Genodermatoses Picture: Difficulties Faced And Way Forward in Nepal.

Genodermatoses are group of genetic disorders that present with cutaneous manifestations. The exact prevalence on many of these conditions are unknown due to its rarity, need of specialized tests for diagnosis and lack of proper reporting system. Most of the patients are faced with life-long disability and associated stigma. There is a need for specialized centers for proper diagnosis of these conditions and a very elaborated yet simple reporting system in Nepal. These rare conditions should be kept in priority by the government in align with the sustainable development goals to ensure healthy-lives and promote well-being for all. A wider engagement of patient-led support groups might be useful in providing necessary information on the disease to the general population and alleviate the stigma associated with these diseases. Keywords: Epidermolysis bullosa; genodermatoses; rare diseases; Nepal.

Leishmania donovani persistence and circulation causing cutaneous leishmaniasis in unusual-foci of Nepal.

Cutaneous leishmaniasis cases have increased dramatically in recent years in Nepal. The study offers molecular identification of the Leishmania species using 40 patient's aspiration biopsy samples, targeting markers kinetoplast minicircle DNA (kDNA) and internal transcribed spacer-1 (ITS1). Among molecularly diagnosed 22 cutaneous leishmaniasis cases, L. donovani complex was identified in 13 instances and L. major in 9 cases. The ITS1 PCR was positive in 12 of the positive nested- kDNA PCR cases (12/22), confirming L. donovani complex in seven of the cases and L. major in five of the cases. In addition, the study conclude that concurrent occurrence of atypical cutaneous infections caused by L. donovani parasite in 59.1% of cases and typical cutaneous infections caused by L. major parasite in 40.9% of cases. A Phylogentic analaysis showed that the detected L. donovani species present null genetic distances from seven references of L. donovani, but slight differences between ITS1 sequences and not grouped into a significant monophyletic cluster.

Cutaneous Tuberculosis among Patients Presenting to Dermatology Outpatient Department of a Tertiary Care Centre: A Descriptive Cross-sectional Study.

Introduction: Cutaneous tuberculosis is an uncommon form of extrapulmonary tuberculosis. It can present in various morphological presentations leading to a late diagnosis in many cases. It is mainly associated with significant scarring and morbidity. It is classified as paucibacillary or multibacillary depending on the bacillary load. Similarly, it can be acquired through either an endogenous or an exogenous source. The mainstay of treatment is anti-tubercular medications. The objective of the study was to find out the prevalence of cutaneous tuberculosis among patients presenting to the dermatology outpatient department of a tertiary care centre. Methods: A descriptive cross-sectional study was done among the patient presenting to the outpatient department of dermatology and venerology in a tertiary care centre where all patients data from medical records were taken from April 2016 to March 2021 after taking ethical approval from the Institutional Review Committee (Reference number: 503/2078/79). Demographic details of the patients including age, sex, site and duration of the lesion were recorded. Convenience sampling was done. Point estimate and 95% Confidence Interval were calculated. Results: Among 1,30,924 cases, 40 (0.03%) (0.02-0.04, at 95% Confidence Interval) cutaneous tuberculosis was seen. Conclusions: The prevalence of cutaneous tuberculosis was similar to the studies done in similar settings. Keywords: cutaneous; extrapulmonary tuberculosis; tuberculid.

Translation, Cultural Adaptation and Validation of Cardiff Acne Disability Index in Nepali Language.

BACKGROUND: Acne causes significant impairment in the quality of life of patients, but clinicians in Nepal lack a simple validated tool to measure the psychological side of acne. We planned to translate and validate Cardiff Acne Disability Index, a five item questionnaire into Nepali language to address this need. METHODS: A linguistic translation with semantic equivalence to the original English language Cardiff Acne Disability Index was achieved through standard forward and backward translation into Nepali language. All eligible patients were requested to fill the Nepali translated version of both Cardiff Acne Disability Index and Dermatology Life Quality Index questionnaires. Reliability and validity of the newly translated questionnaire were established, based on statistical analyses of factor structure, item correlations and concurrent correlations. RESULTS: This validity study included 94 patients with male: female ratio of 1:3.27 and mean age 21.29 (±3.92) years. Reliability analysis revealed a Cronbach's alpha of .72 and mean inter item correlation coefficient of .337. A single factor was extracted on Principal Component Analysis explaining 48.40% of variance. A strong correlation of Cardiff Acne Disability Index scores to the Dermatology Life quality Index score (rs>.7) indicated good concurrent validity. CONCLUSIONS: The newly translated Nepali Cardiff Acne Disability Index is a valid tool to measure the impact of acne in Nepalese patients. This short and simple assessment tool will help clinicians understand the patient's perspective of her acne.

Prevalence of post kala-azar dermal leishmaniasis (PKDL) and treatment seeking behavior of PKDL patients in Nepal.

BACKGROUND: In Nepal, the burden of post kala-azar dermal leishmaniasis (PKDL) is not known since there is no active case detection of PKDL by the national programme. PKDL patients could pose a challenge to sustain visceral leishmaniasis (VL) elimination. The objective of this study was to determine the prevalence of PKDL and assess PKDL patients' knowledge on VL and PKDL, and stigma associated with PKDL. METHODOLOGY/PRINCIPAL FINDINGS: Household surveys were conducted in 98 VL endemic villages of five districts that reported the highest number of VL cases within 2018-2021. A total of 6,821 households with 40373 individuals were screened for PKDL. Cases with skin lesions were referred to hospitals and examined by dermatologists. Suspected PKDL cases were tested with rK39 and smear microscopy from skin lesions. An integrated diagnostic approach was implemented in two hospitals with a focus on management of leprosy cases where cases with non-leprosy skin lesions were tested for PKDL with rK39. Confirmed PKDL patients were interviewed to assess knowledge and stigma associated with PKDL, using explanatory model interview catalogue (EMIC) with maximum score of 36. Among 147 cases with skin lesions in the survey, 9 (6.12%) were confirmed as PKDL by dermatologists at the hospital. The prevalence of PKDL was 2.23 per 10,000 population. Among these 9 PKDL cases, 5 had a past history of VL and 4 did not. PKDL cases without a past history of VL were detected among the "new foci", Surkhet but none in Palpa. None of the cases negative for leprosy were positive for PKDL. There was very limited knowledge of PKDL and VL among PKDL cases. PKDL patients suffered to some degree from social and psychological stigma (mean ± s.d. score = 17.89 ± 12.84). CONCLUSIONS/SIGNIFICANCE: Strengthening the programme in PKDL case detection and management would probably contribute to sustenance of VL elimination. Awareness raising activities to promote knowledge and reduce social stigma should be conducted in VL endemic areas.

Use of mobile tele-dermatology in managing cutaneous leishmaniasis from a remote district of Nepal during the COVID 19 pandemic: A case series.

With the invent of smart phones, tele-dermatology has become a cheap and cost-effective medium to provide medical services to rural population. Its usefulness is more prominently noted during the COVID 19 pandemic and much more in countries with difficult terrain with limited specialist health providers. Cutaneous leishmaniasis (CL) is a neglected tropical disease which may cause significant disability if treatment is delayed. Here, we discuss three cases of CL from a remote district by use of tele-dermatology during the pandemic lockdown.

Skin Diseases in a Pediatric Hospital of Nepal.

The skin diseases of pediatric population are varied which change according to age and season. There is a rarity of studies on pediatric skin conditions from Nepal. This observational study from the only tertiary care referral pediatric center of the country highlighted the burden of pediatric skin diseases in Nepalese population. All new cases of pediatric patients less than 14 years of age consulting the pediatric dermatological OPD of Kanti Children's Hospital from January 2017 to December 2017 were included in this study. Demographic details of all the patients such as age and sex were recorded. The diagnosis was made clinically in most instances and appropriate laboratory and histopathological examination were performed wherever necessary. A total of 7683 pediatric patients were included in the study. Among these, there were 4574 (59.53%) males and 3109 (40.47%) females. The most common skin condition was infections among 2463 (32.12%) followed by eczematous conditions in 1711(22.27%) and hypersensitivity reactions in 1510 (19.65%). Infections were more common during the summer months. Overall, both infectious and noninfectious skin diseases were significantly more common during the warmer (summer and spring) months as compared to colder (autumn and winter) months (p < 0.001). This study shows that the pediatric dermatoses are common in Nepalese population.

Improper Use of Topical Corticosteroids in Tinea Infections in a Tertiary Care Hospital.

BACKGROUND: Steroid-modified tinea, also known as tinea incognito, is an infection by the dermatophytes, where the clinical morphology is modified due to corticosteroids, either systemic or topical. Rampant use of topical corticosteroids has led to increasing recurrence in tinea infections. METHODS: All consenting cases of tinea presenting to outpatient department of dermatology department of Civil Service Hospital from March to August 2020 for a total of 6 months were included in this study. Tinea infection involving only the palms, soles, nails or scalp were excluded. RESULTS: A total of 200 patients were included in this study. Among these, 175 patients (87.5%) were using topical corticosteroids. A significant association was noted between dermatophyte infection of more than one month and topical corticosteroids use (p<0.05). This study revealed that males were using super-potent topical corticosteroids more as compared to females (p<0.05). Moreover, no association was noted between the level of education attained and the use of topical corticosteroids (p=0.91). Only 25 (12%) patients were either using correct or no topical medications during the time of consultation with the dermatologist. Among the patients using topical corticosteroids, 155 (88.6%) patients were using them on recommendation of the local pharmacist and only 2 (1.2%) patients were prescribed by a physician. CONCLUSIONS: In short, use of topical steroids was rampant among patients with tinea whilst 77.5% patients procured steroid topicals over-the-counter. Hence, a tougher law and strict regulatory guidelines deemed necessary to curb the unauthorized and rampant sale of these medicines.

Recurrent Herpes Zoster in an Immunocompetent Male: A Case Report.

Herpes zoster is an infection caused by reactivation of varicella-zoster virus presenting as multiple grouped vesicular eruptions in a dermatomal pattern with associated pain. Recurrent herpes zoster is an uncommon event in an immunocompetent host. Here, we report a case of a young male presenting with herpes zoster over the T9 and T10 dermatome with the previous scarring of herpes zoster over the T6 dermatome over the right upper trunk. The patient improved on treatment with oral acyclovir and analgesics. In any patient with recurrenrt hepes zoster, work-up should be done to rule out immunosuppresion.

Chronic Urticaria and Its Impact on the Quality of Life of Nepalese Patients.

Chronic urticaria (CU) is a skin condition characterized by sudden and recurrent episodes of wheals, angioedema, or both and commonly associated with itching for a duration of more than six weeks. The available data indicate that urticaria markedly affects both objective functioning and subjective well-being of patients. A review of patients' records with chronic urticaria attending Civil Service Hospital from January 2018 to December 2019 was done. A detailed demographic data of all patients with chronic urticaria was also retrieved. Dermatology Life Quality Index questionnaire (DLQI) Nepalese version was used for the assessment of the impact of disease on life quality. Mann-Whitney U-test was applied to compare means, and principle component analysis for factor analysis was used. A total of 149 patients were included, with a male-to-female ratio of 1 : 1.9. The mean age of the study population was 32.86 ± 12.837 years. The mean DLQI score was 8.30 ± 6.73 with men having a significantly greater score than women (p < 0.02). DLQI scores negatively correlated with age (p < 0.01). There was a high internal consistency among items (Cronbach's alpha 0.89), and all items had satisfactory correlation with each other as well. Principle component extraction revealed that there were two underlying factors in the DLQI questionnaire on measuring quality of life in chronic urticaria. Males had a greater impairment in quality of life than females due to chronic urticaria. Most severe impairment was seen in symptoms/feelings subdomain. It also revealed that there were two different underlying factors in DLQI questionnaire.

Case Report: Erysipeloid Cutaneous Leishmaniasis Treated with Oral Miltefosine.

Cutaneous leishmaniasis (CL) can present with various skin lesions ranging from a single ulcer to diffuse multiple nodules. Here, we present a case of a 67-year-old man with a large erythematous well-defined indurated plaque over the left face for a duration of 4 months. A slit skin smear was performed, and it was stained with Giemsa stain which showed multiple amastigotes confirming the diagnosis of CL. Oral miltefosine was started at a dose of 150 mg/day but had to be stopped after 20 days as the patient developed diarrhea, bipedal edema, and renal impairment. This case emphasizes an uncommon variant of CL and the role of systemic treatment with oral miltefosine and its associated adverse effects.

Medical Conference on a Complete Digital Platform in Nepal: A Paperless Experience.

Medical conferences are held by different medical societies around the world on a regular basis. The society of Dermatologists, venereologists and leprologists of Nepal organized their 15th annual national conference in a complete digital platform making it the first paperless medical conference in Nepal. This conference initiated the use of digital platform for information dissemination and discouraged the use of paper. The mobile applications built for the conference provided information on the scientific programs and also created an unique identity of each registered delegate with an unique quick response code. The code was very valuable during registration and served as meal coupons too. This conference was our small effort towards creating an eco-friendly medical conference. Keywords: Conference; dermatology; digital; eco-friendly; medical; mobile apps; paperless.

Cutaneous Leishmaniasis of Lip and Role of Polymerase Chain Reaction: A Case Report.

The diagnosis of cutaneous leishmaniasis is mostly confirmed by the identification of parasite in a skin smear or biopsy. However, this method may not always be sensitive enough to detect the disease when parasitic load is low. Molecular test such as polymerase chain reactions can be useful in such circumstances. Here, we report a case of cutaneous leishmaniasis diagnosed by a polymerase chain reaction test when both smear and biopsy failed to confirm the diagnosis. A 17-years-old female from mountainous district of Nepal, presented with a crusted plaque over the upper lip for a duration of 6 months. Both skin smear and biopsy from the lesion failed to demonstrate Leishmania parasite but a polymerase chain reaction test was positive for Leishmania donovani. This case emphasizes on the importance of molecular testing such as polymerase chain reaction when commonly performed diagnostics test fails to support confirmation of clinical diagnosis.

Multibacillary Leprosy Presenting as Anetoderma in a Young Teenager.

Anetoderma presents as a circumscribed area of slack skin. It can present as either primary or secondary, if associated with other conditions. Leprosy is one of the causes of secondary anetoderma, but it is not commonly reported, especially in multibacillary leprosy. Here, we report a case of a 16-year-old young girl who presented with fever, joint pain, and only three anetodermic plaques. A slit skin smear from the lesion showed multiple acid-fast bacilli with a bacillary index of 3+, thus confirming the diagnosis of leprosy. This case is unique since multibacillary leprosy presented with only few anetoderma lesions in a young teenager girl from a leprosy-eliminated country.

Panniculitis as an Initial Presentation of Dermatomyositis: A Case Report.

Dermatomyositis is an idiopathic muscle disease characterized by proximal muscle weakness, raised muscle enzymes, characteristic changes in electromyography and typical skin rash and biopsy findings. Dermatological features like Gottron's sign and papules are considered as pathognomonic for dermatomyositis. Panniculitis is one of the rare findings in dermatomyositis. Here, we report a case of dermatomyositis in 37 years old female who presented with only panniculitis and the diagnosis was delayed by more than a year.

Porphyria Cutanea Tarda Presenting as Erythema-multiforme Like Lesions.

Porphyria cutaneatarda, is the most common type of porphyria.It is characterized by defective uroporphyrinogen III decarboxylase enzyme.It presents with erosion, bulla with milia formation and sometimes with hypertrichosis and abnormal pigmentation mostly on the photo-exposed sites. A urine fluorescence of coral red color helps in the diagnosis. Here, we present a rare case of porphyria cutanea tarda in a 15 years old male who presented with multiple targetoid plaques. Keywords: Erythema-multiforme; porphyria cutanea tarda; targetoid.