A Case of Palmoplantar Keratoderma in the Constellation of Connective Tissue Diseases.

Overlap syndrome is a clinical challenge and brings together a wide range of treatment options for the treating physician. Addressing each and every complaint of the patient is crucial. A 50-year-old female patient presented with skin thickening, blackening, and hyperkeratosis; dysphagia; joint pain; features of myopathy; Raynaud's phenomenon; and dry mouth. Inflammatory markers were raised along with a positive antinuclear antibody (ANA) with Golgi apparatus pattern, anti-Sjögren's-syndrome-related antigen A (anti-SSA)/Ro60 3+, anti-SSA/Ro52 3+, and anti-PM/Scl 2+ antibodies that suggested overlap syndrome. Although the patient had no respiratory complaints, a unique interstitial lung disease (ILD) pattern was noted during the evaluation. Skin manifestations were puzzling, but the histopathology analyses of skin biopsies taken from two different sites revealed distinguishing features of cutaneous lupus and dermatomyositis. Treatment with hydroxychloroquine, pilocarpine, nifedipine, methotrexate, and topical tacrolimus produced a dramatic improvement in the clinical features. This case highlights subtle and florid features of different autoimmune diseases. The hyperkeratotic skin changes were the most striking feature, but the whole evaluation process unveiled many rare presentations of known autoimmune conditions that can open doors to new areas of our understanding toward connective tissue diseases (CTDs). Our case report demonstrates significant heterogeneity in the ANA patterns, ILD patterns, clinical manifestations, and treatment approaches.

Unusual clinical presentations in leprosy: a case series and review.

Conventionally, leprosy has been divided into various spectra of presentation ranging from the tuberculoid to the lepromatous pole, as well as histoid, pure neuritic leprosy and reactional states. This however is an oversimplification as leprosy can present in unusual clinical forms that may obfuscate the diagnosis. Our objective was to highlight unusual clinical presentations of leprosy occurring across all spectra of the disease. Our case series describes eight uncommon presentations of leprosy seen over a period of 10 y from 2011 to 2021, wherein clinical diagnosis followed by a histopathological confirmation of leprosy was performed. These include rare presentations such as psoriasiform plaques, Lazarine leprosy, verrucous plaques and hypertrophic scarring. Many of these rare presentations remain hitherto unreported, such as primary hypogonadism and annular plaques mimicking erythema annulare centrifugum and erythema gyratum repens. Sarcoidosis and syphilis have been labeled as great mimickers in dermatology. The current case series and review is an attempt to highlight a multitude of unusual presentations of leprosy that need a separate mention to make a correct and timely diagnosis and prevent the debilitating sequelae of this otherwise treatable infectious disease.

The Puzzle of Papules Over Face and Extrafacial Areas: A Rare Case of Disseminated Idiopathic T-Cell Pseudolymphoma.

Papular lesions over face have a wide range of differential diagnoses. The clinical differentiation among these is difficult and often requires clinicopathological correlation. We hereby report the case of a 28 year old female presenting with numerous skin colored papular lesions over face, pinna and upper limbs.

Mycetoma: A Common Yet Unrecognized Health Burden in Central India.

CONTEXT: Mycetoma is a chronic suppurative infective disorder of skin, subcutaneous tissue, fascia, and bones caused by the traumatic inoculation of either fungal (eumycotic) or bacterial (actinomycotic) organisms present in the soil. Triad of tumefaction, discharging sinuses, and grains characterizes the disease. AIMS: This study was undertaken to study the clinical spectrum and treatment response of mycetoma in central India. SETTINGS AND DESIGN: It was a retrospective study of clinical and/or biopsy-proven and treated cases of mycetoma from November 2015 to October 2016. SUBJECTS AND METHODS: Medical records of diagnosed and treated mycetoma patients were enrolled retrospectively during November 2015 and October 2016. Case records of patients were evaluated with respect to demographic, clinical, microbiological details, bone involvement, and treatment. Type of therapies and outcome, wherever available, were also assessed. STATISTICAL ANALYSIS: Statistical analysis was done using proportion, mean, and percentages. RESULTS: Eleven cases (male = 8) were seen during the study period (seven actinomycetoma and four eumycetoma). Foot and lower extremity was the most common site (9/11), whereas upper extremity and forehead were involved in one case each. On culture, the organisms isolated were Phialophora and Fusarium. Modified Welsch regimen was started in six of seven patients with actinomycetoma, whereas one was started on sulfamethoxazole-trimethoprim and a combination of amoxicillin and clavulanic acid therapy. All four cases of eumycetoma were treated with itraconazole. On follow-up, six cases of actinomycetoma cases showed significant improvement. Two cases of eumycetoma showed mild to moderate improvement and one case required surgical intervention. One case each of actinomycetoma and eumycetoma were lost to follow-up. CONCLUSION: Mycetoma has been recognized as a neglected tropical disease by the World Health Organization, recently. There are very few case reports from the central part of India. Prominent case detection in our study emphasizes the need of larger studies to know the extent of disease in this part of India.

A retrospective study of the severe and uncommon variants of erythema nodosum leprosum at a tertiary health center in central India.

BACKGROUND: Erythema nodosum leprosum (ENL) classically presents with tender, coppery, evanescent nodules along with constitutional features and visceral involvement. However, uncommon morphological variants of ENL-like erythema nodosum necroticans, erythema multiforme (EM)-like ENL, Sweet's syndrome (SS)-like ENL, Lucio phenomenon, and reactive perforating type of ENL have also been described in the literature. The primary objective of this study was to describe the clinical features of the severe and uncommon morphological variants of ENL. METHODS: This was an observational case series with retrospective review of records of all ENL patients with ulceronecrotic lesions admitted in the Department of Dermato-venereo-leprology of a tertiary health center of central India over a period of 2 years. RESULTS: Eighteen patients were included, all of whom had ulceronecrotic lesions. Four out of them had EM like ENL, two had SS-like presentation, and one of them had annular bullous lesions over old infiltrated plaques of leprosy. CONCLUSIONS: Uncommon variants of ENL can be very commonly misdiagnosed in patients, especially in those who have not been previously diagnosed with leprosy. Hence, a high index of suspicion is required in such cases to avoid delay in the diagnosis and resulting morbidity.

Hair manifestations of endocrine diseases: A brief review.

Hair disorders are common in clinical practice and depending upon social and ethnic norms, it can cause significant psychosocial distress. Hair growth, cycling and density are regulated by many endogenous factors, mainly circulating hormones. Thus, diseases affecting the endocrine system can cause varied changes in physiological hair growth and cycling. Diagnosis and treatment of these disorders require a multidisciplinary approach involving a dermatologist, gynecologist and an endocrinologist. In this review, we briefly discuss the influence of hormones on the hair cycle and hair changes in various endocrine disorders.

Zosteriform Lichen Planus Pigmentosus Altering Segmental Vitiligo.

A 22-year-old student presented with a 3-year history of hyperpigmented, minimally pruritic skin lesions over his trunk. They had begun as small discrete macules over the left side of the trunk, with some coalescing to form patches. He had had right-sided segmental vitiligo (SV) for the previous 7 to 8 years (Figure 1A), and this had started to cross the midline, covering a margin over the left side of the chest and back. This coincided with the onset and spread of the hyperpigmented patches. He denied any history of drug intake, or history of inflammatory dermatoses at either the vitiliginous or the hyperpigmented sites.

A prospective study to validate various clinical criteria used in classification of leprosy: a study from a tertiary care center in India.

BACKGROUND: Various clinical criteria are used to categorize leprosy patients into paucibacillary (PB) and multibacillary (MB), thus aiding in appropriate treatment. However, comprehensive studies validating these criteria are minimal. AIMS: To assess sensitivity and specificity of different clinical criteria individually and in combination for classifying leprosy into PB/MB spectrum. METHOD: A prospective study was conducted wherein 50 newly diagnosed, untreated leprosy cases were recruited and classified into PB and MB using the following clinical criteria: number of skin lesions (NSL), number of body areas affected (NBAA), and size of largest skin lesion (SLSL). Patients with pure neuritic leprosy, diffuse macular type of lepromatous leprosy, and with reactions were excluded. Sensitivity and specificity of these clinical criteria in classification was calculated taking histopathological findings as gold standard. RESULTS: Among 50 patients, 37 were males and 13 were females with a mean age of 32.08 ± 16.55 years. The sensitivity and specificity of NSL, NBAA, and SLSL was 94.74 and 87.1%, 94.74 and 61.29%, and 73.68 and 16.13%, respectively. Combining all three criteria, the sensitivity increased to 100%, but specificity decreased drastically to 12.9%. The ROC curve for NSL, NBAA, and SLSL showed a cutoff of ≥6 skin lesions, ≥3 body areas affected, and ≤2 cm lesion to classify as MB. CONCLUSION: The current WHO system of leprosy classification based on NSL seems to be best among available clinical criteria. Uniform and sensible application of this criteria itself assures appropriate categorizing and leprosy treatment with reasonable sensitivity and specificity.

Sofosbuvir induced steven Johnson Syndrome in a patient with hepatitis C virus-related cirrhosis.

Sofosbuvir is an imperative drug used in treatment regimens for hepatitis C virus (HCV). It is considered relatively safe with fewer adverse effects than other treatments. Here, we report a rare and potentially serious, dermatologic, adverse effect following the use of sofosbuvir. A 35-year-old man with genotype 3-related HCV cirrhosis presented with decompensated ascites and jaundice following 7 weeks of therapy with peginterferon alpha-2a and oral ribavirin. After peginterferon withdrawal and stabilization, oral sofosbuvir and ribavirin were started; 10 days later, he developed itching over the trunk and legs, followed by multiple papules and vesicles over an erythematous base. Over the next 15 days, the rash progressed with the formation of blisters and peeling skin. Simultaneously, the oral mucosa and lips developed crusting and painful erosions. Considering drug-induced Steven John Syndrome (SJS), sofosbuvir and ribavirin were withdrawn and the patient was treated with topical emollients, steroids, and supportive care. The lesions improved over the next 4 weeks, with some residual hyperpigmentation. Rechallenge with sofosbuvir alone at one eighth the dose resulted in similar skin and mucosal lesions after 2 months; these lesions also improved after sofosbuvir withdrawal. The Algorithm of Drug Causality for Epidermal Necrolysis score was 7, which suggested sofosbuvir as the very probable drug resulting in SJS in our patient. Conclusion: The appearance of SJS following sofosbuvir use is an important and potentially fatal complication from a drug that serves as the backbone of several HCV treatment regimens. Treating physicians must use sofosbuvir with caution and consider withholding or discontinuing this drug in patients with such severe dermatologic manifestations. (Hepatology Communications 2018;2:16-20).