Dr. R. Sowmiya

Progeria, also known as Hutchinson-Gilford syndrome, is an extremely rare, severe genetic condition wherein symptoms resembling aspects of aging are manifested at an early age. It is an autosomal dominant disorder. It is not seen in siblings of affected children although there are very few case reports of progeria affecting more than one child in a family. Here we are presenting two siblings, a 14-year-old male and a 13-year-old female with features of progeria, suggesting a possible autosomal recessive inheritance.

Background: Prevalence of lichen planus (LP) and generalised lichen planus (GLP) is about 2.6% and 1% respectively. Various studies have shown higher prevalence of Diabetes Mellitus (DM) and stronger association between Hepatitis-C virus (HCV) and LP . The main objective of this study was to predict the prevalence of DM and HCV in patients with GLP before starting steroids. Methods: A case-control study was conducted with 33 patients in each group for one year period. Case group included patients with GLP excluding oral LP and drug induced lichenoid dermatitis. Control group included patients with other skin conditions and not on steroids. Preliminary details and history of DM and HCV were collected. Random blood sugar and Anti-HCV were done. Results: The proportion of patients with GLP was higher in women compared to men (63.6% vs. 36.3%). Highest prevalence was observed in the age group 35-45 years for both genders. On stratification by sex, odds of having GLP in women are 0.76 times lower among diabetics compared to non-diabetics whereas no association in males. There was no association between GLP and HCV in both sexes. Conclusions: There is no significant association between Diabetes mellitus and Hepatitis-C infection with GLP. Future study is planned with larger sample size for a definite conclusion.

Background Generalised lichen planus (GLP) is a chronic disease with an overall prevalence of 1% requiring longer treatment. Limited studies are available on GLP and its treatment in the literature, unlike oral lichen planus. Objectives To determine the best steroid-sparing treatment modality for GLP by comparing the efficacy, response, safety, side effects, and remission with azathioprine, dapsone, and narrowband UV-B (NB-UVB) along with their impact on itching severity and life quality. Methodology Open-label, prospective, comparative, interventional study on generalised lichen planus patients treated with systemic steroids along with one of three steroid-sparing modalities. Totally 90 patients were studied including 30 patients each who received azathioprine (Group A), dapsone (Group B), and narrow band UVB (NB-UVB) (Group C), respectively, for 16 weeks. Itch severity index (ISI) and Dermatology life quality Index (DLQI) were assessed at baseline and week 24. All patients received oral prednisolone until there was no more active disease. Response was assessed in terms of occurrence of new lesions, flattening of lesions, post-inflammatory hyperpigmentation (PIH), and grading of lesions two weeks once for 6 months followed by six months of follow-up after treatment completion. Results Females outnumbered males in all 3 groups. Mean patient ages (34, 38, and 34) and the presence of one or more co-morbidities (50%, 42.3%, 37.5%) in Groups A, B, and C, respectively, were comparable. ISI and DLQI improvement at 24 weeks were greatest with NB-UVB, followed by azathioprine and dapsone in that order; the differences in improvement between groups showed high statistical significance. At week 24, occurrence of new lesions (0%, 0%, 3.8%), flattening (100% – all groups), PIH (100% – all groups), grade 3 lesions i.e. poor response, resolution of 20-50% of lesions (7.1%, 11.5%, 0%), grade 2 lesions i.e. partial response, resolution of 50-90% of lesions (35.7%, 76.9%, 8.3%) and grade 1 lesions i.e. complete response, resolution of >90% lesions (57.1%, 11.5%, 91.3%) were noted in Groups A, B and C, respectively; the differences in the extent of resolution of lesions between the groups were highly significant statistically. Remission was seen in 100%, 76.9%, and 87.5% in Groups A, B, and C, respectively, after six months. Limitations The sample size was small. Only 3 treatment options were compared in this study but many more options have been used for lichen planus. Long term follow-up is required. Conclusions NB-UVB with oral steroids showed a better response in terms of improvement in DLQI, ISI, disease control, and side effects than azathioprine and dapsone. Azathioprine showed a faster response and more prolonged remission. Dapsone showed poor response with multiple side effects.