Hyperacute Graft-Versus-Host Disease of the Skin following Allogeneic Stem Cell Transplantation: A Paediatric Case Series.

Hyperacute GVHD (HaGVHD) is a rare complication of hematopoietic stem cell transplantation (HSCT) occurring before engraftment, a syndrome commonly involving skin and/or gut and/or liver, with increased morbidity and mortality. Myeloablative conditioning (MAC) regimes and mismatched donor transplants have an increased risk for HaGVHD. There is a higher chance of steroid-refractoriness and chronic GVHD in those who develop HaGVHD. There is limited literature about HaGVHD, especially in the paediatric age group. This retrospective single-centre case series included five paediatric patients who underwent HSCT between 1st April 2013 and 31st July 2015 at a tertiary care centre in South India, who fulfilled the criteria for HaGVHD as per criteria by Kim et al. and whose follow up data was available. We noted their risk factors, clinical course and prognosis. There were five paediatric HaGVHD patients. The risk factors noted among them were MAC regimen in three and mismatched unrelated donor sources in three. Two had steroid-refractory disease, four went on to develop chronic GVHD and three died of GVHD or treatment-related complications. A high index of suspicion is necessary to recognize HaGVHD, especially in patients with known risk factors developing a fever with rash post-HSCT.

Changing epidemiology of human enteroviruses (HEV) in a hand, foot and mouth disease outbreak in Vellore, south India.

PURPOSE: Hand Foot and mouth disease (HFMD) is a major childhood exanthematous disease causing outbreaks that have become a major public health threat in recent years. In Vellore district of Tamil Nadu, south India, occasional outbreaks are common among the paediatric age group, most commonly in those under 5years of age (U5s). CoxsackieA6, A4, A5, A9, A10, B2 and B5 are the common serotypes causing outbreaks. This study aimed to identify the molecular serotype of the causative agent, co-circulating in this region. METHODS: Adapting the WHO case definition, cases during an HFMD outbreak between October and December 2017, were identified by a clinical criterion of fever, mouth ulcers and rash in the extremities. Vesicle fluid from these lesions were collected in viral transport medium and transported cold to the Clinical Virology laboratory of a tertiary care hospital in Vellore. Identification of the causative agent was undertaken by two real time PCRs (EV1 and EV2) followed by sequencing the VP1-2C region and constructing a phylogenetic tree. RESULTS: Among the 31 HFMD patients included in this study, 23 (74.2%) were U5s, 3 (9.7%) were between 6 and 15 years and the remaining 5 (16.1%) were adolescents (>15 â€‹yrs). The outbreak ran a mild clinical course, with 22(71%) patients having fever as a prodromal symptom. Papulovesicular lesions characteristic of HFMD were present on all 31 (100%) patients' palms and soles, buttocks of 19 (61.3%), oral mucosa of 12 (38.7%), and all over the body in 4 (12.9%) patients. Coxsackie A6(75%) and Coxsackie A16(25%) were the pathogens associated with this outbreak. CONCLUSIONS: Changing epidemiology of HFMD was seen in this outbreak since; other serotypes apart from the classical Coxsackievirus serotypes causing HFMD outbreak were also found co-circulating. EV1 PCR was a better screening assay than EV2 PCR in this region. Continued surveillance and molecular serotyping are necessary for HFMD outbreaks in any region.

Erroneous reduction of HbA1c levels in patients with type 2 diabetes mellitus on dapsone treatment for Hansen's disease - a single-center retrospective cohort study.

Background Dapsone treatment may reduce HbA1c levels in patients with diabetes. Aims To assess the prevalence and characteristics of dapsone associated reduction of HbA1c in patients with Hansen's disease. Methods A retrospective data review of outpatient and inpatient charts of consecutive patients with Hansen's disease and type 2 diabetes mellitus was conducted over two years from January 2014 to January 2016 at the Department of Dermatology, CMC Vellore, India. Results Of the 245 patients with a confirmed diagnosis of Hansen's disease who were on oral dapsone 100 mg/day as part of their treatment regimen, 49 patients had diabetes and were eligible for the study as per predetermined inclusion criteria. Of these, 35 subjects (71%) had an HbA1c discordantly lower than the corresponding mean plasma glucose levels. Patients with discordant HbA1c levels were more likely to be male and to have a higher RBC mean corpuscular volume (MCV). A greater reduction in HbA1c levels was seen during the initial 3 months of therapy of dapsone treatment. Limitations The small sample size and retrospective design were limitations of this study. Also, we did not analyze the role of methemoglobinemia or the utility of alternative measures of glycemic control in these patients. Conclusion We describe a high prevalence of dapsone associated inappropriate HbA1c lowering in type 2 diabetes mellitus patients. This may have serious implications for the management of diabetes in patients on therapy with dapsone.

Upward trends of syphilis in the non-pregnant adults: A six-year report on clinical and epidemiological profile of syphilis from a tertiary care center, India.

Since 2000, a resurgence of syphilis has been noted in many developed and developing countries, especially among men who have sex with men (MSM). Incidence and prevalence of syphilis in pregnant women have been reduced drastically by mandatory screening in early pregnancy. Insufficient data in other populations especially from developing countries limit targeted public health interventions. This study aimed to describe the clinical and epidemiological profile of serologically confirmed syphilis cases among the non-pregnant high-risk group reporting to a tertiary care center in Southern India. A retrospective study was carried out in a tertiary care center in Southern India for 6 years from 2015 to 2020. A total of 265 serologically confirmed syphilis patients were included. A statistically significant increase in positivity from 0.52 to 2.1% was observed in this study (2015 to 2020). Among risk factors, high-risk behavior with multiple heterosexual partners was the commonest (51.3%), followed by marital partners who tested positive (9.4%) and MSM (7.5%). The majority of the patients were diagnosed at the latent stage (79%), followed by secondary syphilis (10%) and tertiary syphilis (8%). A quarter of patients (23%) were coinfected with HIV. Serological non-responsiveness was more common among HIV infected (47 vs. 24%). Sixteen had neurosyphilis and six had ocular involvement. HIV co-infection complicated 50% (8/16) of neurosyphilis patients. Syphilis is still prevalent, especially in high-risk groups including those are attending STI clinics. Further prospective multicentric studies are needed to identify and implement public health measures.

Cutaneous Sarcoidosis: A Retrospective Clinico-Pathological Study from the Indian Subcontinent in Patients Attending a Tertiary Health Care Centre.

CONTEXT: Sarcoidosis is a systemic disorder characterized histologically by noncaseating granulomas. There is paucity of Indian data on cutaneous sarcoidosis. AIMS: To describe the clinical, histopathological findings, and extracutaneous involvement in cutaneous sarcoidosis. MATERIALS AND METHODS: A retrospective study was done in patients of cutaneous sarcoidosis who had attended the dermatology clinic of a tertiary health care center in India from May 2009 to April 2015. The clinical details, histopathological findings, treatment, and response were reviewed. RESULTS: There were 38 patients with cutaneous sarcoidosis. Mean age was 48 ± 13 years; 58% were female. Median duration of disease was 11 months (IQR 4-48 months). More than one morphology was seen in 28.9%, commonest being plaques (65.7%), and papules (50%). Erythema nodosum was rare. More than one site was involved in 55.3%, most commonly trunk (52.6%). Six patients had isolated cutaneous sarcoidosis. Commonest extracutaneous organs involved were lung (73.7%) and lymph nodes (68.4%). Histopathologically, classical naked sarcoidal granulomas were found in only 55.3%. Angiotensin converting enzyme (ACE) levels were elevated in 74.3% (26/35) with significant association with extracutaneous disease. Treatment included topical and/or systemic corticosteroids, hydroxychloroquine, and tacrolimus. STATISTICS: Pearson's Chi-square test was done to analyze associations between the skin lesions, ACE levels, and systemic involvement; P < 0.05 was considered significant. CONCLUSIONS: Cutaneous manifestations of sarcoidosis are varied, commonest being erythematous plaques. Even though most patients had systemic involvement, we found no significant association of the type and number of skin lesions with extracutaneous involvement or prognosis. Elevated ACE levels were significantly associated with systemic involvement.

Cross-Sectional Study of Dermatoscopic Findings in Relation to Activity in Vitiligo: BPLeFoSK Criteria for Stability.

BACKGROUND: Stability in vitiligo is an important concept in guiding patient management and a vital prerequisite before vitiligo surgery. Disease activity of vitiligo based on patient's history is imprecise. It is practically impossible to perform biopsy from all lesions of vitiligo to ascertain stability. Dermatoscopy can be used to examine all clinical lesions in a patient of vitiligo. There is a need to validate many reported dermatoscopic findings for universal use. AIMS: To analyze the significance of dermatoscopic findings in the activity of vitiligo and to devise a cutoff score for stable vitiligo. MATERIALS AND METHODS: Dermatoscopic examination was performed in 85 patients clinically diagnosed with vitiligo. Six dermatoscopic parameters, namely, border, pigment network, perilesional hyperpigmentation, perifollicular pigmentation, satellite lesions, and micro-Koebner phenomenon (acronym: BPLeFoSK) were evaluated against Wood's lamp findings as standard. Chi-square test was used to test association between categorical variables. Cutoff values for stability for these six parameters were plotted in receiver operating curve. RESULTS: A total of 131 vitiligo lesions were analyzed with dermatoscopy. Absence of satellite lesions and absence of micro-Koebner phenomenon were the most sensitive parameters (96.7% and 100%, respectively). Sharp border and absent or reticulate pigment network within the vitiligo patch were the most specific findings (100% and 91.5%, respectively). CONCLUSION: A cutoff score of more than or equal to 1.5 using the "BPLeFoSK criteria" indicates stability in the vitiligo lesion.

Frontal rhytidectomy as surgical treatment for pachydermoperiostosis: a case report.

The thickened folded skin of Touraine-Solente-Golé syndrome can result in cosmetic and functional deformities. The treatment of pachydermoperiostosis is usually centered around improving the cosmetic appearance through plastic surgery. We describe the case of a 27-year-old male who had pachydermoperiostosis with a leonine facies that was managed with frontal rhytidectomy. A greatly improved cosmetic appearance was achieved with this procedure.

Acquired Cutis Laxa Associated with Light and Heavy Chain Deposition Disease.

Acquired cutis laxa (ACL) is a rare connective tissue disorder characterized by pendulous and coarsely wrinkled skin. There have been few cases of its association to monoclonal immunoglobulin deposition disease (MIDD), which constitutes the light chain (LCDD), heavy chain (HCDD), and light and heavy chain (LHCDD) deposition disease. MIDD predominantly involves the kidney. Skin is the next common organ to be affected by HCDD, which presents as ACL. We report the case of a 40-year-old male who presented with ACL associated with LHCDD. The clinical features of ACL in the present case appeared prior to the development of clinical features related to LHCDD.

Erroneous reduction of HbA1c levels in patients with type 2 diabetes mellitus on dapsone treatment for Hansen’s disease - a single-center retrospective cohort study

Background: Dapsone treatment may reduce HbA1c levels in patients with diabetes. Aims: To assess the prevalence and characteristics of dapsone associated reduction of HbA1c in patients with Hansen’s disease. Methods: A retrospective data review of outpatient and inpatient charts of consecutive patients with Hansen’s disease and type 2 diabetes mellitus was conducted over two years from January 2014 to January 2016 at the Department of Dermatology, CMC Vellore, India. Results: Of the 245 patients with a confirmed diagnosis of Hansen’s disease who were on oral dapsone 100 mg/day as part of their treatment regimen, 49 patients had diabetes and were eligible for the study as per predetermined inclusion criteria. Of these, 35 subjects (71%) had an HbA1c discordantly lower than the corresponding mean plasma glucose levels. Patients with discordant HbA1c levels were more likely to be male and to have a higher RBC mean corpuscular volume (MCV). A greater reduction in HbA1c levels was seen during the initial 3 months of therapy of dapsone treatment. Limitations: The small sample size and retrospective design were limitations of this study. Also, we did not analyze the role of methemoglobinemia or the utility of alternative measures of glycemic control in these patients. Conclusion: We describe a high prevalence of dapsone associated inappropriate HbA1c lowering in type 2 diabetes mellitus patients. This may have serious implications for the management of diabetes in patients on therapy with dapsone.

Late-onset acute graft-versus-host disease mimicking hand, foot, and mouth disease.

Acute skin graft-versus-host disease (GVHD) classically presents as a pruritic erythematous maculopapular rash. We describe a patient who underwent allogeneic hematopoietic stem cell transplantation and presented with a hand foot and mouth disease like clinical presentation. Histopathology was suggestive of acute GVHD. This case is being reported to make dermatologists aware of this unusual presentation of GVHD.

Ross Syndrome: A Case Report and Review of Cases from India.

Ross syndrome is a rare dysautonomia characterized by a clinical complex of segmental anhidrosis or hypohidrosis, areflexia, and tonic pupils. A very few cases (≃50) have been reported in literature since its original description in 1958. Here, we report the case of a middle-aged homemaker from Odisha, India, who presented with complaints of segmental hypohidrosis for the past 7 years.

Yellow nail syndrome in rheumatoid arthritis: an aetiology beyond thiol drugs.

Yellow nail syndrome (YNS) is a rare entity characterized by a triad of nail changes, lymphoedema and lung involvement. We report a 57-year-old man with rheumatoid arthritis (RA) and YNS. We have reviewed the previous case reports of RA and YNS and discuss the pulmonary manifestations.

An analysis of the dermatological uses of mycophenolate mofetil in a tertiary hospital.

BACKGROUND: Mycophenolate mofetil (MMF) is increasingly being used by dermatologists to treat various skin conditions, but limited evidence exists regarding its efficacy. OBJECTIVE: To evaluate safety and efficacy of MMF in the treatment of dermatological conditions. METHODS: A retrospective chart review of all the dermatology patients treated with MMF between October 1999 and July 2008 at a university-based teaching hospital in Australia. RESULTS: Sixty-nine patients included 43 females (62%) and 26 males. Nineteen patients (27%) achieved complete remission (CR) and 14 (20%) had no response to therapy. The average duration of treatment was 18.4 months and the mean daily dose was 2 g. Thirty-two patients (47%) experienced side effects, though most were mild. Factors influencing response to MMF were studied, and older age (p=0.005), diagnosis (p=0.008) and duration of treatment (p=0.02) were found significant. In a multivariate analysis, only the diagnosis remained statistically significant. There was a differential response to MMF between the various dermatological disorders studied. Complete response was achieved in 56%, 53% and 46% of the patients with atopic dermatitis (AD), immunobullous disorders and neutrophilic dermatoses, respectively, while none of the patients with psoriasis achieved CR. CONCLUSION: Our study shows that MMF is more effective in AD and immunobullous disorders than in psoriasis and pyoderma gangrenosum.

Toxicity and clinical outcomes in patients with HIV on zidovudine and tenofovir based regimens: a retrospective cohort study.

BACKGROUND: Adverse drug reactions are a major concern with zidovudine/stavudine treatment regimens. The less toxic tenofovir regimen is an alternative, but is seldom considered due to the higher costs. This study compared adverse drug reactions and other clinical outcomes resulting from the use of these two treatment regimens in India. METHODS: Baseline, clinical characteristics and follow-up outcomes were collected by chart reviews of HIV-positive adults and compared using univariate/multivariate analysis, with and without propensity score adjustments. RESULTS: Data were collected from 129 and 92 patients on zidovudine (with lamivudine and nevirapine) and tenofovir (with emtricitabine and efavirenz) regimens, respectively. Compared to patients receiving the zidovudine regimen, patients receiving the tenofovir regimen had fewer adverse drug reactions (47%, 61/129 vs 11%, 10/92; p<0.01), requiring fewer regimen changes (36%, 47/129 vs 3%, 3/92; p0.01). With the propensity score, the zidovudine regimen had 8 times more adverse drug reactions (p<0.01). Opportunistic infections were similar between regimens without propensity score, while the zidovudine regimen had 1.2 times (p=0.63) more opportunistic infections with propensity score. Patients on the tenofovir regimen gained more weight. Increase in CD4 levels and treatment adherence (>95%) was similar across regimens. CONCLUSIONS: Patients on a tenofovir regimen have better clinical outcomes and improved general health than patients on the zidovudine regimen.

Sorafenib Induced Hand Foot Skin Rash in FLT3 ITD Mutated Acute Myeloid Leukemia-A Case Report and Review of Literature.

Sorafenib is a novel small molecule multiple kinase inhibitor which has been used for metastatic renal cancer, hepatocellular cancer. Sorafenib induced skin rash has been discussed as a side effect in trials in both, FLT3 wild type and mutated acute myeloid leukemia (AML), as monotherapy or as combination with other chemotherapeutic agents. We describe a patient with FLT 3 ITD mutated AML, who was started on adjunctive Sorafenib therapy. Skin reactions manifested as NCI Grade III palmoplantar erythrodysesthesia (PPE), requiring drug discontinuation. Several pathogenic mechanisms have been implicated in Sorafenib induced skin reactions, but none has been conclusively proven. While treatment options are varied for early stage skin reactions, drug discontinuation remains the only possible therapy presently for severe grade skin reaction.

Novel multidrug therapy for disseminated rhinosporidiosis, refractory to dapsone ­ case report.

Rhinosporidiosis is a chronic granulomatous disorder, caused by Rhinosporidium seeberi endemic in India and Sri Lanka. The most common sites are the nasal mucosa and the nasopharynx and cutaneous lesions usually occur as a part of disseminated rhinosporidiosis. Dapsone has been frequently used in treating disseminated disease in immunocompetent individuals. Here we report a case of disseminated rhinosporidiosis in an immunocompromised individual on antiretroviral drugs, non-responsive to Dapsone and therefore treated with a multidrug therapy of Cycloserine, Dapsone and Ketoconazole with good response.

Focal dermal hypoplasia in a male.

Focal dermal hypoplasia (FDH) is a rare mesoectodermal dysplasia syndrome characterized by cutaneous, skeletal, dental, ocular and soft-tissue defects. An X-linked dominant mode of inheritance with lethality in male subjects has been proposed. Only around 30 cases of FDH have been reported in male subjects. Live born affected males are mosaic for mutations in PORCN gene . We present the mosaic pattern of FDH in a young boy.