Langerhans Cell Histiocytosis (LCH) is a rare disorder characterized by the abnormal proliferation of histiocytes, predominantly affecting the bones and skin. However, it can also involve the bone marrow, liver, spleen, lungs, pituitary gland, central nervous system, and other organs. The disorder is named for the neoplastic cells that resemble dendritic Langerhans cells found in the skin and mucosa.

We present the case of a 2-year-old Filipino female diagnosed with recurrent LCH, highlighting the diagnostic challenges and therapeutic interventions encountered. The patient initially presented with characteristic papules and plaques indicative of LCH. Initial treatment involved multi-agent chemotherapy, which resulted in significant clinical improvement. However, following the cessation of therapy, the patient experienced recurrence of symptoms, necessitating reevaluation. A skin punch biopsy confirmed the diagnosis of LCH, reinforcing the decision to reinitiate chemotherapy. Complications arose during treatment, including febrile neutropenia, which required hospitalization and adjustments to the management plan. After completing the chemotherapy cycles, the patient demonstrated marked clinical improvement, with the resolution of systemic symptoms and a reduction in the severity of cutaneous lesions.

This case underscores the complexities in managing recurrent LCH in pediatric patients. A comprehensive diagnostic evaluation, vigilant monitoring for treatment-related complications, and prompt therapeutic interventions are critical for achieving optimal outcomes. Effective management requires a multidisciplinary approach to address the unique challenges presented, ensuring timely interventions to improve patient outcomes.

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.

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.

Cutaneous tuberculosis is a rare form of extrapulmonary tuberculosis seen in 1% of all cases. Lupus vulgaris is one of the most common types of cutaneous tuberculosis. Its cutaneous patterns may mimic other dermatologic conditions such as pyoderma gangrenosum. Clinical, diagnostics and histopathologic correlation is important in diagnosing lupus vulgaris.

This is a case of a 17-year-old Filipino male with multi-drug resistant Pulmonary Tuberculosis who presented with three-months history of erythematous papule that gradually progressed into plaque on the scalp, abdomen and left popliteal area with associated pruritus 5/10. The initial working diagnosis was cutaneous tuberculosis versus pyoderma gangrenosum. Incision biopsy revealed a granulomatous dermatitis surrounded by a dense mixed cell infiltrate of lymphohistiocytes from superficial to mid dermis suggestive of an infectious process. Chest radiograph confirmed pulmonary tuberculosis, interferon gamma detection by enzyme linked immunosorbent assay (Quantiferon TB Gold Plus) and nucleic acid amplification test (GeneXpert TB) further verified the presence of Mycobacterium tuberculosis (MTB). The patient was managed as lupus vulgaris, plaque type and started on second line anti-Koch’s medications. Excellent clinical response was seen after 3 months of treatment.

Lupus vulgaris is a challenging disease and may mimic a myriad of other cutaneous disorders, in this case pyoderma gangrenosum. This case highlights a high index of suspicion, trained clinical eye and multi-specialty care to diagnose and treat complicated cutaneous tuberculosis cases. In geographic locations where MTB is still endemic and drug resistance burdens pose complications in treatment, second line pharmacologic interventions for MTB treatment is a viable option.

Congenital syphilis is a worldwide public health concern. This occurs when an infected mother transmits the infection to the fetus during pregnancy or at birth.

We present a case of a 6-day-old male, term, born to a mother with secondary syphilis, via normal spontaneous delivery. Upon birth, patient was well and not in cardiorespiratory distress. However, cutaneous examination revealed multiple, well-defined vesicles and pustules on an erythematous background, some topped with erosions and crusts on the scalp, face, extremities, and trunk. Laboratory work-up and imaging were done which revealed congenital syphilis. He was managed with intravenous Penicillin (100,000iu) 160,000 IV for ten days, and wound healing was hastened by use of a coconut-based cellulose wound dressing on the erosions. He was then referred to a multispecialty team to assess and co-manage possible complications. Regular interval follow-up and repeat laboratory tests were advised for observation and for monitoring.

Congenital syphilis is caused by the bacterium Treponema pallidum. Sequelae include preterm birth, low birth weight, skin lesions, bone deformities, hepatosplenomegaly, anemia, and neurological problems. Diagnosis can be made on clinical suspicion combined with Rapid Plasma Reagin (RPR) and Venereal Disease Research Laboratory (VDRL). Aside from Penicillin G, wound care, nutritional build up, and close monitoring of growth and development with regular follow-ups are essential aspects in the management of congenital syphilis. With timely and adequate treatment, infants have a higher likelihood of complete resolution of symptoms, prevention of long-term complications, and improved overall health outcomes.

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.

Palmoplantar psoriasis is a rare subtype of psoriasis. It is a chronic, relapsing, inflammatory, immunologically-mediated disease affecting the palms and soles.

This is a case of a 58-year-old female with multiple, well-defined, yellowish plaques on slightly erythematous base with some fissures on bilateral palmoplantar areas affecting 4% of body surface area. On Dermatology Life Quality Index, she scored 14. She was advised biopsy but deferred. The lesions were also tested with potassium hydroxide for fungal elements, yielding positive results. She was initially managed as tinea pedis et manuum with three pulse doses of oral antifungal medication for three months. With minimal improvement, patient finally consented for biopsy, confirming diagnosis of psoriasis. She was subsequently treated with potent topical corticosteroids and narrowband-ultraviolet B localized phototherapy, leading to a complete clearance of lesions after 16 weeks of steroid treatment and 52 sessions of phototherapy. There was no more erythema, plaques and fissures, with affected BSA down to 0% and DLQI score to 1. Three months post-treatment, there is still no recurrence of lesions.

Palmoplantar psoriasis is an uncommon variant of psoriasis featuring hyperkeratotic plaques and fissures limited to the palms and soles with associated significant functional impairment. It can be difficult to diagnose, often mistaken for other diseases, and is typically resistant to treatment with poor long-term remission. Biopsy plays a crucial part in the effective management especially among patients with refractory disease. There are numerous treatment modalities but psychosocial needs are equally important to be addressed.

Klippel-Trenaunay syndrome (KTS) is a rare slow-flow congenital vascular disorder with an incidence of 1:100,000. 1 , 2 KTS is classically characterized by a clinical triad of capillary malformation, venous malformation, and bony or soft tissue hypertrophy. RAD50 and POLE genes act directly on deoxyribonucleicacid (DNA) and genome stability. Although distinct from the more studiedphosphatidylinositol-4,5-bisphosphate3-kinase catalytic subunit alpha (PIK3CA)gene, RAD50 and POLE genes coexist as a deficient gene in few vascular malformations and papillary thyroid carcinoma (PTC).

This is a case of a 7-month-old Filipino female patient clinically and radiologically diagnosed as KTS presenting with multiple capillary malformations and left limb length-girth discrepancies. Dermoscopy showed various vessel patterns in all affected areas. Soft tissue ultrasound and magnetic resonance imaging/angiography (MRI/MRA) of the left extremities revealed subcutaneous capillary malformations, hypertrophy of the subcutaneous structures and compartment muscles. Strong family history of PTC was elicited and genetic sequencing revealed detected RAD50 and POLE genes. She was treated using the mammalian target of rapamycin inhibitor sirolimus with careful monitoring of trough levels and radiographic tests. A significant outcome one year post-sirolimus revealed no abnormal vessels on ultrasound, a lesser degree of hypertrophy and capillary malformations were no longer appreciated in MRI/MRA of left extremities. Port-wine stains (PWS) and affected limbs showed a decrease in erythema and growth rate during the treatment period.

KTS detected with RAD50 and POLE genes successfully treated with sirolimus with trough-level monitoring. Radiographic evaluation and regular anthropometric assessment remain valuable in the diagnosis and monitoring.

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

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.