1.A rare case of juvenile dermatomyositis in a 2-year-old Filipino female with pulmonary tuberculosis and ascariasis
Maria Monica L. Manalo ; Wilsie M. Salas-Walinsundin
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):3-3
Juvenile Dermatomyositis (JDM) is a rare type of idiopathic inflammatory myopathy affecting children, characterized by symmetric proximal muscle weakness and pathognomonic cutaneous manifestation such as heliotrope rash and Gottron papules. In the Philippines, there are only 40 cases from 2011 to 2022. It is an autoimmune disease, although several studies have associated its onset to the presence of systemic infections. In cases complicated by systemic infection, early initiation of comprehensive treatment is essential in order to achieve remission.
This is a case of a 2-year old female presenting with a 2 month history of erythematous macules over the metacarpophalangeal (MCP), proximal interphalangeal (PIP), distal interphalangeal joints (DIP), and knees, nailfold changes, and facial erythema on sun exposure. This was associated with decreased activity, inability to walk continuously, and symmetric proximal muscle weakness. Skin punch biopsy was done which revealed interface vacuolar dermatitis, alcian blue stain positive. Laboratories revealed elevated ANA, aldolase, LDH, and SGPT which were all consistent with dermatomyositis. Patient was started on oral prednisone, hydroxychloroquine and topical corticosteroids. Notably, the patient was also diagnosed with pulmonary tuberculosis and ascariasis. Thus, she was also started on anti-Kochs regimen and mebendazole. After 2 months of steroid therapy and hydroxychloroquine, there was improvement in cutaneous lesions with significant increase in activity and mobility.
In a country where pulmonary tuberculosis and ascariasis is common, it is of utmost importance to probe for underlying infections which may occur with or may be contributory to the onset of juvenile dermatomyositis.
Human ; Female ; Child Preschool: 2-5 Yrs Old ; Ascariasis ; Juvenile Dermatomyositis ; Dermatomyositis ; Tuberculosis, Pulmonary
2.A case of linear IgA bullous dermatosis in a 7-year-old Filipino female
Charlotte Giselle Lu Ty ; Wilsie M. Salas-Walinsundin
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):11-12
Linear IgA Bullous Dermatosis (LABD) is an autoimmune mucocutaneous disease characterized by linear deposits of IgA at the basement membrane zone on immunopathology. Direct immunofluorescence (DIF) remains the gold standard for diagnosis. Dapsone is the most commonly used therapeutic agent, but potential side effects such as hemolysis, agranulocytosis and methemoglobinemia in G6PD deficient patients necessitate the use of other treatments such as topical corticosteroids, tetracyclines, erythromycin, sulfonamides, nicotinamide, rituximab, omalizumab, methotrexate, cyclosporine and intravenous immunoglobulin.
We present a 7 year old Filipino female presenting with a 19 day history of multiple erythematous pruritic papules on the lower extremities which progressed to vesicles and bullae. Laboratory tests showed increased WBC, Koch’s Infection on Chest X-ray and a low G6PD Enzyme Assay. Skin punch biopsy and DIF also showed subepidermal blistering disease and findings consistent with LABD. The patient was placed on Prednisone 20 mg/5 mL syrup 5 mL for 2 weeks, Erythromycin 250 mg/5 mL syrup and Niacinamide 300 mg/paper tab for 1 month. She was also treated with Clobetasol Propionate 0.05% ointment and Betamethasone Valerate 0.1% ointment OD for 1 month. On follow up, the patient was well with no new onset of vesicles and bullae.
LABD has relied on Direct Immunofluorescence (DIF) for diagnosis and Dapsone as its first line treatment. Given that the patient has a low G6PD result, Dapsone was not given. Instead, systemic antibiotic and corticosteroids, Niacinamide supplement and topical corticosteroids was used as treatment. Patient factors such as age, comorbidities and disease severity play a role in therapeutic selection.
Human ; Female ; Child: 6-12 Yrs Old
3.Shell to success: A journey through rare ostraceous psoriasis in pediatrics and the impact of targeted therapies
May G. Silva ; Wilsie M. Salas-Walinsundin ; Marie Len Camaclang-Balmores ; Criselda L. David ; Vilma C. Ramilo
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):20-21
Psoriasis may manifest as severe hyperkeratotic lesions resembling an oyster shell called ostraceous psoriasis. This type of psoriasis is extremely rare and is often associated with psoriatic arthritis. Psoriatic arthritis is a chronic inflammatory disease of the joints presenting with pain, stiffness, swelling, tenderness, and limited movements. This is a case of a 16-year-old Filipino female presenting with pruritic erythematous plaques topped with thick adherent ostraceous scales associated with bilateral knee and elbow pains. Laboratory tests and biopsy were done. Histopathology is consistent with psoriasiform dermatitis and psoriatic arthritis is established through Early Psoriatic Arthritis (EARP) Screening Questionnaire and Classification Criteria for Psoriatic Arthritis (CASPAR). Patient was started on potent topical corticosteroids and Narrow-band Ultraviolet B (NB-UVB) phototherapy with minimal improvement. Hence, Secukinumab was initiated which showed significant improvement on the skin lesions and joint pains 7 days after the first dose of Secukinumab. Ostraceous psoriasis is rarely found in pediatric population. It is commonly associated with psoriatic arthritis that should be screened during routine consult. Its characteristic firmly adherent thick scales are resistant to topical treatments. One of the therapeutic options is Secukinumab, an IL-17A selective inhibitor targeting the release of proinflammatory cytokines, chemokines and mediators of tissue damage. It demonstrates immediate effect, significant and sustained improvement in the skin lesions, minimal adverse reactions, as well as improved quality of life and physical function. It is therefore a preferred treatment for patients with moderate to severe psoriasis and psoriatic arthritis requiring rapid clearance.
Human ; Female ; Adolescent: 13-18 Yrs Old ; Arthritis, Psoriatic ; Secukinumab
4.Knowledge, attitude, perception and practices of primary care physicians regarding common dermatological diseases: A cross-sectional study
Tanya Patricia A. Marasigan ; Ma. Angela M Lavadia ; Wilsie Salas-Walinsundin
Journal of the Philippine Dermatological Society 2022;31(2):21-30
Introduction:
Dermatologic diseases are one of the common reasons for consult in primary care. Primary care physicians such as the doctors
to the barrios (DTTBs) assigned in geographically isolated areas play a critical role in providing primary skin health services since they are the
first, and sometimes, only doctors patients rely on for consult. In managing skin diseases, adequate knowledge and skills are needed to arrive at
a correct diagnosis, and a physician’s proper initial treatment and timely referral to dermatologists will lead to less affectation on quality of life.
Identifying what is lacking in skin health services and referral system would aid dermatologists in finding out how to bridge the gap in knowledge
and access to our specialized skin health care.
Objectives:
The study aimed to determine the knowledge, attitudes, practices, and perceptions of primary care physicians in the Philippines
regarding common dermatologic conditions.
Methods:
This is a cross-sectional study that utilized a self-administered questionnaire to collect data from rural government primary care phy-
sicians (doctors to the barrios). At the end of the study, the proponents collated the data and data analysis was done using STATA 13.1 guided by a
statistician.
Results:
A total of 118 DTTBs were included in the study. The mean age of the physicians is 28 years old. One-half of the physicians were assigned to
low-income class municipalities; 26.85% and 25% are from 4th class and 5th class municipalities respectively. Factors such as age, sex and clinical
experience were not associated with level of knowledge of the respondents. Majority or 55% of the primary care physicians were classified as hav-
ing insufficient knowledge on common skin diseases. The respondents have an average of 250 consultations per week and 6% of these are derma-
tologic diseases. The most common skin diseases they encountered were impetigo (46.61%), scabies (46.61%), contact dermatitis (43.22%), fungal
infection (25.42%) and cellulitis (20.34%). The most prescribed and available medications for skin diseases are oral and systemic antibiotics. Only
42.37% of the physicians are able to refer to dermatologists through various online communication platforms while the rest refer through phone
calls or advise their patients to seek dermatologic consult. 25% of the respondents have direct access to dermatologists. DTTBs also have recog-
nized the importance of having adequate knowledge on skin diseases and majority are very interested in learning more about these conditions.
The top 3 barriers to the proper management of skin diseases were lack of training, lack of experience, and lack of medications. Most of the re-
spondents have rated themselves as average to bad in their perceived competency in diagnosing and managing different common skin diseases.
Conclusion
The study showed that majority of the respondents had insufficient knowledge on the diagnosis and management of common der-
matological diseases but had a strong interest to learn more. A significant number of the respondents do not always refer to dermatologists and
have poor access to specialty care. Overall, our findings suggest that there are indeed barriers to delivery of skin-related health services that
should be addressed.
Physicians, Primary Care
5.Guidelines for the management of atopic dermatitis: A literature review and consensus statement of the Philippine Dermatological Society
Ma. Teresita Gabriel ; Hester Gail Lim ; Zharlah Gulmatico-Flores ; Gisella U. Adasa ; Ma. Angela M. Lavadia ; Lillian L. Villafuerte ; Blossom Tian Chan ; Ma. Angela T. Cumagun ; Carmela Augusta F. Dayrit-Castro ; Maria Victoria C. Dizon ; Angela Katrina Esguerra ; Niñ ; a Gabaton ; Cindy Jao Tan ; Marie Eleanore O. Nicolas ; Julie W. Pabico ; Maria Lourdes H. Palmero ; Noemie S. Ramos ; Cecilia R. Rosete ; Wilsie Salas Walinsundin ; Jennifer Aileen Ang-Tangtatco ; Donna Marie L. Sarrosa ; Ma. Purita Paz-Lao
Journal of the Philippine Dermatological Society 2020;29(2):35-58
Introduction: Atopic dermatitis (AD) is a complex disease with an interplay of genetic and environmental factors. In the United States, AD affects 10.7% of children andyc 7.2% of adults. Similarly in the Philippines, the prevalence of AD is 12.7% in the under 18 population, and 2% in the over 18 population. While AD affects all ages, the burden of the disease is greater in the pediatric population. The pathogenesis of AD is multifactorial. Variations in genes responsible for epidermal barrier function, keratinocyte terminal differentiation, and the innate and adaptive immune responses have been linked to AD. A null mutation involving the filaggrin gene is the strongest known risk factor for AD. This mutation results in a loss of filaggrin (FLG) protein by at least 50%. Filaggrin breakdown products form part of the natural moisturizing factor (NMF) of the skin, which is essential in skin hydration. A decrease in NMF and an increase in transepidermal water loss (TEWL) are observed in AD patients with FLG mutation. The defective barrier in AD patients decreases skin defenses against irritation and allergen penetration. Exposure to certain environmental chemicals like formaldehyde may worsen this barrier. This may lead to increased skin permeability to aeroallergens that leads to dermatitis in sensitized patients. Barrier defects may also play a role in epicutaneous sensitization and the subsequent development of other atopic conditions, such as bronchial asthma and allergic rhinitis. The Philippine Dermatological Society (PDS) consensus on AD aims to provide a comprehensive guideline and evidence-based recommendations in the management of this condition, with consideration of cultural factors that are often encountered in the Philippine setting. These guidelines are intended to provide practitioners with an overview of the holistic approach in the management of AD, ameliorating the negative effects of the disease and improving overall quality of life..
Methodology: A group of 21 board-certified dermatologists from the Philippine Dermatological Society (PDS) convened to discuss aspects in the clinical management of AD. Database and literature search included the full-text articles of observational studies, randomized controlled clinical trials, and observational studies using the Cochrane library, PubMed, Hardin (for Philippine based studies) as well as data from the PDS health information system. The terms used in combinations from the literature included “atopic dermatitis”, “atopic eczema”, “emollients”, “topical corticosteroids”, “topical calcineurin inhibitors”, “anti-histamines” and “phototherapy”. A total of fifty (50) full text articles were reviewed and found applicable for the scope of the study. Articles were assessed using the modified Jadad scale, with score interpretations as follows: (5- excellent, 3- good, 1– poor). Consensus guidelines for AD from within and outside of the region were also reviewed, from the 2013 Asia-Pacific guidelines, 2014 Taiwanese Dermatological Association consensus, 2016 guidelines in the management of AD in Singapore, 2014 American Academy of Dermatology guidelines, and the 2020 Japanese guidelines for AD. From the literature review, proposed consensus statements were developed, and a Delphi survey was conducted over two separate virtual meetings. Individual dermatologists provided Likert Scoring (1-
strongly disagree to 5- strongly agree) based on consensus statements. A consensus was deemed reached at mean scores of > 4.00, a near consensus at > 3.5, and no consensus at <3.5.
Summary: AD is a chronic relapsing condition with a significant burden of disease, most commonly affecting the pediatric population. The PDS AD Consensus Guidelines summarizes the standards of therapy and the therapeutic ladder in the management of AD based on published clinical trials and literature review. While these modalities remain the cornerstone of therapy, an individualized approach is the key to the holistic management of an AD patient. Knowledge and awareness of frequently associated conditions, whether in the realm of food allergies, contact allergies, or secondary infections, is paramount. In addition to the standard therapeutic armamentarium, the physician must also consider cultural practices and be knowledgeable of alternative therapeutic options. Referral to a specialist is recommended for recalcitrant cases of AD, or when initiation of systemic immunosuppressive agents, phototherapy, or biologic agents is contemplated.