Hyper-Immunoglobulin E Syndrome: Case Series of 6 Children from India.

Hyper-immunoglobulin E syndrome is a rare primary immunodeficiency syndrome characterized by severe atopic dermatitis, recurrent pulmonary and staphylococcal skin infections. Its diagnosis requires a high degree of suspicion, typical clinical features, and not mere rise in serum IgE levels. Genetic studies are not always possible in a resource poor setting in developing countries. In this case series, all children had recurrent eczematoid rash, secondary infections, multiple episodes of hospitalization for pulmonary infection and raised serum IgE levels. Diagnostic genetic study was feasible in only one of the cases which revealed pathogenic homozygous deletions of exons 15 to 18 (Transcript: NM_203447) in DOCK8 gene. The main goal of management of hyper-immunoglobulin E syndrome is aggressive treatment of infections and optimum skin care. Our case series highlights various characteristic, presentations, and management of this rare syndrome childhood cases. Awareness of these manifestations may facilitate early identification and contribute to optimal care of patients as representative data on the same is limited in literature.

DRESS, the maverick among SCARS: A case series-based review of literature.

DRESS is a potentially life-threatening severe cutaneous adverse reaction (SCAR). Historically, it was most frequently linked with phenytoin and was initially described as phenytoin hypersensitivity syndrome; however, it was later found to be caused by various other medications, with the commonest been aromatic anticonvulsants, allopurinol and sulfonamides. The severity of this entity is related to systemic involvement, which can result in multiorgan failure and death. The diagnosis of DRESS, especially in the early stages, remains challenging and elusive due to its heterogeneous clinical presentation and the complex course of the disease with different patterns depending on the causal drug. The most important step in the management of DRESS is early diagnosis and immediate cessation of the suspected offending drug along with oral steroids or immunosuppressants to control the disease. We describe the varying presentation and management of six adults with DRESS from a tertiary care hospital, observed over a two-year period with a brief review of the literature.

An observational study on ocular manifestations in patients of psoriasis in a tertiary care hospital in western India.

Background: The aim of the study is to observe the ocular manifestation in patients of psoriasis. Methods: All the diagnosed cases of Psoriasis by the dermatology department of this tertiary care hospital were included in this study. Relevant details of the history pertaining to disease duration, type of psoriasis, and treatment undertaken including ocular symptoms were obtained. Disease severity was quantified using the PASI score. Complete ocular examination including intraocular pressure, Schirmer I and II tests, Tear Film Breakup Tme (TBUT); was carried out for all the patients. Results: Of 126 patients of psoriasis, ocular manifestations were seen in 76 patients (60.3%). Dry eyes (27%) and blepharitis (15.9%) were the most common ocular manifestations. Uveitis was seen in 3.2% of the patients of which 75% patients were HA B27-positive psoriatic arthritis, which was statistically significant (p = 0.001). There was no statistical correlation between duration of the disease and ocular manifestations (p value is 0.077 using chi square test). The ocular manifestations were more common in patients with PASI score 10 when compared with the patients with PASI score 10 (p value = 0.028) which was statistically significant. Conclusions: In our study, prevalence of ocular manifestation was 60.3% which increased with the increasing PASI score. Dry eyes and blepharitis were the most common manifestations. Hence, routine ocular examination is recommended in patients with psoriasis.

Awareness of Leprosy in an urban slum of Western Maharashtra Post 35 Years of the National Leprosy Eradication Program (NLEP).

Background: Even after 35 years of the National Leprosy Eradication Program (NLEP) and 15 years post-elimination, leprosy continues to be a public health challenge in India. This paper discusses the current awareness of leprosy among people living in urban slums of western Maharashtra. Methods: The study was conducted in an urban slum of western Maharashtra with 400 participants. A closed-ended questionnaire regarding the knowledge, attitude, practices, and stigma existing among the people was administered, followed by a small awareness talk and screening for leprosy. Results: Of the total 400 participants, 205 (51.25) were females and 195 (48.75) were males. Only 154/400 (38.5%) people were aware of leprosy. 130/400 (32.5%) people thought that it is treatable; however, 71/130 (54.6) of them thought that it would recur even after completing the treatment. Only 103/400 (25.75) said that they would marry a person with leprosy, denoting prevalent stigma in the society, and 79/400 (19.75) were aware of government services for leprosy and NLEP. Screening of all the participants surveyed did not reveal any new or doubtful cases of leprosy. Conclusion: The present study shows a lack of awareness and knowledge of leprosy among the target population. With only 20% of them being aware of government services and the NLEP, combined with an extremely low knowledge about the disease; it shows the need to further augment the government programs. There is also an increasing need to educate people to accomplish a positive attitude of the community towards leprosy patients.