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.

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.

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.

Clinical profile of hereditary angioedema from a tertiary care centre in India.

Introduction: Hereditary angioedema (HAE) is a clinical condition which could be fatal if not identified and managed appropriately. Knowledge of this condition is mostly confined to individual case reports and literature reviews in India. In this retrospective study we describe HAE cases which presented to a tertiary care centre in India over a period of three and half years.

Rhinofacial conidiobolomycosis: Clinical and microbiological characterisation and shift in the management of a rare disease.

Background: Conidiobolomycosis is a rare tropical rhinofacial fungal infection which has not been well characterised. The available evidence in its management is sparse due to lack of clinical studies and the limited data on antifungal susceptibility patterns.

Objective: To analyse the clinical manifestations, antifungal treatment and outcomes of patients with conidiobolomycosis and to determine antifungal susceptibility profiles of the isolates.

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.

PSENEN Mutation in Coexistent Hidradenitis Suppurativa and Dowling-Degos Disease.

Hidradenitis suppurativa is a chronic relapsing disease with multiple abscesses, nodules, and scars in the apocrine bearing areas. Dowling-Degos is a rare autosomal dominant genodermatosis characterized by multiple hyperpigmented macules or papules in reticulate pattern, affecting mainly the flexures. We report a case of coexisting hidradenitis suppurativa and Dowling-Degos disease in a 31-year-old male in whom mutation analysis revealed a splice site mutation c.

Disseminated cutaneous chrysosporium infection.

Cutaneous chrysosporium infection is extremely rare and underdiagnosed. We present an immunocompromised patient who presented with recurrent cutaneous abscesses. Histopathology of the abscess showed thick-walled conidia and septate fungal hyphae within the subcutis and fungal culture grew species.

Bathing Suit Variant of Autosomal Recessive Congenital Ichthyosis (ARCI) in Two Indian Patients.

Bathing suit ichthyosis (BSI) is a rare variant of autosomal recessive congenital ichthyosis (ARCI) due to transglutaminase-1 gene () mutations leading to a temperature sensitive phenotype. It is characterized by dark-grey or brownish scaling restricted to the "bathing suit" areas. We report two Indian girls with bathing suit ichthyosis and mutations in (patient 1: homozygous for c.

Clinicopathological study on rickettsial spotted fever from south India.

In a prospective study conducted between November 2006 and April 2008 of 35 patients (male:female ratio 2:1) with proven rickettsial spotted fever, a generalised rash with involvement of palms and soles were seen in 80% of patients. Vasculitis on histopathology of rash was seen in 54%.

Annular cutaneous sarcoidosis with systemic involvement.

Sarcoidosis is a granulomatous disease involving multiple systems. Cutaneous involvement is present in 25% of patients. A 42-year-old woman presented with itchy skin lesions on her face for 5 years duration.

Keratin 17 Mutations in Four Families from India with Pachyonychia Congenita.

Pachyonychia congenita (PC) is a rare autosomal dominant genetic skin disorder due to a mutation in any one of the five keratin genes, , , , , or . The main features are palmoplantar keratoderma, plantar pain, and nail dystrophy. Cysts of various types, follicular hyperkeratosis, oral leukokeratosis, hyperhidrosis, and natal teeth may also be present.

Cutaneous metastases from signet cell carcinoma of the gut: A report of two cases.

Cutaneous metastasis from visceral tumors is a rare entity with a reported incidence between 0.3% and 9.8%.

Role of Oxidative and Nitrosative Stress in Pathophysiology of Toxic Epidermal Necrolysis and Stevens Johnson Syndrome-A Pilot Study.

Background: Oxidative and nitrosative stress caused by drug metabolism may be a trigger for keratinocyte apoptosis in the epidermis seen in toxic epidermal necrolysis (TEN) and Stevens Johnson syndrome (SJS).

Aims: To estimate oxidative damage in the serum and to examine the role of nitric oxide in mediating epidermal damage in patients with TEN and SJS.

Materials And Methods: A prospective study was conducted among TEN and SJS patients and controls in a tertiary care center between January 2006 and February 2010.

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.

Ethnic-Specific WRN Mutations in South Asian Werner Syndrome Patients: Potential Founder Effect in Patients with Indian or Pakistani Ancestry.

Werner syndrome is a rare autosomal recessive disorder characterized by multiple features consistent with accelerated aging. It is caused by mutations in the gene, which encodes a RecQ type helicase. To date, more than 70 disease-causing mutations have been reported.

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.