Renu George

Acitretin for chemoprevention of non‐melanoma skin cancers in renal transplant recipients

A prospective, open randomized crossover trial was conducted to evaluate the efficacy of acitretin for chemoprevention of squamous cell carcinomas and basal cell carcinomas in renal allograft recipients. Analysis was performed according to the intention‐to treat principle. Twenty‐three patients with previous history of non‐melanoma skin cancer enrolled into the study and were randomly allocated into two groups. They crossed over at the end of 1 year. Eleven (47.8%) patients completed the 2‐year trial. Twelve (52.2%) patients withdrew from the trial. Nine of these withdrew because of side‐effects of acitretin. The majority of the patients who continued with the acitretin could tolerate 25 mg of acitretin daily or on alternate days. The number of squamous cell carcinomas (SCC) observed in patients while on acitretin was significantly lower than that in the drug‐free period (P = 0.002). A similar trend was observed in patients with basal cell carcinomas, but this was not significant and the numbers were small. Side‐effects were a major limiting factor. A severe rebound increase in SCC occurred in one patient after the acitretin was ceased.

DIAGNOSTIC EVALUATION OF THE LUPUS BAND TEST IN DISCOID AND SYSTEMIC LUPUS ERYTHEMATOSUS

Background. The usefulness of the lupus band test (lbt) in the diagnosis of cutaneous lupus erythematosus remains controversial. The study was done to determine the sensitivity, specificity, and predictive value of the lbt in discoid lupus erythematosus (dle) and systemic lupus erythematosus (sle).

The matrix protein Fibulin-5 is at the interface of tissue stiffness and inflammation in fibrosis.

Fibrosis is a pervasive disease in which the excessive deposition of extracellular matrix (ECM) compromises tissue function. Although the underlying mechanisms are mostly unknown, matrix stiffness is increasingly appreciated as a contributor to fibrosis rather than merely a manifestation of the disease. Here we show that the loss of Fibulin-5, an elastic fibre component, not only decreases tissue stiffness, but also diminishes the inflammatory response and abrogates the fibrotic phenotype in a mouse model of cutaneous fibrosis. Increasing matrix stiffness raises the inflammatory response above a threshold level, independent of TGF-β, to stimulate further ECM secretion from fibroblasts and advance the progression of fibrosis. These results suggest that Fibulin-5 may be a therapeutic target to short-circuit this profibrotic feedback loop.

Genital syndromes and syndromic management of vaginal discharge in a community setting

The objective of the study was to determine the community prevalence of genital syndromes in women and evaluate the syndromic management of vaginal discharge in this setting. A representative sample for the state of Tamilnadu was chosen using probability proportional to size cluster technique. Thirty clusters were selected from three districts. Demographic, sexual behaviour, risk factors, clinical and laboratory data were collected from the selected population using a structured questionnaire. Direct smear examination for Trichomonas vaginalis, culture for Neisseria gonorrhoeae and Haemophilus ducreyi, serological tests for syphilis (RPR and TPHA), hepatitis B (Hbs Ag ELISA), IgM and IgG antibodies to HSV2 (Novum diagnostics, Germany) and PCR test for detection of C. trachomatis from urine were done. There were 1157 women in the selected population. On examination, vaginal discharge was the most common genital syndrome (38.4%). The sensitivity, specificity, positive and negative predictive value of vaginal discharge as a marker for STD in women was found to be 43.3%, 61.6%, 10.7% and 91.1%, respectively. We concluded that treatment on the basis of syndromic management would result in over-treatment of 90% of women with vaginal discharge.

The Clinical Profile of Children in India with Pigmentary Anomalies along the Lines of Blaschko and Central Nervous System Manifestations

Abstract:  The aim of this study was to describe the clinical profile of children with congenital pigmentary anomalies along Blaschko lines and the associated manifestations in the central nervous system. Twenty‐six children aged 12 years or less (14 boys and 12 girls), with hypopigmentation and hyperpigmentation along Blaschko lines and central nervous system manifestations were included during the period June 2001 to December 2003. Detailed physical, cutaneous, and systemic examinations were carried out. Relevant investigations included histopathology, karyotyping, electroencephalogram, computerized tomography scans, and magnetic resonance imaging of the brain whenever possible. Twenty children had hyperpigmentation along the lines of Blaschko, four had hypopigmentation, and two had a combination of the two. Eight children had diffuse involvement. Of these, two each had hypomelanosis of Ito, incontinentia pigmenti and linear and whorled nevoid hypermelanosis. The remaining 20 patients could not be categorized as any of the described entities. The majority (92.3%) of patients manifested skin and central nervous system disorders before the age of 2 years. Hyperpigmentation along Blaschko lines was significantly higher in patients with central nervous system manifestations (p = 0.01). Developmental delay was the most frequent central nervous system presentation. Multiple systems were affected, including the eyes. Histology was useful to distinguish incontinentia pigmenti from other types of nevoid hyperpigmentation.

CUTANEOUS LUPUS ERYTHEMATOSUS IN INDIA: IMMUNOFLUORESCENCE PROFILE

The clinical profile and cutaneous lesions of 65 patients with lupus erythematosus (LE) are described. This included 28 discoid LE (13 disseminated, 15 localized), five subacute cutaneous LE, and 32 systemic LE. The need to recognize a pigmented macular form constituting 25% of discoid LE is emphasized. Increased incidence of involvement of the lower lip in discoid LE and pigmentation in systemic LE is noted. Lupus band test was found to be highly sensitive; it was positive for lesional skin of all untreated patients with subacute cutaneous LE and systemic LE, it was, however, not useful on nonlesional skin.

A comparison of economic aspects of hospitalization versus ambulatory care in the management of neuritis occurring in lepra reaction

Neuritis is one of the important causes of deformities and disabilities in leprosy. Neuritis has been managed both in the field and in hospital. This study was done to compare the economic aspects of cost of ambulatory vs in-patient management of neuritis in leprosy. The quality of life of the affected patients and the clinical improvement in the 2 groups were also studied. Twenty six patients fulfilling the study criteria were randomized into the ambulatory and in-patient group (13 in each group). The primary outcome examined was cost, in various categories; the secondary outcomes included pre- and post-treatment comparison of Quality of Life (QOL) scores and tests of sensory and motor function. The direct and indirect medical costs incurred by patients in the hospitalized group were higher than those patients in the ambulatory group. The difference in the direct medical costs between the two groups was Rs. 9110.5, and the extra direct non medical costs incurred by patients in the hospitalized group was Rs. 888.50 because of more frequent visits of family members. A greater percentage of ambulatory than in-patients returned to work in </= 15 days (53.8% vs 15.3%), and the mean duration before returning to work was 19.5 days ambulatory patients compared to 66.8 days for in-patients group. The QOL scores and motor and sensory function tests showed no significant difference between groups. Although the sample size was small, these preliminary results suggest that substantial cost minimization by ambulatory care is possible without significantly affecting the quality of life or peripheral nerve function.

Role of imatinib in the treatment of pediatric onset indolent systemic mastocytosis: a case report

Background: Mastocytosis is a sporadic disease characterized by an abnormal accumulation of mast cells (MCs) in single or multiple organs. It has a heterogeneous clinical picture which is a reflection of underlying tissue MC burden, mediators released and the organs involved. Here, the authors report significant symptomatic, cutaneous and systemic response to imatinib in a case of childhood onset indolent D816V KIT unmutated systemic mastocytosis (SM). Case report: A 19-year-old female presented with a history of itchy skin lesions over the face, trunk and extremities since 6 months of age associated with recurrent bouts of angioedema. The skin and bone marrow examination were consistent with mastocytosis. No pathogenic mutations were detected in exons 8 and 17. In view of the severity of cutaneous symptoms and evidence of bone marrow involvement, she was treated with imatinib which resulted in marked improvement. Conclusion: Imatinib has a therapeutic role in the presence of an imatinib-sensitive KIT mutation or in KIT816-unmutated patients with aggressive SM. Its role in the treatment of indolent and cutaneous mastocytosis is less well established. However, the authors have demonstrated the usefulness of imatinib in the treatment of c-KIT-negative indolent SM with extensive cutaneous involvement.

Systemic inflammatory response syndrome in diseases of the skin

Background A number of dermatological conditions present with features of systemic inflammatory response syndrome (SIRS). This study evaluated the incidence and outcome of SIRS in patients with dermatological diseases. Study design Prospective cohort study. Results Patients admitted to a university hospital with a skin disease and fulfilling at least two SIRS criteria were included. The primary outcome measure was mortality. Secondary outcomes included incidence of multiple organ dysfunction syndrome (MODS), sepsis, severe sepsis and shock. Over 14 months, 2765 inpatients with skin related problems were examined. These included 721 patients admitted directly to the dermatology ward and 2044 patients referred from other departments within the hospital, with cutaneous manifestations. The incidence of SIRS in this cohort was 2.4% (n=67). The mean (SD) age was 32.6 (19.7) years with a male:female ratio of 1.2:1. Cutaneous adverse drug reaction (CADR) was the most common cause of SIRS (35.8%). During hospitalisation, 37 patients (55.2%) developed sepsis, 23 (34.4%) MODS, 15 (22.4%) severe sepsis and 6 (9%) shock. Methicillin sensitive Staphylococcus aureus was the most common skin isolate (41.4%) and Enterococcus the most common blood isolate. Overall mortality was 15% (10/67). Older age, low albumin, MODS, severe sepsis and shock were associated with an increased risk of death (p<0.03). Positive blood cultures, liver or lung involvement were also significantly associated with increased mortality (p<0.01). Conclusion The incidence of SIRS was low in dermatological diseases. In this cohort, CADR was the most common cause of SIRS. Patients who developed sepsis or MODS had a poor outcome.

Linear epidermolytic acanthoma of vulva: An unusual presentation

Epidermolytic acanthoma (EA) is a rare benign tumor that shows epidermolytic hyperkeratosis (EH) on histopathology. It can occur in a solitary or disseminated form. This condition needs to be distinguished from other hereditary or acquired conditions that may show EH. We diagnosed an unusual case of EA of the vulva presenting in a linear pattern in a 50-year-old lady based on the clinical features and typical histopathological findings and stress the importance of considering epidermolytic acanthoma in the differential diagnosis of verrucous lesions of the genitalia.