Haberland syndrome or Fishman syndrome also known as encephalocraniocutaneous lipomatosis (ECCL) is a rare, congenital neurocutaneous disorder. It is characterized by unilateral involvement of skin, eyes and central nervous system. We report the case of a 28-year-old woman who presented with soft lipomatous swelling over right temporal area with nonscarring alopecia of part of frontal and parietal region. The patient had a history of seizures and ipsilateral scleral dermoid. Computed tomography scan findings were suggestive of lipomas and calcification of falx. Magnetic resonance imaging showed right-sided hemi atrophy and two intracranial cysts. We report this case because of its rarity.
Background:In India, leprosy still continues to be one of the major public health problems and demands a continuous awareness for its eradication. The reduction of the load of infection is the cornerstone of leprosy control.Aims:The aim of the present study was to enumerate the epidemiology of leprosy in the Ahmedabad district of Gujarat.Methods:Three hundred new cases of leprosy (multidrug therapy cases) and patients who had completed antileprosy treatment and developed new signs and symptoms after that during the period from June 2010 to December 2012 were included in the study. A detailed history, clinical examination, and relevant tests were done.Results:Out of a total of 300 patients, male-to-female ratio was 2.1:1. Family history was positive in 4.3% of patients. Lepromatous leprosy was seen in 27.3%, pure neuritic leprosy occurred in 12 (3.9%), and smear positivity was seen in (44.6%). Thirty (10%) patients developed type 1 reaction and 32 (10.6%) patients had type 2 reaction. Nearly 1.3% of the cases were relapsing cases.Limitations:The study had geographical limitation and the study did not cater the whole population but only the cases who reported to the hospital. Hence, the social stigma factor associated with leprosy could not be annulled.Conclusions:Leprosy still prevails and adequate knowledge and clinical skills, sensitive diagnostic tools and proper supply of medicines from the medical end and early reporting of symptoms, compliance regarding medicine intake, and social awareness to null social stigma associated with it from the patient's end may bring the country as a whole to the target of leprosy elimination.
<p class="abstract"><strong>Background:</strong> Chronic urticaria (CU) is defined as urticaria persisting daily as or almost daily for more than 6 weeks and affecting 0.1% of the population. In chronic autoimmune urticaria, immunoglobulin G (lgG) auto antibodies react to the alpha subunit of the high-affinity lgE receptor (FceR1) on dermal mast cells and basophils, leading to chronic stimulation of these cells. This results in the release of histamine and other inflammatory mediators which cause urticaria and angioedema. More than half of the patients have autoimmune urticaria. ASST is the only in vivo test to detect autoimmune urticaria. The aim was to study the frequency of ASST positivity among patients with chronic spontaneous urticaria and to identify the clinical and laboratory parameters associated with positive ASST.</p><p class="abstract"><strong>Methods:</strong> The present study is prospective observational study of chronic spontaneous urticaria. ASST was performed in 250 patients fulfilling exclusion and inclusion criteria. Baseline investigations (CBC, ESR, urine, stool, RBS and CRP) were done for all the patients whereas specific investigations (ANA, Thyroid Profile, IgE, Anti H.Pylori IgG and skin biopsy) were done in selected cases.<strong></strong></p><p class="abstract"><strong>Results:</strong> Out of 250 patients examined, 60% had positive autologous serum skin test suggestive of autoimmune urticaria. H. pylori antibodies were significantly higher (29%) in ASST positive patients. While antinuclear antibody (ANA) was positive in 4% and thyroid antibodies were present in 7% of patients with positive ASST. Serum IgE was elevated in 32% ASST positive patients.</p><p class="abstract"><strong>Conclusions:</strong> ASST is an easy, simple and cost effective screening test for chronic autoimmune urticaria.</p>
Biotinidase deficiency (BD) is a rare autosomal recessive, vitamin-responsive inborn error of metabolism associated with a wide spectrum of dermatological, neurological, auditory, and metabolic abnormalities. This case report reiterates that a high index of suspicion in childhood diseases with periorificial desquamation can lead to timely detection and active intervention in BD.
Pregnancy is one of the most precious, memorable and unique experience in each mother’s life. The pregnant mothers should understand the normal physiological changes that are occurring in their body during the stages of pregnancy and should take into account the health care and lifestyle considerations. With a view to that the investigators conducted a descriptive study to assess the knowledge regarding self-management of minor ailments among antenatal mothers attending antenatal OPD in selected hospital of Dadra and Nagar Haveli. A Structured questionnaire was prepared to assess the knowledge regarding self-management of minor ailments among 40 antenatal mothers. The findings of study shows that 2.5% of antenatal mothers have poor knowledge (0-50%), 75% mothers have average knowledge (50-75%) and 22.5% mothers have good knowledge (75-100%) regarding the minor ailments.
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