Harlequin Ichthyosis in a Filipino newborn: Management pearls in a resource-limited setting

Introduction@#Harlequin ichthyosis (HI) is a rare type of autosomal recessive congenital ichthyosis. There are approximately 200 documented cases worldwide, with less than five published reports in the Philippines. Despite its rarity, current literature suggests a better prognosis for these patients. @*Case description@#We describe a preterm male newborn who presented at birth enclosed in a thick hyperkeratotic armor-like scale plates with areas of fissures, with associated ectropion, conjunctiva dehiscence, and eclabium. The thickened encasement also covered the hands and feet, causing severe contractures. A diagnosis of harlequin ichthyosis was given based on the clinical features. The patient was managed through a multidisciplinary approach, including referral to the tele-ichthyosis platform of a US-based foundation for patients with ichthyosis. Thermoregulation, nutrition, and hydration were carefully managed. Bland emollients were applied generously following normal saline soaks to improve barrier protection. Acitretin was administered on day 2 of life to facilitate the desquamation of the thickened encasement. A marked decrease in erythema and the thickness of the hyperkeratotic skin, and reduced conjunctival dehiscence were noted after one week of therapy. However, the constrictions on the hands and feet showed bluish discoloration and signs of necrosis. Linear band excision was performed to release the constrictors. Despite aggressive management, the patient succumbed to sepsis on day 12 of life. @*Conclusion@#Improved prognosis amongst HI patients is correlated with optimal quality of care regardless of resource limitations. A multidisciplinary approach and early administration of retinoids cannot be overemphasized. Linear band excision within the first week of life is suggested for constrictions on the extremities that do not improve with retinoids to avoid necrosis and autoamputation.

Traversing the spectrum of non-Langerhans Cell Histiocytosis: A case of Rosai-Dorfman Disease with features of Necrobiotic Xanthogranuloma

Introduction@#Non-Langerhans cell histiocytoses (non-LCH) are a group of rare diseases with varied clinical manifestations and overlapping features seen among the subtypes. Here, we present a case of Rosai-Dorfman disease with features of necrobiotic xanthogranuloma. @*Case@#A 45-year-old female presented with a 10-year history of an enlarging neck mass with normal overlying skin accompanied by dysphagia and multiple asymptomatic pink to yellowish-brown papules, nodules, and plaques on the face, trunk and extremities. Biopsies of a skin nodule and plaque revealed granulomatous dermal infiltrates (lymphocytes, foamy histiocytes, and Touton giant cells), emperipolesis and areas of necrosis. CD1A and Fite-Faraco staining showed negative results while CD68 and S100 positively stained the tissues of interest. Histopathology of the neck mass paralleled these findings in addition to being negative for lymphoid markers. Patient had monoclonal gammopathy and thyromegaly with enlarged cervical lymph nodes on further tests and imaging. Intralesional and systemic steroids were given which led to flattening of skin lesions and improvement in dysphagia, respectively. @*Conclusion@#Diagnosis and classification of a particular type of non-LCH may be difficult due to similarities across its subtypes. Hence, it is our belief that these diseases may occur on a spectrum. Treatment involves a multidisciplinary approach for the best possible care.

Complementary and alternative medicine use and quality of life among Filipino Adult Psoriasis Vulgaris Patients seen in a tertiary center: A cross-sectional study

Objective@#To determine the prevalence of complementary and alternative medicine (CAM) use and its association with quality of life (QOL) among Filipino adult psoriasis vulgaris patients. @*Methods@#A cross-sectional study was conducted in an outpatient dermatology department of a tertiary hospital, using a semi-structured, interview-guided questionnaire, and a self-administered QOL questionnaire, the dermatology life quality index (DLQI). @*Results@#A total of 135 Filipino adult patients with psoriasis vulgaris were included. The prevalence of CAM use was 47%, with most CAM users being female and single. Completion of tertiary education was found significantly associated with CAM use (p < 0.05). A greater body surface area involvement and longer disease duration were more common among CAM users but these were not statistically significant. Special diet (56.3%) was the most commonly used type of CAM, followed by herbal medicine (46.9%), bath therapy (18.9%) and faith healing (12.5%). Major sources of CAM information were families (43.8%), internet/social media (28.1%) and health professionals (25%). Around 40% of the participants used CAM out of curiosity. The mean DLQI score of the respondents was 11.3 (±7.3) corresponding to poor quality of life. CAM use was significantly associated with negative impact on physical symptoms and feelings, daily activities, and work and school (P = 0.044; P = 0.019; P = 0.047). After adjusting for confounding variables, patients with poor QOL were twice more likely to use CAM but this was not statistically significant (odds ratio [OR], 1.76; 95% confidence interval [CI], 0.78-3.95; P = 0.17). @*Conclusions@#The use of CAM is prevalent among Filipino adult patients with psoriasis vulgaris. The significant association between CAM use and a poor quality of life may reflect the unmet physical and psychosocial needs of patients. A patient-perspective approach should acknowledge the reasons for CAM use, which could guide the physicians in imparting available scientific evidence, or the lack thereof, for the use of CAM to these patients.

Efficacy and safety of topical Adenosine for Androgenetic Alopecia in adults: A systematic review

Background@#Androgenetic alopecia (AGA), also known as pattern hair loss, is the most common type of hair loss in men and women. Due to very limited therapeutic options, search for other effective and safe drugs is necessary.@*Objectives@#This review aims to evaluate the efficacy and safety of a potential treatment option, topical adenosine, for AGA in male and female adults.@*Methods@#A search of databases (Cochrane Library, Pubmed Medline, and others) was performed with no time limitations placed. We included human interventional studies published in English involving the use of topical adenosine for AGA in healthy adult males and females. Risk bias assessment was performed using the Cochrane Collaboration criteria.@*Results@#All four trials in this review, with a total of 260 participants, used 0.75% topical adenosine lotion twice a day for a period of 6-12 months. Comparators were placebo, topical niacinamide, and topical minoxidil. Evaluated parameters include improvement in baldness grading as assessed by dermatologists and investigators, improvement and satisfaction as assessed by participants, anagen growth, thick/thin/vellus hair ratio, and hair density. Two trials found significant improvement with thick hair ratio (>60 or >80 μm) with the use of topical adenosine while two trials showed higher overall participant satisfaction with topical adenosine. Few to no adverse effects were reported with its use. @*Conclusion@#This is the first systematic review involving topical adenosine for AGA. Topical adenosine may be effective in increasing thick hair ratio and improving the self-perception of hair growth. With minimal to no adverse effects, it may serve as an adjunct or alternative to present treatment options. However, more studies are needed to strengthen these findings.

A case of Sneddon-Wilkinson disease with hypersensitivity to dapsone successfully managed with colchicine

Introduction@#Sneddon-Wilkinson disease (SWD) is a rare, recurrent neutrophilic dermatosis presenting as sterile pustules, with a predilection for flexural and intertriginous areas.@*Case summary@#A 49-year-old Filipino female presented with a three-year history of recurrent pustules and papules on the flexural areas of trunk and extremities. Skin punch biopsy was done and histopathology was consistent with subcorneal pustular dermatosis/SWD. She was started on Dapsone but after two weeks of intake, the patient developed generalized erythematous desquamating plaques on the trunk and extremities, with palmoplantar involvement. The patient did not have fever, jaundice, lymphadenopathy, and abdominal tenderness. Laboratory investigation such as complete blood count and liver function tests were normal. The final diagnosis was SWD with hypersensitivity to Dapsone. Dapsone was immediately discontinued and she was shifted to oral colchicine. After six weeks of oral colchicine therapy, the lesions have completely resolved. Patient was in remission for six months thereafter.@*Conclusion@#SWD is rare and the drug of choice is dapsone. In instances where dapsone is not suitable, oral colchicine can be an ideal alternative treatment.

Treatment of morphea with narrowband ultraviolet B: A case series

Introduction@#Morphea, is a rare autoimmune disease presenting with fibrotic changes in the dermis and subcutis. It is a benign condition associated with significant atrophy and sclerosis leading to disfigurement, flexure contractures, and impaired function. Ultraviolet A1 and photochemotherapy are highly effective treatment options but are not readily available in the country. Narrowband ultraviolet B (NBUVB), on the other hand, is readily available, affordable, and safe to use. @*Case summary@#Three patients diagnosed with different variants of morphea (bilateral generalize morphea, unilateral generalized morphea, and circumscribed morphea). underwent 30 sessions of NBUVB. Treatment response was assessed using tightness and itch Visual Analogue Scale (VAS), Modified Skin Score (MSS), photographic comparison, ultrasonographic measurement, and histopathologic analysis. NBUVB treatment resulted to 14-60% decrease in the tightness and itch VAS. MSS was also reduced by 35-50%. The size, pigmentation, and erythema of the lesions also decreased. Ultrasonography showed an improvement in the thickness of lesions after treatment. Histopathologic study showed less packed collagen with increase in inter-bundle spaces.@*Conclusion@#Response to treatment was influenced by the age of the lesion and anatomical location. More chronic lesions tend to have less response. Lesions on the face exhibited the greatest improvement while lesions on the lower extremities had the least improvement. This is the first case series study in the country that uses NBUVB as treatment for morphea. The improvement of the sclerotic and atrophic lesions treated with narrowband UVB treatment may be an acceptable substitute for UVA1 and PUVA.

A case of adult Filipino male presenting with co-localized Psoriasis and segmental Vitiligo

@#A 29-year-old male presented with a four-year history of recurrent generalized pruritic erythematous scaly annular plaques followed by a solitary depigmented patch on the right hemiabdomen extending to the back with abrupt midline demarcation. Skin biopsies confirmed the rare case of co-existing and colocalized psoriasis and segmental vitiligo. Treatment with topical steroids, tacrolimus, and narrowband ultraviolet B phototherapy resulted to the improvement of the psoriatic lesions but no change in the depigmented patch.

Combination of excimer laser and topical treatment for psoriasis: A systematic review and meta-analysis

@#<p style="text-align: justify;"><strong>OBJECTIVES: </strong>To assess the efficacy and safety of excimer laser in combination with topical standard therapies for treatment of plaque-type psoriasis in comparison to excimer laser alone, standard topical treatment alone, or placebo.</p><p style="text-align: justify;"><strong>METHODS: </strong>A literature search using Medline, Cochrane and HERDIN was conducted. Data were analyzed using mean difference at 95% confidence interval, with heterogeneity determined by I2 test.</p><p style="text-align: justify;"><strong>RESULTS:</strong> Three articles with total of 130 patients fulfilled the inclusion criteria. Topical treatments studied were vitamin analog (calcipotriol), anthralin (dithranol), and steroid (flumethasone pivalate). A subgroup analysis comparing combination therapy and excimer laser alone showed a greater reduction in pooled PASI score reduction (-2.52; 95% CI: -4.28, -0.77) in the combination group after five to six weeks. There was also a significantly greater reduction in cumulative UVB dose (-3.29; 95% CI: -4.29, -2.30) needed for clearing in the combination group. Pigmentation was the commonly observed adverse event in both groups.</p><p style="text-align: justify;"><strong>CONCLUSIONS:</strong> Excimer laser, in combination with topical treatment, is more effective than excimer laser alone, with significantly lower cumulative UVB dose, but the quality of current evidence is low. Long-term controlled trials are warranted to increase our confidence in the estimates of these outcomes.</p>

Knowledge, attitudes and practices concerning sun exposure and sun protection among laypersons and health personnel in a tertiary care hospital in the Philippines

BACKGROUND: This is the first study to assess the level of sun awareness among Filipinos, and the need for a formal campaign to promote sun protection. OBJECTIVE: To assess the knowledge, attitude and practices concerning