel is endemic for Old-World cutaneous leishmaniasis. The most common species is Leishmania major. However, the available treatment options are limited. This study's objective was to compare the authors' experience with different antimony intralesional treatments of Leishmania major cutaneous leishmaniasis. A retrospective evaluation was undertaken for cases of Leishmania major cutaneous leishmaniasis treated by pentavalent antimony in a university-affiliated medical centre in Israel. The previous treatment of intralesional sodium stibogluconate (Pentostam®) was compared with the current treatment of meglumine antimoniate (Glucantime®). One hundred cases of cutaneous leishmaniasis were treated during the study period, of whom 33 were treated with intralesional sodium stibogluconate and 67 were treated with intralesional meglumine antimoniate. The patients were 78 males and 22 females, mean age 24 (range 10-67) and there was a total of 354 skin lesions. Within 3 months from treatment, 91% (30/33) of the intralesional sodium stibogluconate group and 88% (59/67) of the intralesional meglumine antimoniate group had complete healing of the cutaneous lesions after an average of 3 treatment cycles (non-statistically significant). In conclusion, the 2 different medications have the same efficacy and safety for treating cutaneous leishmaniasis. Pentavalent antimoniate intralesional infiltration treatment is safe, effective, and well tolerated with minimal side effects for Old-World cutaneous leishmaniasis.

New-Onset Guttate Psoriasis: A Long-Term Follow-Up Study.

BACKGROUND: Guttate psoriasis (GP), a distinct variant of psoriasis, is more common in children and adolescents. The long-term course of these patients has sparsely been examined, with few studies reporting the rates of relapse, persistence, and further development of the psoriasis vulgaris phenotype. OBJECTIVES: The objective of this study was to characterize the long-term outcomes of new-onset GP and elucidate the potential factors associated with a persistent disease course. METHODS: This was a retrospective cohort study. Patients diagnosed with new-onset GP between 2009 and 2020 with a follow-up period of at least 1 year, were enrolled. The examinees were evaluated by dermatologists. Detailed data retrieved from the examinees' medical files included demographics, disease characteristics, treatment, and comorbidities. A structured telephone questionnaire was used to determine the current psoriasis status: type, severity, and extent. At the end of follow-up, patients with a persistent disease course, defined as having lesions at least a year after disease onset, were compared with patients in complete remission without further psoriasis symptoms. RESULTS: A total of 120 patients (mean age 28.8 years [±15.2], 58.3% women) with new-onset GP flare were identified. At the end of follow-up period (mean 6.2 years [±3.1]), 49.1% (n = 59) of the patients reported active persistent psoriasis. A switch to the psoriasis vulgaris phenotype occurred in 17.5% (n = 21) of the study cohort. Persistent psoriasis was associated with male sex (OR = 2.1, p < 0.05), multiple disease flares (>3; OR = 9.1, p < 0.001), switch to the vulgaris phenotype (OR = 4.16, p < 0.001), and palmoplantar involvement (OR = 5.2, p < 0.01). CONCLUSION: A persistent disease course is common among patients with new-onset GP, with most retaining their guttate phenotype throughout the disease course. Persistency was associated with male sex, multiple GP flares, switching to the vulgaris phenotype, and palmoplantar involvement.

Prevalence, Spectrum and Clinical Implications of Malignancies in Patients with Bullous Pemphigoid.

Current research on the malignancy rate and spectrum of malignancies in patients with bullous pemphigoid is contradictory. The aims of this study were to determine the prevalence and spectrum of malignancy in patients with bullous pemphigoid and to compare demographic, clinical, therapeutic and outcome data between bullous pemphigoid patients with and without malignancy. This retrospective cohort study enrolled 335 patients (194 women and 141 men; mean age at diagnosis of bullous pemphigoid 77.5 ± 12 years) followed up at an Israeli tertiary centre between January 2009 and December 2019: 107 (32%) had malignancy and 228 (68%) did not. Malignancy occurred before and after bullous pemphigoid diagnosis in 82 (77%) and 25 (23%) patients, respectively. Bullous pemphigoid patients with cancer were older (p = 0.02) and had a higher mortality rate (p < 0.0001) than those without malignancy. The 2 groups did not differ in terms of sex, comorbidities, or clinical characteristics. Those who developed malignancy before bullous pemphigoid were younger than those who developed malignancy after bullous pemphigoid (mean age 69.3 vs 82.4 years, p < 0.0001). Overall malignancy rates did not differ between patients with bullous pemphigoid and the general population; therefore, comprehensive malignancy workup may be unnecessary. However, patients with bullous pemphigoid had a greater risk of melanoma (10.7% vs 4.3%, p = 0.0005); therefore, routine skin screening may be recommended.

Skin Eosinophil Counts in Bullous Pemphigoid as a Prognostic Factor for Disease Severity and Treatment Response.

Dermal infiltration of eosinophils and eosinophilic spongiosis are prominent features of bullous pemphigoid lesions. Although several observations support the pathogenic role of eosinophils in bullous pemphigoid, few studies have examined the impact of skin eosinophil counts on disease severity and treatment response. This retrospective study assessed the association between eosinophil counts in skin biopsy samples of 137 patients with bullous pemphigoid and their demographic characteristics, comorbidities, disease severity, and treatment response. There was no relationship between eosinophil count and age, sex, or disease severity at disease onset. There was a positive relationship between eosinophil counts and neurological comorbidity and a negative relationship between eosinophil counts and treatment response. At all follow-up points patients with no tissue eosinophils had a better response to treatment than patients with any tissue eosinophil count. In conclusion, skin eosinophil counts in patients with bullous pemphigoid are not correlated with disease severity at onset, but can serve as a negative prognostic marker for treatment response.

Bullous pemphigoid and dipeptidyl peptidase-4 inhibitors: evaluation of clinical course and treatment response.

BACKGROUND: Dipeptidyl peptidase-4 inhibitors (DPP4is), drugs used to treat type 2 diabetes mellitus (DM2), show a significant association with bullous pemphigoid (BP) development. OBJECTIVES: To evaluate the clinical course and development of BP among patients with DM2 treated with DPP4is. METHODS: This retrospective cohort study included all the patients with BP and comorbid DM2 who visited Sheba Medical Center during 2015-2020. RESULTS: Among 338 patients with BP, 153 were included in our study. In 92 patients, BP diagnosis was attributed to the use of DPP4is. The patients with DPP4i-associated BP had fewer neurological and cardiovascular comorbidities and higher blistered body surface area (BSA) at first presentation, with noticeable upper and lower limb involvement. These patients were younger and more responsive to treatment, with a greater reduction in their BSA score after 2 months of treatment. CONCLUSIONS: The clinical features of patients with BP treated with DPP4is were initially more severe; however, during follow-up, a marked clinical improvement was noticed, especially among patients who had ceased the drug. Therefore, although withdrawal of the drug may not impose disease remission, it can alleviate the disease course and avert the need for treatment escalation.

Changing spectrum of hair and scalp disorders over the last decade in a tertiary medical centre.

BACKGROUND: Hair- and scalp-related disorders (HSRDs) encompass a wide range of conditions that affect people of all ages. OBJECTIVE: To evaluate the workload and trends in HSRDs at an outpatient dermatological clinic in a tertiary medical centre over a 10-year period. METHODS: Medical records for HSRD visits to the outpatient dermatology clinic at the Sheba Medical Center, an Israeli tertiary care center, were reviewed between 1 January, 2010 and 31 December, 2020. RESULTS: There were 10,396 HSRD visits with a new-to-follow-up visit ratio of 1:1.9. The annual number of HSRD visits, as well as their proportion out of all dermatological outpatient visits, have increased from 295 (1.24%) in 2010 to 1726 (9.44%) in 2020. The patients' mean age was 35.3 years (women 39.5 years, men 28.8 years), age range 1-87 years. Over the decade, there was a growing predominance of women with an average female-to-male ratio of 2:1. The winter season accounted for 28.7% of annual visits, followed by the autumn (25.6%), summer (24.3%) and spring (21.4%). The most prevalent HSRDs included androgenetic alopecia (30.6%), alopecia areata (19.3%), telogen effluvium (15.4%), non-scarring folliculitis (15.4%), seborrheic dermatitis (14.9%), lichen planopilaris (7.1%) and folliculitis decalvans (6.6%). Androgenetic alopecia demonstrated the highest increase over the decade examined (from 17% to 32%). CONCLUSION: HSRDs account for a significant percentage of visits at a tertiary dermatology outpatient clinic. The number of HSRD visits has increased annually over the past decade. The recent advancement in diagnosis and treatment may account, at least in part, for the growing burden of HSRDs within dermatological ambulatory care.

The Role of Histological Examination of Nail Clipping in the Diagnosis of Onychomycosis.

BACKGROUND: Diagnosis of onychomycosis is based on potassium hydroxide (KOH), direct smear, culture, and polymerase chain reaction. Nail clippings are rarely used as a diagnostic tool. OBJECTIVES: To evaluate nail clippings for the diagnosis of onychomycosis and to compare it to KOH smears. METHODS: Nail clipping specimens of 39 patients were collected: 34 with onychomycosis proved by positive culture and 5 from normal nails. The specimens were submitted to histological processing and then stained with periodic acid-Schiff (PAS) and Grocott-Gomori's methenamine silver (GMS) stains. For each nail, KOH smear was also performed. Two pathologists who had no information on the KOH smear and the culture results evaluated the nail clipping histology for the presence of fungal element. Their assessment was compared to the KOH smear and culture results. RESULTS: Of the 34 specimens that had positive culture, 25 were dermatophytes, 5 were molds, and 4 were candida. Clipping specimens were positive in 30 cases (88%): 23/25 dermatophyte, 4/5 molds, and 3/4 candida. Pathologists were able to classify the pathogens into dermatophytes and non-dermatophytes based on the morphology. PAS stain results were the same as GMS in evaluation of the nail specimen. KOH smear was positive in 29 nails (85%): 20/25 dermatophytes, all 5 molds, and 4 candida. In all five nails where the culture was negative, both clipping and KOH smear did not show fungal elements. CONCLUSIONS: Nail clippings can serve as a rapid, inexpensive, and reliable method for evaluation of onychomycosis, comparable to KOH smear, with the advantage of pathogen group identification.

Diagnostic delay, comorbid hidradenitis suppurativa and the prognostic value of bacterial culture in folliculitis decalvans: A cohort study.

BACKGROUND: Folliculitis decalvans (FD) is a type of primary neutrophilic cicatricial alopecia often leading to irreversible hair loss. Data on its epidemiology, clinical features, outcomes, and prognostic factors are limited. OBJECTIVE: To evaluate a cohort of patients with FD and identify characteristics of severe disease and prognostic factors which impede remission. PATIENTS AND METHODS: This retrospective cohort study included 192 patients diagnosed with FD and followed for at least six months at a tertiary center between 2010 and 2020. RESULTS: There was a diagnostic delay averaging 22.2 (± 29.7) months. Comorbid follicular occlusion disorders were common. Bacterial cultures were positive in 45.6% of the cases, with Staphylococcus (S.) aureus being the most common pathogen. Severe disease was associated with comorbid hidradenitis suppurativa and a positive bacterial culture, particularly S. aureus. 50.7% of patients experienced complete remission: 32% within the first six months of treatment and 18.7% later during follow-up. Relapses were frequent. Negative prognostic factors for achieving remission included younger age and a positive bacterial culture. CONCLUSIONS: There is a need for the education of dermatologists to reduce the diagnostic delay. Screening FD patients for comorbid hidradenitis suppurativa and obtaining bacterial cultures is important for treatment planning.

Illness Perception Regarding Early-stage Mycosis Fungoides among Dermatologists: A Multi-centre Cross-sectional Study.

Early-stage mycosis fungoides is a rare cutaneous T-cell lymphoma with a good prognosis. Data regarding patients' illness perception of mycosis fungoides are accumulating. However, investigating the dermatologists' viewpoint is also essential, as it shapes the therapeutic relationship and doctor-patient communication. The aim of this study was to investigate the aspects of dermatologists' illness perception towards early-stage mycosis fungoides and the way they present it to patients. Twenty-five dermatology residents and 55 attending physicians from all Israeli dermatology departments and the community completed the study questionnaires online. Dermatologists viewed mycosis fungoides as a chronic disease, causing a moderate emotional burden. In contrast to previously published data regarding patients' illness perception, dermatologists demonstrated dominancy in the notion that patients were able to control their disease. Most dermatologists thought that patients perceived mycosis fungoides as an indolent lymphoma that causes anxiety. Dermatologists used a high diversity of themes when presenting mycosis fungoides to patients. The differences between the residents' and attending physicians' perceptions were minimal. Dermatologists have a kaleidoscope of views regarding the way they perceive mycosis fungoides, the way they think patients perceive it, and the way they communicate with patients. Maintaining patient-centred communication enables dermatologists to identify these gaps and view mycosis fungoides from their patients' perspective.

Efficacy of Systemic Treatment for Leishmania tropica Cutaneous Leishmaniasis.

The effectiveness of systemic treatment for Leishmania tropica cutaneous leishmaniasis remains unclear. The purpose of the study is to evaluate the efficacy and safety of systemic treatments for L. tropica cutaneous leishmaniasis. This retrospective study was performed in 114 patients. Systemic treatments included liposomal amphotericin B and sodium stibogluconate. All patients underwent systemic treatment for L. tropica cutaneous leishmaniasis. Favourable treatment responses were recorded in 72.5% and 70.2% of the patients in the liposomal amphotericin B and sodium stibogluconate groups, respectively; 25.3% and 46% of those in the liposomal amphotericin B and sodium stibogluconate groups respectively, experienced at least one adverse effect. Lesions in cartilaginous areas were associated with higher treatment failure. Prior topical or systemic treatment increased the chance of future systemic treatment success. Liposomal amphotericin B was associated with a shorter intravenous treatment duration and better safety profile. Thus, liposomal amphotericin B is the treatment of choice for L. tropica cutaneous leishmaniasis.

Relationship Between Pemphigus Vulgaris Severity and PCR-positive Herpes Simplex Virus.

Pemphigus vulgaris is a rare autoimmune skin disease. Although herpes simplex virus has been associated with autoimmune diseases, evidence regarding its association with pemphigus vulgaris exacerbations is scarce. This retrospective cohort study aimed to characterize the epidemiological and clinical features of patients with pemphigus vulgaris who were herpes simplex-positive, compared with those who were herpes simplex-negative, during disease onset. Of 62 patients with pemphigus vulgaris who underwent PCR testing for herpes simplex virus, 25 (40.3%) were positive, with a mean age of 56.1 ± 15.5 years; 35.5% were male. The herpes-positive group had significantly elevated levels of C-reactive protein, Pemphigus Disease Activity Index score, and shorter time to relapse. The time to remission, number of exacerbations per year, and remission status were non-significantly elevated in the herpes-positive group. Thus, routine testing lesions from patients with pemphigus for herpes simplex virus should be performed. If positive, antiviral treatment should be initiated; and preventive antiviral treatment should be considered in severe cases.

Clinical Characteristics, Management, and Natural History of Chronic Inducible Urticaria in a Pediatric Cohort.

BACKGROUND: Some forms of chronic urticaria (CU) can be specifically attributed to a response to a definite trigger, referred to as chronic inducible urticaria (CIndU). We aimed to assess the demographics, clinical characteristics, comorbidities, natural history, and management of pediatric patients with CIndU. METHODS: Over a 6-year period, children presenting to the allergy clinic at the Montreal Children's Hospital (MCH) with CIndU were prospectively recruited. CU was defined as the presence of wheals and/or angioedema, occurring for at least 6 weeks. A standardized diagnostic test was used to establish the presence of a specific form of urticaria. Resolution was defined as the absence of hives for 1 year without treatment. RESULTS: Sixty-four patients presented with CIndU, of which 51.6% were male, with a median age of 12.5 (interquartile range 7.3, 15.9) years. Cold CU and cholinergic CU were the most common subtypes (60.3 and 41.3%, respectively). Basophil counts were undetectable in 48.4% of the cases, and C-reactive protein levels were elevated in 7.8% of patients. Of all cases, 71.4% were controlled with second-generation antihistamines. The resolution rate was of 45.3% (95% confidence interval 33.1-57.5%), based on per-protocol population within the 6-year course of the study. Resolution was more likely in patients who presented with well-controlled urticaria control test scores and elevated CD63 counts and in those suffering from thyroid comorbidity. CONCLUSION: The natural history of CIndU resolution in pediatric patients was relatively low and was associated with elevated CD63 levels, as well as thyroid comorbidity.

Hematological Inflammatory Markers in Patients with Pemphigus Vulgaris.

BACKGROUND: Emerging evidence indicates that several hematological markers can be used to evaluate treatment response, prediction, and early relapse detection in different inflammatory conditions. This study aimed to investigate the correlation between the neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, platelet-to-neutrophil ratio, mean platelet volume, and disease activity in patients with pemphigus vulgaris. METHODS: Fifty-six patients (20 men, 36 women; mean age 54 ± 14 years) diagnosed with pemphigus vulgaris were included in this retrospective study. Patients were divided into those treated and not treated with rituximab (groups 1 and 2), and into those who did and did not develop relapse (groups 3 and 4). The neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, platelet-to-neutrophil ratio and mean platelet volume were evaluated at the time of diagnosis, remission, and relapse. The relationship between each marker and disease stage was analyzed using the Wilcoxon rank-sum test for pairwise comparisons. RESULTS: The neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio showed a positive correlation with disease activity, while the platelet-to-neutrophil ratio and mean platelet volume showed a negative correlation. The neutrophil-to-lymphocyte ratio significantly decreased in remission (p < 0.001) and significantly increased in relapse (p < 0.01). The platelet-to-lymphocyte ratio significantly decreased in remission (p < 0.001) and showed no significant change in relapse. The platelet-to-neutrophil ratio significantly increased in remission (p < 0.001) and significantly decreased at relapse (p < 0.001). The mean platelet volume significantly increased in remission (p < 0.001) and decreased non-significantly at relapse. A more significant decrease in the neutrophil-to-lymphocyte ratio in remission was found in patients not treated with rituximab. No significant differences were observed between patients who developed relapse and those who did not. CONCLUSION: Our results suggest that the neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, platelet-to-neutrophil ratio, and mean platelet volume can be useful markers for monitoring treatment response, while the neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio can also assist in detecting early relapse.

Toe Web Infection: Epidemiology and Risk Factors in a Large Cohort Study.

INTRODUCTION: Toe web infection (TWI) is a bacterial infection of the interdigital space. In most cases, the infection is caused by gram-negative bacteria, secondary to a chronic fungal infection (dermatophytosis). The typical presentation includes macerations and erosions in the interdigital space. Predisposing factors include interdigital tinea, hyperhidrosis, and humidity. OBJECTIVE: The aim of this study was to characterize the TWI patient population and identify associated risk factors. METHODS: We conducted a retrospective study of patients diagnosed with TWI from 2006 to 2020 at Sheba Medical Center, Israel. Collected data included patients' demographics (age, sex, weight, and occupation), smoking pack-years, comorbidities, medications, and course of disease. RESULTS: A total of 200 patients were diagnosed with TWI. The median age at diagnosis was 51 years. The majority of the patients were men (72.5%). The most common comorbidities were dyslipidemia, hypertension, diabetes, and ischemic heart disease. We found that 71.2% of patients were smokers, and 46.4% of patients had occupations that required closed-toe shoes. TWI incidence did not increase seasonally. Bilateral TWI was found in 50% of the patients, 33% had recurrent infections, and 20% had secondary cellulitis. CONCLUSIONS: Smoking and diabetes were more prevalent among TWI patients than in the general population, and there was a correlation between smoking and TWI recurrences. We identified risk factors for TWI to identify at-risk populations.

Hair Shaft Morphology, Elemental Composition, and Nanoparticles in Frontal Fibrosing Alopecia: A Case-control Study.

Frontal fibrosing alopecia is a cicatricial alopecia with rising incidence. Titanium nanoparticles were suggested as a potential environmental trigger, yet this is unproven. This study assessed hair morphology, chemical composition and nanoparticles in 20 patients and 40 healthy controls using scanning electron micro-scopy and energy-dispersive X-ray spectroscopy. Morphological evaluation revealed a significantly higher degree of cuticle weathering in patients compared with controls when there were no differences in hair care routine. There were no differences in the background elemental composition, while particle analysis revealed a significant increase in particles containing titanium, chlorine, silicon, magnesium, and iron in the patient group. Titanium-containing nanoparticles showed the most significant increase, being 8.6 times greater than in controls, without relation to age and disease duration. The results indicate that patients with frontal fibrosing alopecia should be advised to avoid aggressive topical cosmetic and medical hair treatments, and refrain from using cosmetic preparations containing titanium nanoparticles.

Illness Perception, Perceived Social Support and Quality of Life in Patients with Pemphigus Vulgaris: What Should Dermatologists Know?

Data regarding the impact of psychological factors in patients with pemphigus is sparse. This study evaluated the correlation of demographic, clinical, and psychological parameters with quality of life in 58 patients with pemphigus. Illness perception showed a realistic view, with the highest scores for cyclical course (3.35 ± 0.09) and treatment control (3.08 ± 0.06). Mean scores for perceived social support were relatively high from family and significant others (5.89 ± 0.18 and 5.66 ± 0.23, accordingly), and relatively low from friends (4.55 ± 0.24). There were no correlations be-tween demographic, clinical parameters, perceived social support, and Dermatology Life Quality Index. Beliefs in cyclical course, emotional influence, psychological cause, and treatment control correlated significantly with quality of life. Stronger beliefs in self-control, chronicity, and accidental cause predicted higher quality of life, while stronger beliefs in cyclical course, psychological cause, and risk factors predicted lower quality of life. In order to enhance QoL, dermatologists should deliver the message to the patients that pemphigus is a chronic disease rather than cyclical and unexpected, and stress the patients' role in controlling it.

The effect of narrow-band ultraviolet B radiation on sleep, happiness, and appetite: A prospective cohort study.

BACKGROUND: The health benefits of sunlight are well documented, including the effects on happiness and sleep. However, only a few studies have investigated the benefits of artificial narrow-band ultraviolet B (NB-UVB) radiation. OBJECTIVES: To investigate the effect of NB-UVB on sleep quality, happiness, and appetite. METHODS: Patients from a single phototherapy unit were selected, and their epidemiological characteristics were documented. Subjects were asked to complete questionnaires including the Pittsburg Sleep Quality Index (PSQI), Subjective Happiness Scale (SHS), and the Simplified Nutritional Appetite Questionnaire (SNAQ) 4 weeks before and after the initiation of the NB-UVB therapy. The sample consisted of 52 patients. RESULTS: Four weeks after the initiation of NB-UVB sessions, sleep quality was significantly improved (the PSQI decreased from 6.5 to 5.23 (t = -3.52, P < .01). In contrast, subjective happiness did not improve (the SHS decreased from baseline 5.5 to 5.2, P > .05). Similarly, appetite did not change (the SNAQ score increased from baseline 14.75 to 15.05, P > .05) and body mass index was not affected by phototherapy. In general, men were found to have better sleep quality before and after UV light therapy. CONCLUSION: Our data suggest that NB-UVB exposure over 4 weeks improves sleep quality; however, it does not influence happiness or appetite.

Evaluation of the polymerase chain reaction-based T-cell receptor ß clonality test in the diagnosis of early mycosis fungoides.

BACKGROUND: T-cell receptor (TCR) clonality may help establish a diagnosis of mycosis fungoides (MF). Routine clonality analysis is performed by using a polymerase chain reaction TCR- gamma assay, yet with this method, 10% to 50% of T-cell lymphomas escape detection. TCR- beta gene rearrangement is an additional assay. Data about its efficacy are controversial. OBJECTIVE: To evaluate the role of TCR-ß assay in the diagnosis of early MF. METHODS: A retrospective study of 61 skin biopsies, 20 from patients with MF, 30 from patients suspected to have early MF, and 11 from patients with chronic inflammatory skin disease. RESULTS: Monoclonality was detected in 16 of 20 (80%) MF cases: 15 (75%) with TCR-ß and 12 (60%) with TCR-γ assay. Of the 30 suspected cases of early MF, 14 showed monoclonality with TCR-ß, and only 5 of 14 showed monoclonality with TCR-γ assay. None of the chronic inflammatory condition samples showed monoclonality. Therefore, TCR-ß clonality assay was more sensitive than TCR-γ in early MF (83% vs 43%; P = .002). LIMITATIONS: This was a retrospective, relatively small study. CONCLUSION: TCR-ß showed a higher sensitivity rate compared with TCR-γ in early-stage MF. The combined use of the TCR-ß and TCR-γ clonality tests can significantly improve the diagnosis rate of early-stage MF.

Diagnosis and management of pemphigus: Recommendations of an international panel of experts.

BACKGROUND: Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management. OBJECTIVE: We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations. METHODS: A preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology conference. Following the meeting, a second survey was sent to more experts to achieve greater international consensus. RESULTS: The 39 experts participated in the first round of the Delphi survey, and 54 experts from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II on the basis of Delphi results and meeting discussion. LIMITATIONS: Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available. CONCLUSIONS: We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first-line therapy option for moderate-to-severe pemphigus.

Efficacy of Repeated Courses of Rituximab as Treatment for Pemphigus Vulgaris.

Rituximab targets the B-lymphocyte antigen CD20, providing pemphigus vulgaris patients with long-term remissions. However, the effects of repeated courses have not yet been established. This study aimed to evaluate the effect of repeated rituximab courses on remission length in pemphigus vulgaris. A total of 73 patients with pemphigus vulgaris treated with rituximab at a single centre were retrospectively analysed. Of 73 study participants (28 men, 45 women), 42 (58%) received a 2nd course of rituximab, 24 (33%) received a 3rd course, 4 (6%) received a 4th course, and one (1%) received a 5th course. Rituximab remained efficacious in each course, irrespective of previous treatments (complete remission 75-81%). Following the 2nd and 3rd courses, the results indicated longer remissions with reduced flare-ups, and the remission length increased with each subsequent course. We conclude that rituximab serves as a disease-modifying agent, notably for patients with moderate-to-severe pemphigus vulgaris.