Pemphigus foliaceus treated with rituximab monotherapy

In pemphigus foliaceus (PF), immunoglobulin G (IgG) autoantibodies directed against desmoglein-1 (Dsg-1), a cell adhesion molecule expressed mainly in the granular layer of the epidermis, are responsible for the intercellular widening between desmosomes resulting in intraepidermal blisters. Rituximab is a chimeric monoclonal antibody that by binding specifically to the transmembrane antigen CD20 found on the surface of normal and malignant B cells, leads to B-cell depletion. We report a 19-year-old Filipino woman with PF and controlled idiopathic thrombocytopenia purpura, initially treated with high-dose prednisone and azathioprine. Due to rapid PF progression with associated moderate reactive depression, rituximab was added to the treatment regimen with prompt improvement of lesions and clearance after five months. Five years later, lesions recurred with erythematous, dry, scaly plaques on both breasts, axillae, and on the scalp, associated with moderate to severe intermittent pruritus. After the first of a series of four weekly infusions, rituximab monotherapy resulted in immediate and sustained clearance up to 22 months. In parallel with skin clearance, serum CD19 and CD20 B cells decreased to almost zero after the first infusion, to zero after the second, while the decrease of Dsg-1 levels was more gradual, and down to normal after four months.We offer this case report to show that rituximab can be given as a first-line monotherapy option for indications similar to ours such as drug reactions (steroid-induced depression) or a history of recalcitrant PF to the usual medications; and to suggest using CD19 and CD20 in addition to the desmoglein levels to monitor disease activity and molecular change from which to learn how to continue to monitor for disease activity after clearance.

A multi-center retrospective study on atopic dermatitis at the outpatient departments of Philippine Dermatological Society-accredited training institutions from 2007-2011

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. 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. 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. 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. 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.

Ustekinumab for adult Filipino patients with moderate to severe chronic plaque psoriasis: A clinical series

BACKGROUND: Psoriasis is a chronic inflammatory disease occurs worldwide. At the Philippine General Hospital dermatology clinic, psoriasis accounted for 2.2% -2.4 % of new consults seen in 2004-2010. Its pathogenesis remains obscure but current studies indicate that activated Thai and Thai response mechanisms mediate inflammation and are implicated as key players in psoriasis genesis. Ustekinumab is a fully human monoclonal antibody that targets two interleukins (IL): IL-12 and IL-23 which influence T-cell differentiation into Thi and Thai, respectively. These naturally occurring proteins help regulate the immune system secondary to their role in linking innate and adaptive immune responses.CASE SERIES: This is a retrospective chart review on the use of ustekinumab in 22 adult Filipinos (10 males and 12 females) conducted at six (6) dermatologists' clinics in 2010. Included were patients enrolled in the Named Patient Program (NPP) of Janssen Pharmaceutical Companies of Johnson & Johnson Philippines, diagnosed with moderate to severe long-standing plaque psoriasis and contraindicated for, or with inadequate response to, conventional systemic treatment. Patients received ustekinumab subcutaneously at loading doses of 45mg during the initial visit and at four weeks. Subsequently, it was given every twelve weeks. For patients who weighed 100 kg or more, 90mg of ustekinumab was administered. Clinical responses to the drug were assessed using Psoriasis Area and Severity Index (PASI) at initial visit and at the end of the program (52 weeks). At the end of the one-year program period, the median (range) PASI score of patients was 1.50 (0-29.2). Sixteen of the twenty-two subjects (72.73%) were able to achieve ±75% improvement from baseline (PASI 75). There was a significant (94.52%) reduction in median PASI scores of the patients from baseline to end visit (p CONCLUSION: Ustekinumab was shown to significantly reduce the median PASI scores of 22 adult Filipino patients with moderate to severe plaque psoriasis. It was also shown to be well tolerated, with relatively mild adverse events.