Changes in the Cutaneous Nerve Fiber Staining and Distribution of PGP9.5 in Clinically Uninvolved Skin in Leprosy Patients after Completion of Multidrug Therapy and Assessing PGP9.5 as a Marker of Treatment Response.

Background: Subclinical involvement of nerves may sometimes be present much before the overt clinical manifestations become apparent. Protein gene product (PGP) 9.5, a ubiquitin-C-terminal hydrolase, has been widely used as a marker to study the involvement of peripheral nerve fibers in many diseases. Aim and Objectives: To evaluate the change in cutaneous nerve fiber staining and distribution from pre-treatment and post completion of multidrug therapy through the expression of PGP9.5 and to assess PGP9.5 as a marker of treatment response. Materials and Methods: In this prospective single-center observational study, skin biopsy was taken in patients with leprosy, having areas of nerve function impairment (NFI), based on findings of nerve conduction studies (NCSs), but not having lesions or impaired tactile or thermal impairment clinically. The thin nerve fiber density in the clinically normal skin in areas supplied by nerve showing changes of sensory neuropathy was evaluated to study the density of the fibers. A second biopsy was taken at the end of treatment from a site near the previous site to assess the changes in intra-epidermal nerve fiber staining and distribution. Results: Thirty-three patients were recruited in the present study (24 males and 9 females). Pre-treatment, 27 patients had abnormal NCSs, while six patients did not have any evidence of neuropathy on NCSs. Staining for nerve fibers using PGP9.5; in the epidermis was positive in five patients pre-treatment and 11 patients post treatment (P = 0.181). Staining in the dermis revealed positivity in 14 pre-treatment, which increased to 18 post treatment (P = 0.342). Adnexae showed positivity in five patients pre-treatment and increased to 17 post treatment (P = 0.005). Conclusion: A reduced PGP9.5 staining in the epidermal, dermal, and adnexal regions was seen in leprosy patients, which improved post treatment. Thus, PGP9.5 may serve as a marker of NFI and treatment response.

Tofacitinib in Pediatric Alopecia Areata Totalis and Alopecia Universalis: A Retrospective Analysis From India.

Alopecia areata totalis and universalis are disabling conditions and therapeutically challenging as they are refractory to conventional options. Tofacitinib is a Janus-kinase (JAK) inhibitor utilized to treat alopecia areata (AA) as an off-label drug. In India, FDA-approved JAK inhibitors such as baricitinib and ritlecitinib are not available. There are only a few case reports on tofacitinib in AA in the Indian population. We present the data of 9 pediatric cases of clinically and histologically proven alopecia areata totalis (AT) and alopecia universalis (AU), for whom oral tofacitinib was given after baseline investigations. The following parameters were analysed: Photographic image and severity of alopecia tool (SALT) score at baseline, 3 months and 6 months, and Children Dermatology Life Quality Index (cDLQI) at baseline and 6 months. The mean ± standard deviation (M ± SD) of the SALT score and cDLQI(M ± SD) at baseline were 95 ± 5 and 17 ± 2. At weeks 4 and weeks 12, the SALT (M ± SD) score was 92.7 ± 6.1 and 34.35 ± 11.16, respectively. At weeks 24, the SALT (M ± SD) score and cDLQI (M ± SD) were 3.33 ± 5 and 6 ± 2. The final reduction in SALT score from the baseline was 100% in 6/9 cases (66.67%), 75% to 99% in 3/9 (22.23%), and 50 to 75% in 1/9 (11.12%). We also observed minimal adverse effects (one child developed herpes zoster) with tofacitinib. Our study demonstrates that oral tofacitinib represents a viable modality in managing difficult-to-treat pediatric AA, such as AT and AU, with a good safety profile.

Linear indurated plaque over the neck.

Isolated cutaneous swelling can have varied etiologies. The clinical diagnosis is usually difficult, and a correct diagnosis always requires a pathological examination. Hereby, we report a case of linear keloidal morphea on the neck of an 18-year-old male who presented with an asymptomatic, firm lesion for 6 months. Histopathological examination was consistent with morphea. This case highlights the uncommon form of morphea in an unusual location, which can be misdiagnosed for numerous neoplastic conditions and for which simple histopathological evaluation can clinch the diagnosis.

Trichoscopic Features of Scalp Discoid Lupus Erythematosus versus Lichen Planopilaris: A Systematic Review.

Introduction: Lichen planopilaris (LPP) and discoid lupus erythematosus (DLE) are primary scarring alopecias that pose diagnostic challenges clinically, where trichoscopy features may provide benefit in delineating these two cicatricial alopecia, and also helps in assessing the evolution and therapeutic response. To date, there are few reviews on dermoscopic findings in differentiating these two alopecias. Methods: A systematic literature review was conducted using the PubMed and Google Scholar databases. The search terms included for scalp DLE were 'lupus' OR 'discoid lupus' OR "scalp lupus" and for scalp LPP were "lichen planopilaris" OR "scalp follicular lichen planus" OR "lichen planus follicularis" and were combined with "dermoscopy" OR "dermatoscopy" OR "videodermoscopy" OR "video dermatoscopy" OR "trichoscopy". The differences in the prevalence of dermoscopic features in scalp DLE and LPP were calculated using the Chi-square test. Results: Of 52 articles, 36 (17 LPP, 19 DLE) were eligible for quantitative analysis. We found predominant peripilar tubular casts and perifollicular erythema with the presence of arborizing vessels in the vicinity of these changes, indicating early LPP. In contrast, follicular red dots, speckled brown pigmentation, and hair diameter variability indicated active DLE. Shiny white areas were common in both the groups in late stages. The target pattern of distribution of blue-grey dots, milky red areas, and irregular white fibrotic dots were seen in LPP, and pink-white background, follicular plugs, perifollicular and interfollicular scale, rosettes, chrysalides, and red spider on yellow dots were detected in DLE. Features such as yellow dots and blue-grey structureless areas were nonspecific and did not have a major role in differentiating DLE from LPP. Conclusion: This article provides a comprehensive review of the literature and delineates the trichoscopic differences and peculiarities of scalp DLE and LPP, including the correlation of dermoscopic features with histopathological findings.

Teleophthalmology at a primary and tertiary eye care network from India: environmental and economic impact.

OBJECTIVE: To evaluate the environmental and economic impact of teleophthalmological services provided by a primary (rural) and tertiary (urban) eyecare network in India. METHODS: This prospective study utilised a random sampling method, and administered an environmental and economic impact assessment questionnaire. The study included 324 (primary: 173; tertiary: 151) patients who received teleconsultations from July to September 2022. The primary network (rural) used a colour-coded triage system (Green: eye conditions managed by teleconsult alone; yellow: semi-urgent referral within 1 week to a month, red: urgent referral within a day to a week). The tertiary network (urban) included new and follow-up patients. The environmental impact was assessed by estimating the potential CO2 emissions saved by avoiding travel for various transport modes. Economic impact measured by the potential cost savings from direct (travel) and indirect (food and wages lost) expenses spent by yellow and red referrals (primary) and the first-visit expenses of follow-up (tertiary) patients. RESULTS: The primary rural network saved 2.89 kg CO2/person and 80 km/person. The tertiary urban network saved 176.6 kg CO2/person and 1666 km/person. The potential cost savings on travel expenses were INR 19,970 (USD 250) for the primary (average: INR 370 (USD 4.6) per patient) and INR 758,870 (USD 9486) for the tertiary network (average: INR 8339 (USD 104) per patient). Indirect cost savings (food and wages) were of INR 29,100 (USD 364) for the primary and INR 347,800 (USD 4347) for the tertiary network. CONCLUSION: Teleophthalmology offers substantial environmental and economic benefits in rural and urban eyecare systems.

Xeroderma pigmentosum group G with pellagroid rash: A rare presentation.

Xeroderma pigmentosum (XP), a heterogeneous genodermatoses, has a variable clinical spectrum ranging from mild freckling and photosensitivity to severe skeletal and neurological abnormalities and cutaneous malignancies. Herein, we present the case of a 4-year-old boy with XP group G who presented with a pellagroid rash.

Oral Acitretin Plus Topical Triamcinolone vs Topical Triamcinolone Monotherapy in Patients With Symptomatic Oral Lichen Planus: A Randomized Clinical Trial.

Importance: Symptomatic oral lichen planus (OLP) can be challenging to treat. Objective: To compare the efficacy of oral acitretin plus topical triamcinolone acetonide (TAC), 0.1%, with TAC monotherapy in patients with symptomatic OLP. Design, Setting, and Participants: This monocentric, investigator-initiated, placebo-controlled, investigator- and patient-blinded randomized clinical trial was conducted from December 2018 to June 2020 at the Postgraduate Institute of Medical Education and Research, a tertiary referral center in Chandigarh, India. Sixty-four patients 18 years or older with symptomatic OLP were recruited by consecutive sampling. Data were analyzed from July to December 2020. Intervention: The patients were randomized to receive either a combination of oral acitretin (25-35 mg/d) and TAC (treatment group) or TAC in combination with placebo (placebo group) for 28 weeks, with an additional 8 weeks of treatment-free follow-up after the end of treatment (36 weeks of total study duration). Main Outcomes and Measures: The disease severity and treatment response were assessed using Oral Disease Severity Score (ODSS), Oral Health Impact Profile 14 (OHIP-14), and visual analog scale (VAS). The primary aim was to assess the number of patients achieving ODSS-75 (75% reduction in ODSS compared with baseline) in both groups at 28 weeks and at the end of 36 weeks. Results: Among 64 patients, 31 in the treatment group and 30 in the placebo group completed the study (mean [SD] age, 50.6 [15.2] years vs 49.2 [14.4] years; male-female ratio, 13:19 vs 16:16). Baseline ODSS, visual analog scale, and Oral Health Impact Profile 14 scores were comparable in both groups. In the intention-to-treat analysis, there was a statistically significant higher number of patients achieving 75% or higher reduction in ODSS in the treatment group compared with the placebo group at the end of 28 weeks (28 [88%] vs 15 [47%], a 41 [95% CI, 20-61] percentage point difference between groups; P < .001; Cramér V = 0.47) and 36 weeks (27 [84%] vs 13 [41%], a 43 [95% CI, 23-67] percentage point difference between groups; P < .001; Cramér V = 0.47). Relapses during the posttreatment follow-up of 8 weeks were low among patients in both treatment and placebo groups (1 [3%] vs 2 [6%], a 3 [95% CI, -13 to 7] percentage point difference between groups; P > .99; Cramér V = 0.07). Conclusion and Relevance: In this randomized clinical trial, the combination of oral acitretin and TAC was more effective than TAC monotherapy in patients with symptomatic OLP. Trial Registration: Clinical Trial Registry of India Identifier: CTRI/2018/11/016448.

Contact hypersensitivity to Indian standard patch test series correlates with disease severity among children with atopic dermatitis.

Background There is emerging evidence of a relationship between atopic dermatitis (AD) and allergic contact dermatitis (ACD), though the data available are scarce with conflicting viewpoints. We explored the occurrence of contact hypersensitivity among children with atopic dermatitis by patch testing them with the Indian standard series and tried to correlate the presence of contact hypersensitivity with the clinical severity of AD in these children. Methods In this single-centre, cross-sectional study, children between 6 months and 12 years diagnosed with atopic dermatitis were included and patch tested with the Indian standard series. Outcome parameters were the proportion of patients having positive patch-test reactions, the proportion of positive patch-test reactions for each allergen and factors associated with patch test positivity in atopic dermatitis. Results Of the 136 patients, 80 were boys. The mean age of the study population was 5.6 ± 3.2 years. Twenty-eight (20.6%) patients had patch test positivity at 96 h. Fragrance mix was the commonest allergen, followed by potassium dichromate, cobalt chloride hexahydrate and nickel. SCORing atopic dermatitis (SCORAD) was significantly higher in patients with positive patch tests as compared to patients with negative patch tests (P = 0.009). Conclusion Greater disease severity in atopic dermatitis was found to be associated with patch test positivity. Limitations Inability to establish relevance in about 50% of the patients was a limitation of our study. Follow-up data regarding the impact of allergen avoidance is not available.

A randomized prospective study to assess the role of topical tacrolimus as preventive therapy in unstable acral vitiligo.

BACKGROUND: Acral vitiligo is a significantly distressing condition and tends to be treatment-resistant. The occurrence of new lesions on acral areas further causes greater psychological trauma. Topical tacrolimus has been widely used in the management of vitiligo and its role in preventing flares in other dermatoses such as atopic dermatitis has been well documented. OBJECTIVES: To assess the role of topical tacrolimus as preventive therapy in unstable acral vitiligo. MATERIALS AND METHODS: In this single-centre randomized prospective study, 60 patients aged 16-60 years having unstable acral vitiligo with symmetrical lesions were enrolled and randomized (1:1) into two groups. Patients in group A were instructed to apply topical tacrolimus 0.1% ointment on both vitiliginous and normal skin while patients in group B were instructed to apply topical tacrolimus 0.1% ointment only on vitiliginous skin for 6 months. Only the distal hand till the wrist joint was chosen for observation. Vitiliginous patches were assessed monthly for 6 months for a change in the number of lesions and total area involved, extension of preexisting lesions and adverse effects if any. RESULTS: A reduction in the number of lesions was observed in both groups. The decrease in the number of lesions in group A was 5.6% as compared to 2.3% in group B (p-0.001). The decrease in depigmented area in group A was 10.5% as compared to 4.6% in group B (p-0.048). Treatment failure was seen in 11 out of 60 (18.3%) patients. CONCLUSION: Tacrolimus 0.1% ointment application showed effectiveness in preventing the appearance of new lesions in unstable acral vitiligo and hastening the repigmentation when applied on both lesional and perilesional skin in vitiligo.

Deep Brain Stimulation for the Management of AIFM1-Related Disabling Tremor: A Case Series.

The AIFM1 gene encodes a mitochondrial protein that acts as a flavin adenine dinucleotide-dependent nicotinamide adenine dinucleotide oxidase and apoptosis regulator. Monoallelic pathogenic AIFM1 variants result in a spectrum of X-linked neurological disorders, including Cowchock syndrome. Common features in Cowchock syndrome include a slowly progressive movement disorder, cerebellar ataxia, progressive sensorineural hearing loss, and sensory neuropathy. We identified a novel maternally inherited hemizygous missense AIFM1 variant, c.1369C>T p.(His457Tyr), in two brothers with clinical features consistent with Cowchock syndrome using next-generation sequencing. Both individuals had a progressive complex movement disorder phenotype, including disabling tremor poorly responsive to medications. Deep brain stimulation (DBS) of the ventral intermediate thalamic nucleus ameliorated contralateral tremor and improved their quality of life; this suggests the beneficial role for DBS in treatment-resistant tremor within AIFM1-related disorders.

Outpatient Deep Brain Stimulation Surgery Is a Safe Alternative to Inpatient Admission.

BACKGROUND: Deep brain stimulation (DBS) is usually performed as an inpatient procedure. The COVID-19 pandemic effected a practice change at our institution with outpatient DBS performed because of limited inpatient and surgical resources. Although this alleviated use of hospital resources, the comparative safety of outpatient DBS surgery is unclear. OBJECTIVE: To compare the safety and incidence of early postoperative complications in patients undergoing DBS procedures in the outpatient vs inpatient setting. METHODS: We retrospectively reviewed all outpatient and inpatient DBS procedures performed by a single surgeon between January 2018 and November 2022. The main outcome measures used for comparison between the 2 groups were total complications, length of stay, rate of postoperative infection, postoperative hemorrhage rate, 30-day emergency department (ED) visits and readmissions, and IV antihypertensive requirement. RESULTS: A total of 44 outpatient DBS surgeries were compared with 70 inpatient DBS surgeries. The outpatient DBS cohort had a shorter mean postoperative stay (4.19 vs 39.59 hours, P = .0015), lower total complication rate (2.3% vs 12.8%, P = .1457), and lower wound infection rate (0% vs 2.9%, P = .52) compared with the inpatient cohort, but the difference in complications was not statistically significant. In the 30-day follow-up period, ED visits were similar between the cohorts (6.8% vs 7.1%, P = .735), but no outpatient DBS patient required readmission, whereas all inpatient DBS patients visiting the ED were readmitted ( P = .155). CONCLUSION: Our study demonstrates that DBS can be safely performed on an outpatient basis with same-day hospital discharge and close continuous monitoring.