Amit G. Pandya

Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus

Our scientific knowledge of pemphigus has dramatically progressed in recent years. However, despite the availability of various therapeutic options for the treatment of inflammatory diseases, only a few multicenter controlled trials have helped to define effective therapies in pemphigus. A major obstacle in comparing therapeutic outcomes between centers is the lack of generally accepted definitions and measurements for the clinical evaluation of patients with pemphigus. Common terms and end points of pemphigus are needed so that experts in the field can accurately measure and assess disease extent, activity, severity, and therapeutic response, and thus facilitate and advance clinical trials. This consensus statement from the International Pemphigus Committee represents 2 years of collaborative efforts to attain mutually acceptable common definitions for pemphigus. These should assist in development of consistent reporting of outcomes in future studies.

Disorders of Hyperpigmentation

Hyperpigmentation is a common disorder of the skin, particularly in brown-skinned patients. Melasma is a common cause of facial hyperpigmentation and can be resistant to treatment. A combination of topical creams and gels, chemical peels, and sunscreens may be necessary for significant improvement. Erythema dyschromicum perstans is a dermal pigmentation seen on the trunk and proximal extremities, most commonly presenting in dark-skinned Hispanics. Drug-induced and postinflammatory hyperpigmentation may last for many months after the offending drug or dermatitis has been eliminated. These disorders, including their management, is reviewed in this article.

Melasma: A comprehensive update: Part II

Melasma is a common disorder of hyperpigmentation affecting millions of people worldwide. While it is thought to be triggered or exacerbated by sun exposure and hormones, much remains to be understood about its pathogenesis. A thorough understanding of the etiology of melasma and the research tools available to study this condition are crucial to enhancing management and developing novel targeted therapies of this often frustrating condition.

Definitions and outcome measures for bullous pemphigoid: recommendations by an international panel of experts

Our scientific knowledge of bullous pemphigoid (BP) has dramatically progressed in recent years. However, despite the availability of various therapeutic options for the treatment of inflammatory diseases, only a few multicenter controlled trials have helped to define effective therapies in BP. A major obstacle in sharing multicenter-based evidences for therapeutic efforts is the lack of generally accepted definitions for the clinical evaluation of patients with BP. Common terms and end points of BP are needed so that experts in the field can accurately measure and assess disease extent, activity, severity, and therapeutic response, and thus facilitate and advance clinical trials. These recommendations from the International Pemphigoid Committee represent 2 years of collaborative efforts to attain mutually acceptable common definitions for BP and proposes a disease extent score, the BP Disease Area Index. These items should assist in the development of consistent reporting of outcomes in future BP reports and studies.

Reliability and Convergent Validity of Two Outcome Instruments for Pemphigus

A major obstacle in performing multicenter controlled trials for pemphigus is the lack of a validated disease activity scoring system. Here, we assess the reliability and convergent validity of the PDAI (pemphigus disease area index). A group of 10 dermatologists scored 15 patients with pemphigus to estimate the inter- and intra-rater reliability of the PDAI and the recently described ABSIS (autoimmune bullous skin disorder intensity score) instrument. To assess convergent validity, these tools were also correlated with the Physicians Global Assessment (PGA). Reliability studies demonstrated an intra-class correlation coefficient (ICC) for inter-rater reliability of 0.76 (95% confirdence interval (CI)=0.61-0.91) for the PDAI and 0.77 (0.63-0.91) for the ABSIS. The tools differed most in reliability of assessing skin activity, with an ICC of 0.39 (0.17-0.60) for the ABSIS and 0.86 (0.76-0.95) for the PDAI. Intra-rater test-retest reliability demonstrated an ICC of 0.98 (0.96-1.0) for the PDAI and 0.80 (0.65-0.96) for the ABSIS. The PDAI also correlated more closely with the PGA. We conclude that the PDAI is more reproducible and correlates better with physician impression of extent. Subset analysis suggests that for this population of mild-to-moderate disease activity, the PDAI captures more variability in cutaneous disease than the ABSIS.

NONTUBERCULOUS MYCOBACTERIAL INFECTIONS OF THE SKIN

Nontuberculous mycobacteria are playing an increasingly important role in human disease owing to higher prevalence of antibiotic resistance and immunodeficiency. These organisms cause a variety of cutaneous findings which are often misdiagnosed by the clinician. Compounding this problem is the fact that most mycobacteria require special culture conditions, which if not specifically requested, are frequently not used. Recognition of susceptible patients is imperative and is not limited to the immunocompromised. Successful treatment of mycobacterial infections requires knowledge of currently available and recommended antibiotics followed by tailoring of the antimicrobial regimen after sensitivity testing is performed.

A global survey of the role of ultraviolet radiation and hormonal influences in the development of melasma.

Background It has been generally believed that the four main causes of melasma are pregnancy, hormonal contraception, family history and sun exposure; however, there are few published comprehensive studies that confirm these assertions. The Pigmentary Disorders Academy – an international group of experts in pigmentary disorders – designed and conducted a global survey of women to investigate the effect of these factors on onset and chronicity of melasma and the course of the disease in order to gain a better understanding of the causative factors associated with this disorder, with a particular focus on hormonal factors and UV exposure in females.

Reliability assessment and validation of the Melasma Area and Severity Index (MASI) and a new modified MASI scoring method

BACKGROUND The Melasma Area and Severity Index (MASI), the most commonly used outcome measure for melasma, has not been validated. OBJECTIVE We sought to determine the reliability and validity of the MASI. METHODS After standardized training, 6 raters independently rated 21 patients with mild to severe melasma once daily over a period of 2 days to determine intrarater and interrater reliability. Validation was performed by comparing the MASI with the melasma severity scale. The darkness component of the MASI was validated by comparing it with the difference between mexameter scores for affected versus adjacent normal-appearing skin. The area component of the MASI was validated by comparing it with the area of each section of the face determined by computer-based measurement software. RESULTS The MASI score showed good reliability within and between raters and was found to be valid when compared with the melasma severity scale, mexameter scores, and area measurements. Homogeneity assessment by raters showed the least agreement and can be removed from the MASI score without any loss of reliability. LIMITATIONS Patients were limited to Hispanic, African, and Asian backgrounds. CONCLUSION The MASI is a reliable measure of melasma severity. Area of involvement and darkness are sufficient for accurate measurement of the severity of melasma and homogeneity can be eliminated.

Continuing medical educationMelasma: A comprehensive update: Part I

Melasma is a common disorder of hyperpigmentation affecting millions of people worldwide. While it is thought to be triggered or exacerbated by sun exposure and hormones, much remains to be understood about its pathogenesis. A thorough understanding of the etiology of melasma and the research tools available to study this condition are crucial to enhancing management and developing novel targeted therapies of this often frustrating condition.

Minocycline effect on meibomian gland lipids in meibomianitis patients.

The objective of this research was to determine the effect of oral minocycline on the meibomian gland nonpolar and free fatty acid lipids of chronic blepharitis patients. Patients--seborrheic blepharitis (SBBL), acne rosacea (AR) without ocular involvement, and acne rosacea with meibomianitis (AR-MKC). Minocycline treatment--50mg orally for 2 weeks followed by 100mg to the end of 3 months; this was followed by 3 more months with no treatment. Meibomian gland secretions (meibum) were collected before treatment, at the end of the 3 months on treatment, and 3 months after stopping treatment. Lipids were separated and analyzed for wax and sterol esters, triglycerides, diglycerides, free cholesterol and free fatty acids. Data were analyzed statistically by ANOVA. Minocycline treatment resulted in decreased diglycerides and free fatty acids in the group AR-MKC, which continued into the second 3 months (off treatment) and was significant. Cholesterol decreased, but triglycerides initially decreased with treatment and then increased when treatment in the group was discontinued (second 3 months); these results, however, were not significant. Thus, minocycline has its greatest effect on lipid types, which result from degradation (lipase) reactions, suggesting a lipase inhibition effect and/or direct effect on ocular flora. This minocycline effect continues even after treatment is discontinued, suggesting a more lasting effect on ocular microflora. Minocycline may be most effective when the treatment period is longer than 3 months. These results give insight into disease mechanisms associated with chronic blepharitis.