'Monkeypox 2.0': Case series on a reconditioned virus causing sexually transmissible disease in urban population.

BACKGROUND: MPOX (Monkeypox) viral infection, a zoonotic disease previously confined to the African sub-continent, has caught attention worldwide recently due to its resurgence in a new 'avatar' among urban communities. Dermatologists in the U. A. E. started to see patients with fever and a self-limiting pustular necrotic rash that was negative for all other infectious investigations. METHODS: We performed a prospective observational multicenter clinical study of the demographics, skin manifestations, and outcomes of patients presenting with necrotic pustular lesions and/or fever. RESULTS: 35 cases of PCR confirmed MPOX cases, mostly in the expatriate population, were followed up and found to have high-risk heterosexual contact on an average of 1 week prior to disease onset. We found that they have characteristic annular pustular lesions with necrotic center or "Smoke ring pustules' in all cases. Lesion tenderness and predilection for the lower abdomen, pubic area, and genitalia were observed. Most cases were systemically stable, with fever lasting for an average of 4 days and elevated CRP levels. Genital lesions were prone to secondary bacterial infections. The disease was severe, with larger annular plaques in one of our patients found to be living with HIV. CONCLUSIONS: The overall prognosis in healthy individuals is good, with lesions healing within an average of 2 weeks without scarring. 'New world MPOX' should be unclassified from zoonosis to a sexually transmitted infection (STI) capable of transmission in an urban population. Our findings can help in early clinical suspicion and differentiation from other STI's for primary and secondary health care physicians.

Major histocompatibility complex (MHC) gene frequency in acquired dermal macular hyperpigmentation: a case control study.

BACKGROUND: Human leukocyte antigen (HLA) allele frequencies have a known association with the pathogenesis of various autoimmune diseases. METHODS: We recruited 31 Indian patients of acquired dermal macular hyperpigmentation (ADMH) and 60 unrelated, age-and-gender-matched healthy controls. After history and clinical examination, 5 ml of blood in EDTA vials was collected. These samples were subjected to DNA extraction and the expression of HLA A, B, C, DR, DQ-A, and DQ-B was studied. RESULTS: There was a predominance of females with a gender ratio of 23 : 8 and the most common phototype was Fitzpatrick type IV (83.9%). There was a significant association of HLA A*03:01 (OR: 5.8, CI: 1.7-17.0, P = 0.005), HLA B*07:02 (OR: 5.3, CI: 1.9-14.6, P = 0.003), HLA C*07:02 (OR: 4.3, CI: 1.8-9.6, P = 0.001), HLA DRB1*10:01 (OR: 7.6, CI: 1.7-38.00, P = 0.022), and HLA DRB1*15:02 (OR: 31.0, CI: 4.4-341.8, P < 0.001) with patients compared to controls, whereas HLA DQB*03:01 was less associated with patients compared to controls (OR: 0.2, CI: 0.0-0.6, P = 0.009). CONCLUSION: Patients with ADMH are more likely to have the HLA A*03:01, HLA B 07*02, HLA C*07:02, HLA DRB1*10:01, HLA DRB1*15:02 and less likely to have the HLA DQB*03:01 allele. Larger cohort studies may thus be conducted studying these specific alleles.

Clinical and Genetic Characteristics of Ectodermal Dysplasia in Four Indian Children.

Introduction: Ectodermal dysplasias (EDs) affect structures derived from the ectoderm such as skin, its appendages, nail, and teeth. In this series, we describe four patients presenting with a clinical phenotype of dysplasia of one or more ectodermal structures who underwent next-generation sequencing for mutational analysis. Case Series: The clinical phenotype of three patients was hypohidrotic ectodermal dysplasia (HED) and one patient was diagnosed with autoimmune polyglandular syndrome (APS) type 1. Two patients with classical clinical features of X-linked HED (XLHED) had mutations in EDA gene; variant c.924+ 8C>G (5' proximal splice site) and c.760C>T (p.Gln254Ter). Case 3 had clinical phenotype of HED with urticaria pigmentosa, which was confirmed on skin biopsy and immunohistochemistry. This patient was found to have mutation in C1orf172; c.449G>A (p.Arg150Gln) which has not been reported previously. Case 4 was diagnosed to have APS type 1 with cutaneous features of discoloration of teeth and chronic mucocutaneous candidiasis. This patient had a compound heterozygous mutation of AIRE gene. The two variants detected were c.169C>T (p.Gln57Ter) and c.47C>T (p.Thr16Met). Conclusion: The present series highlights the clinic-genetic correlation in four patients with features of ED. Two variants of uncertain significance and two previously unreported variants were also found in this study.

Acquired Dermal Macular Hyperpigmentation: An Update.

Acquired dermal macular hyperpigmentation (ADMH) is an umbrella term that includes disorders clinically characterized by small and large pigmented macules/patches and histopathologically showing an evidence of current or resolved interface dermatitis with pigment incontinence, without clinically significant prior inflammatory phase. The term intends to include diseases previously described in the literature as lichen planus pigmentosus, Riehl's melanosis/pigmented cosmetic dermatitis and ashy dermatosis/erythema dyschromicum perstans. The nomenclature and origin of these disorders have always been a matter of discussion. These disorders share many clinicopathological similarities, are difficult to treat and adversely affect the quality of life. Recent consensus points towards the need for a unifying term to facilitate research and therapeutic trials. This article aims to provide a comprehensive review of the recent advances in ADMH.

Clinico-Epidemiological Characteristics of Hidradenitis Suppurativa: A Retrospective Cohort Study from a Tertiary Care Centre in Northern India.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic debilitating disease with a relapsing and remitting course. Due to delay in diagnosis, patients are often referred when the disease is very severe. Management strategies vary across multiple guidelines. AIMS: The aim of this study was to analyze the demographic and clinical characteristics of patients with HS among our outpatient attendees and to study the outcomes of various treatments offered. METHODOLOGY: This was a retrospective cohort study analyzing case files and photographic records of all patients diagnosed with HS, presenting to our tertiary care institute over 18 months. RESULTS: A total of 22 patients (10 males and 12 females) of HS were studied with majority having Hurley stage 2 and 3 diseases. The most common site affected was axilla. Overweight and obese patients were 45.4% and 18.1%, respectively. Rifampicin-clindamycin combination or doxycycline was the first line therapy offered. Adalimumab was given in only two patients but could not be continued for long term due to financial issues. Surgery was performed in six patients. Procedures included wide local excision and deroofing which is left to heal by secondary intention. Least number of remissions and most satisfactory improvement was seen with a combination of antibiotics and surgery compared to medical treatment alone. LIMITATIONS: Retrospective nature and a single center study were the major drawbacks. CONCLUSION: Patients undergoing procedural intervention in addition to pharmacotherapy have best overall outcomes and involvement of a multidisciplinary team plays a key role, however a larger follow-up study is required.

Clinical, biochemical, and serologic predictors of drug reaction with eosinophilia and systemic symptoms syndrome: A prospective case-control study.

BACKGROUND: Detailed scoring systems such as the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) score for validating a diagnosis of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome are available, but there is no rapid, easy tool to identify DRESS at presentation. OBJECTIVE: To identify the clinical, biochemical, and serologic markers predicting the DRESS syndrome and its severity. METHODS: In this prospective observational study, 25 patients with the DRESS syndrome and 25 control patients with maculopapular drug rash were recruited. Baseline clinical, biochemical, and serologic markers, such as high-sensitivity C-reactive protein (hsCRP), erythrocyte sedimentation rate, and thymus and activation-regulated chemokine (TARC) levels, were recorded and their utility in identifying the DRESS syndrome at presentation and predicting severity was analyzed. RESULTS: The effectiveness of TARC level (>613.25 pg/mL), total body surface area (TBSA, >35%), hsCRP (>5 mg/L), eosinophils (>6%), absolute eosinophil count (>450 cells/mm3), and aspartate transaminase (>92 U/L) were statistically similar to the effectiveness of the RegiSCAR DRESS validation score (≥2) in diagnosing the DRESS syndrome. A combination model (TBSA at baseline, eosinophil count, and hsCRP) at the cutoff of 6.8 had a sensitivity of 96% and a specificity of 100%. Baseline serum TARC levels did not predict the DRESS severity or outcome. LIMITATIONS: Small sample size. CONCLUSION: The combination of TBSA involvement, eosinophil count, and hsCRP levels can predict the DRESS syndrome at presentation.

Application of mucous membrane dermoscopy (mucoscopy) in diagnostics of benign oral lesions - literature review and preliminary observations from International Dermoscopy Society study.

Dermoscopy of mucosal surface termed "mucoscopy" is an upcoming offshoot of dermatological imaging. However, the literature on mucoscopy is limited to individual cases and small case series. An organized review or systematic analysis of mucoscopy is lacking. The aim of this review was to summarize the published literature on mucoscopic features of benign conditions affecting the oral mucosa and semi-mucosa. Additionally, the results of mucoscopic features of diseases, which have not been described before have been presented.

Persistent plaques after release from treatment in slit-skin smear negative leprosy patients: Wait and watch or adopt a proactive approach?

A subset of leprosy patients has clinical and histopathological activity in the form of persistent plaques and granulomas after completion of multidrug therapy (MDT) which can have significant impact on their quality of life. In the absence of clear guidelines regarding management of such patients, majority of the times they are treated either as late reversal reaction with corticosteroids or no active treatment is offered. We observed 11 patients of leprosy with persistent plaques after completing the 6/12-months MDT who were treated favorably with minocycline 100 mg once daily for 16 weeks. Complete clinical resolution was observed in 9/11 patients while two patients had partial improvement. Histopathological improvement in the form of disappearance of granulomas corroborated with the clinical improvement. All the patients tolerated the treatment well and hyperpigmentation was the only adverse effect noted. Minocycline may be considered as a useful and well tolerated therapeutic option for this subset of leprosy patients due to its immune modulatory and anti-inflammatory effects.

Case Report: Multidrug-Resistant Mycobacterium leprae in a Case of Smear-Negative Relapse.

The ongoing transmission of leprosy in India is worrisome, and emerging drug resistance may be one of the factors responsible for the continued transmission of leprosy in India. Emerging cases of multidrug-resistant Mycobacterium leprae pose a great threat to eradication of leprosy and must be addressed with utmost priority. We report a case of multidrug-resistant M. leprae in a case of relapse where slit skin smear (SSS) was negative and histopathology was inconclusive. Drug resistance studies in leprosy are undertaken only in smear-positive relapse cases, and detection of this type of multidrug resistance in a case with negative SSS and innocuous histopathology is rather unusual and highlights the importance of undertaking drug resistance tests even in smear-negative cases of leprosy relapse. Resistance to ofloxacin (OFL) is also a cause for concern as OFL is one of the reserve drugs recommended for treatment of rifampicin-resistant strains.

Dermatoscopy of nonvenereal genital dermatoses: A brief review.

The scope of dermatoscopy has now vastly expanded and shows promising use for characterization of both pigmentary and inflammatory dermatoses affecting the skin, nail, and mucosae. Due to concerns of contamination and spread of infection, dermatoscopy has not been widely studied for genital mucosal dermatoses. In this article, we review the dermatoscopic features of nonvenereal dermatosis affecting the genitalia. Although biopsy is required for a definitive diagnosis, dermatoscopy is useful to identify atypical and suspicious pigmentary lesions. For the inflammatory dermatoses and other benign dermatoses, presence of few characteristic findings can aid in the diagnosis.