<p style="text-align: justify;">This is a report of a case of a 6-year-old boy who came in due to generalized crusted erosions. His condition started at 2 days of age as appearance of tense vehicles which progress into bullae on normal or erythematous base after mild trauma on the extremities and later become generalized. The vesicles would become ersosions and crusts and heal with minimal residual scarring. As old lesions heal, new vesicles would appear. Nikolsky and Asboe-Hansen signs were negative. No extracutaneous or mucosal involvement was noted. Family history revealed that all of his five siblings had appearance of similar vesicobullous lesions few days after birth. Four of them died during the first six months of life. The older surviving sister, now age 10, showed gradual improvement of the condition as she grew older.Paternal and maternal family histories have no similar lesions. Skin punch biopsy revealed a subepidermal blistering disease. Direct and indirect immunofluoroscence were negative, consistent with generalized atrophic benign epidermolysis bullosa. Treatment of this disorder remains elusive. Management is focused mainly in the prevention of trauma as well as supportive measures. Patient education and genetic counseling are also cornerstones of management as in any genetic disorder. Advances in prenatal testing and gene therapy provide hope for early diagnosis and intervention.p>
Human ; Male ; Child ; Biopsy ; Blister ; Chiroptera ; Cicatrix ; Death ; Early Diagnosis ; Epidermolysis Bullosa, Junctional ; Genetic Counseling ; Genetic Therapy ; Siblings ; Skin ; Wound Healing

<p style="text-align: justify;">This case is reported because of the very rare combination of paraneoplastic pemphigus and myasthenia gravis as paraneoplastic manifestations secondary to thymoma. A case of a 37 year old male who presented with skin lesions initially as papules in the face and thighs that evolved into blisters and became widespread, leaving crusted and hyperpigmented patches and plaques. It was associated with cough, dyspnea, ptotis, dysphagia, dysphonia and facial asymmetry. Histopathology and direct immunofluorescence were consistent with paraneoplastic pemphigus. Anticholinerase test confirmed the diagnosis of myasthenia gravis and histologic finidngs of the anterior mediastinal mass confirmed it as thymoma.p>
Human ; Male ; Adult ; Blister ; Cough ; Deglutition Disorders ; Dysphonia ; Dyspnea ; Facial Asymmetry ; Fluorescent Antibody Technique, Direct ; Hoarseness ; Myasthenia Gravis ; Paraneoplastic Syndromes ; Pemphigus ; Thymoma

We report a case of an 11-year-old girl who presented with a gradually enlarging verrucous plaque on the left knee for 3 years. Physical examination showed a solitary, slightly erythematous, scaly, verrucous plaque on the left knee measuring about 1.5 cm x 2 cm. Biopsy revealed granulomatous dermatitis consistent with cutaneous tuberculosis. A diagnosis of tuberculosis verrucosa cutis (TBVC) was made and anti-tuberculous therapy was initiated consisting of rifampicin, isoniazid, pyrazinamide and ethambutol for 2 months followed by rifampicin and isoniazid for 4 months. Upon completion of therapy, only a slightly atrophic scar remained, supporting our diagnosis. This report highlights TBVC must be considered in patients with chronic skin lesions in countries with high prevalence of tuberculosis.

<p style="text-align: justify;">BACKGROUND: To date, no multicenter studies have been conducted on the prevalence and clinical profile of AD in the Philippines. Since AD is one of the top 10 skin diseases seen in the outpatients departments of all the Philippine Dermatologic Society (PDS)- accredited institutions, conducting a multicenter study provides important epidemiological information about this disease and serve as a valuable reference for future studies.p> <p style="text-align: justify;">OBJECTIVES: To determine the prevalence and clinical profile of patients with atopic dermatitis (AD) seen at the outpatient departments (OPD) of Philippine Dermatological Society (PDS) - accredited training institutions from 2007 to 2011.p> <p style="text-align: justify;">METHODS: Records of patients with a diagnosis of AD seen from January 1, 2007 to December 31, 2011 were retrieved and clinical data were collected.p> <p style="text-align: justify;">RESULTS: There were 744,673 dermatological consults in the 10 PDS-accredited outpatient clinics from 2007-2011. A total of 4,275 records of atopic dermatitis were reviewed for this study. The prevalence of atopic dermatitis was determined to be 0.57%. Most institutions reported a prevalence rate of less than 1% except for St. Luke's Medical Center (3.36%), and Research Institute for Tropical Medicine (7.07%). More than half of the patients (65.1%) were children between 1 to 12 years old. Twenty-four percent (24%) were infants less than one year. The average age was seven years old while the youngest was one month and the oldest was 94 years old. There were more females (56.1%) than males (42.75%). Bronchial asthma was the most prevalent co-morbid medical condition. Majority of AD patients seen in institutions were newly diagnosed. Those with previous consultations were mostly seen by dermatologists and pediatricians. Moisturizers and topical corticosteroids were the most commonly used topical preparation while antihistamines followed by oral antibiotics were the commonly prescribed oral medications. Follow-up rate was low.p> <p style="text-align: justify;">CONCLUSION: The prevalence of atopic dermatitis among the 10 PDS-accredited institutions is low except for SLMC and RITM. The clinical profile of patients is consistent with published literature. However, this study revealed the patient follow-up is low. This practice needs to be addressed since optimal management of this chronic disease requires close and regular follow-up to prevent complications and irrational drug use.p>
Human ; Male ; Female ; Multicenter Study ; Prevalence ; Patients ; Dermatology

Background: Due to the high prevalence and incidence of leprosy in the Philippines, there is a continuing need to detect and document the occurrence of dapsone-induced hemolytic anemia. Objective: The aim of this study is to determine the incidence of dapsone-induced hemolytic anemia in non-glucose-6-phosphate dehydrogenase deficient leprosy patients receiving multidrug therapy (MDT) in Southern Philippines Medical Center. Methodology: This is a retrospective study through chart review of leprosy patients treated with MDT regimen at Southern Philippines Medical Center from January 2016 to December 2018. The demographic profile, clinical characteristics, hemoglobin and hematocrit concentrations before and after initiation of MDT, the presence of symptoms of anemia, and the occurrence of dapsone-induced hemolytic anemia in leprosy patients were collected. The main outcome measure for this study was the incidence rate of dapsone- induced hemolytic anemia. Statistical-based analysis were used for continuous and categorical data which were summarized using means and standard deviations, and frequencies and percentages, respectively. Results: There was a decrease in the mean hemoglobin and hematocrit levels noted in the majority of patients after initiation of MDT from baseline 143.46 g/dl and 0.44, respectively, to 94 g/dl and 0.28 on the third month of MDT. The incidence rate of dapsone-induced hemolytic anemia during the 3-year period was 20 cases per 100. Conclusion The relatively high incidence rate of dapsone-induced hemolytic anemia highlights the importance of frequent monitoring of hemoglobin and hematocrit concentrations in leprosy patients being treated with multidrug therapy.
Hansen&rsquo ; s disease ; leprosy ; Dapsone ; hemolytic anemia