Effectiveness of Pulsed Dye Laser Treatment in Tufted Angioma: A Promising Intervention.

Introduction: Tufted angioma (TA) is a rare benign vascular tumor usually occurring in the first year of life. It may present as reddish papules or purplish red to black plaques. Tender lesions commonly affect the neck, upper trunk, and shoulders. Histologic tufts of capillaries infiltrating the dermis in a "cannon ball" distribution pattern confirm the diagnosis. However, effective treatments for TA are scarce. Pulsed dye laser (PDL) is an alternative therapy for TA, particularly concerning cosmetics and pain relief. This case report demonstrates the effectiveness of PDL as a treatment for TA. Case: We report a 15-year-old girl with five-year history of painful purplish red plaques on her left cheek, neck, chest, left shoulder, and back. Histopathological examination from skin biopsy showed discrete "cannon ball" pattern in the dermis. Laboratory examination revealed normal platelet count and fibrinogen level. Based on these presentations, the diagnosis of TA without complications was made. We treated the lesion using 595-nm PDL with 6.5-10 J/cm2 fluence of, 1.5 ms pulsed duration, and 5-7 mm spot size every three weeks. Reductions in redness and pain were seen after four sessions of treatment. Discussion: PDL promotes selective vascular damage with minimal injury to the surrounding skin. Capillary tufts in TA could therefore serve as a target for laser treatment, which may result in fading redness and pain reduction. Conclusion: PDL is effective in reducing redness and pain in TA.

A Retrospective Study on the Clinical, Laboratory, and Nutritional Status of Pediatric Epidermolysis Bullosa in a Tertiary Referral Hospital in West Java, Indonesia.

Background: Epidermolysis bullosa (EB) is a genodermatosis disease with bullae and erosions of the skin and mucous membrane that can last for a lifetime and decrease quality of life. Oral and gastrointestinal disorders inhibit the patients' ability to achieve optimal nutrition, making the patients prone to infection, leading to prolonged wound healing, and delayed growth and developmental process. However, there has been no research on the clinical, laboratory, and nutritional status of pediatric EB patients in Indonesia. Purpose: This study aims to describe the clinical, laboratory, and nutritional characteristics of pediatric EB patients treated in Dr. Hasan Sadikin General Hospital Bandung, Indonesia. Patients and Methods: This was a retrospective descriptive study of pediatric EB patient records in Dermatology and Venereology Outpatient of Dr. Hasan Sadikin General Hospital Bandung, Indonesia, from April 2018-March 2020. Results: Study results showed 12 pediatric EB patients consisting of 7 dystrophic EB (DEB) (4 recessive dystrophic EB [RDEB] patients and 3 dominant dystrophic EB [DDEB]), 3 junctional EB (JEB), and 2 EB simplex (EBS). The most extensive EB wounds was found affecting 10-20% of the body surface area with a <10% infected wound area. Pain was found in all patients. The most frequent abnormalities in laboratory examination were anemia and low zinc levels. Severe malnutrition was found in almost half of the patients. Conclusion: RDEB is the most commonly found type of pediatric EB. Wounds on the skin, tooth decay, hand deformity, pain when changing dressings, low zinc levels, and low hemoglobin levels are the clinical features and laboratory findings that contribute to the development of moderate and severe malnutrition in RDEB patients.

Successful Treatment of Non-Langerhans Cell Histiocytosis With Topical Rapamycin in Two Pediatric Cases.

Non-Langerhans cell histiocytosis (non-LCH) is a group of diseases characterized by the proliferation of histiocytes in tissues that is excluded from the diagnostic criteria for LCH. Juvenile xanthogranuloma (JXG) and benign cephalic histiocytosis (BCH) are the most common types of cutaneous non-LCH. These two diseases share similarities in both clinical and histological features, therefore, they can be difficult to differentiate. Thorough physical, dermoscopic, and histopathological examinations are required to distinguish between JXG and BCH. We hereby present two rare cases of non-LCH in pediatric patients, presented with JXG and BCH. The dermoscopic examination of both cases showed a setting-sun appearance, while the histopathological examination revealed Touton giant cells in the JXG case, and massive lymphocyte infiltration in the BCH case. Both patients were treated with 1% topical rapamycin in a split-side comparison for the first 12 weeks, followed by applications on both sides for a total duration of 24 weeks. As a result, there was a significant reduction in the size of the lesion, leading to patient's satisfaction. Rapamycin is an immunosuppressive agent with antineoplastic activity. Rapamycin can be used as an alternative non-invasive topical treatment option for JXG and BCH. However, long-term observations are required to assess its effectiveness and side effects.

Melatonin and Sleep Disturbances in Children With Atopic Dermatitis.

Objective: To evaluate the correlation between melatonin levels and sleep quality, based on the Children's Sleep Habit Questionnaire (CSHQ), among children with atopic dermatitis (AD). Methods: This was an analytic study with a cross-sectional design involving two groups, AD children as the case group and participants without AD as the control group, conducted at the Pediatric Dermatology Clinic of Dr. Hasan Sadikin Hospital, Bandung, Indonesia. Melatonin levels and CSHQ score in the case group were compared to that in the control group. Correlation analysis was performed between melatonin levels and sleep quality based on CSHQ in the case group. Results: Participants in this study consisted of 19 children with moderate AD, (no patient with mild and severe AD was recruited), and 19 participants without AD. The mean CSHQ score in the case group was significantly higher than the control group (47.84 vs 36.79; p<0.05). The mean melatonin level in the case group was significantly lower than the control group (320.18 pg/mL vs 383.86 pg/mL; p<0.05). Comparative analysis was conducted using independent t-test. There was a significant Pearson's correlation index between SCORAD and CSHQ (p<0.05). However, there was no correlation between melatonin levels and CSHQ, as well as between melatonin levels and SCORAD (p<0.05). Conclusion: Children with moderate AD have impaired sleep quality and lower melatonin levels compared to controls. No correlation was found between melatonin levels and CSHQ, as well as melatonin levels and SCORAD, although there was a strong and significant correlation between SCORAD and CSHQ. Therefore, melatonin levels may not be the main cause of sleep disturbances in children with moderate AD.

Efficacy of Triamcinolone Acetonide Injection in a Case of Giant Spiradenoma.

Spiradenoma is a rare benign adnexal tumor with eccrine differentiation. The clinical manifestations include painful, skin-colored, red, gray, or bluish nodules on the upper half of the body. We report a case of spiradenoma in a 31-year-old man. The diagnosis was established from the patient's history, physical examination, and histopathological examination. In this case, the patient was treated with intralesional injection of triamcinolone acetonide (TA) 10 mg/mL. After the fourth injection, the lesions grew smaller and thinner. TA injection is easy to administer and showed good efficacy in spiradenoma case, although further research with a larger number of patients is needed.

Retinal Neovascularization in Two Patients with Incontinentia Pigmenti.

Incontinentia pigmenti (IP) is a rare genodermatosis, inherited in an X-linked dominant pattern, making it generally found among women. Among several characteristics of IP are four phases of skin manifestation that tend to follow Blaschko's lines, in addition to abnormalities of the eye, central nervous system (CNS), and teeth. Ocular involvement in IP patients can occur since birth, which can be classified into retinal or non-retinal disorders. Retinal disorders can result in detachment, which is a major ocular threat for IP patients. This article reports two IP cases with overlapped phases of skin disorders in baby girls with ocular manifestations since early life. Clinical signs and additional examination of the skin and eyes are utilized to make the diagnosis. All the features of the histopathological examination supported the diagnosis of IP, and ocular exams revealed abnormalities in the form of retinal neovascularization (RN). Although RN may resolve spontaneously, patients should be monitored for the development of other eye disorders such as visual impairment.

Xeroderma Pigmentosum with Simultaneous Cutaneous and Ocular Squamous Cell Carcinoma.

Xeroderma pigmentosum (XP) is a rare autosomal recessive disease that disrupts deoxyribonucleic acid (DNA) repair due to ultraviolet (UV) radiation. XP is characterized by extreme sensitivity to sunlight, photophobia, cutaneous lesions in the form of freckle-like hyperpigmented macules, and neoplasia on the skin surface. Malignancy is a common complication found in areas exposed to UV light. Squamous cell carcinomas (SCC) is the most common malignancy seen in patients with XP. This report illustrates a case of XP in a six-year-old girl with cutaneous and ocular SCC. The diagnosis of XP was established based on the patient's history and the presence of typical clinical manifestations. Dermoscopy and histopathology examinations confirmed the presence of SCC on the face and eyes. The management of XP patients includes early diagnosis, lifelong UV protection, and early detection of cutaneous malignancy. Early detection and appropriate management are very important in preventing the occurrence of malignancy.

Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case.

Aplasia cutis congenita (ACC) is a congenital disorder characterized by the absence of epidermis, dermis, and sometimes subcutaneous tissue and bone. There are nine types of ACC based on the number and location of the lesions as well as the presence or absence of associated deformities, with type I ACC being the most common. Type V affects the trunk with a characteristic pattern resembling the "H" letter of the alphabet, generally accompanied by fetus papyraceus (death of one of the twin fetuses) or placental infarction. Type V ACC without fetus papyraceus and placental infarction is a rare case. We reported a case of type V ACC in a 3-day-old baby girl, with clinical manifestation of extensive ulcers on the scalp, back, buttocks, and both lower extremities, in an "H"-shaped pattern, with history of placenta accreta, but no fetus papyraceus or placental infarction was found. The patient received dialkyl carbamoyl chloride hydrophobic swab with hydrogel (Sorbact® gel dressing-BSN Medical) and hydrocolloid wound dressing with good response, as indicated by the wounds becoming dried up, leaving an atrophic scar. Complete epithelialization was achieved in 72 days. The cause of type V ACC remains not completely understood. The diagnosis of ACC can be established based on clinical manifestations. Further examination is required if extracutaneous involvement is suspected. Early identification of the etiology of the lesions and appropriate investigation and treatment, including conservative wound management, are needed so that aplastic lesions can heal successfully, although cosmetic repair may be required at a later stage.

Self-improving dystrophic epidermolysis bullosa: First report of clinical, molecular, and genetic characterization of five patients from Southeast Asia.

Self-improving dystrophic epidermolysis bullosa is a rare subtype of dystrophic epidermolysis bullosa (DEB) characterized by significant improvement in skin fragility within the first few years of life. Genetic inheritance has previously been reported as autosomal dominant or recessive with both forms harboring mutations in COL7A1. To date, there have been no reports of this rare clinical entity from various Southeast Asian ethnicities. Here, we describe the clinical and molecular features of five patients from the Southeast Asia region who presented with predominantly acral-distributed blisters and erosions in the first few days of life. Blistering resolved over several months, without appearance of new blisters. By immunofluorescence, intraepidermal retention of Type VII collagen was observed in all patient skin biopsies when investigated with antibody staining. Genetic analysis of four patients revealed pathogenic variants in COL7A1 which have not been previously reported. The clinical diagnosis in these rare patients is confirmed with molecular histology and genetic characterization.

Schimmelpenning Syndrome with Large Nevus Sebaceous and Multiple Epidermal Nevi.

Epidermal nevus syndromes are a group of rare multiorgan disorders. Schimmelpenning syndrome is a clinical variant of epidermal nevus syndromes. This congenital syndrome is characterized by the existence of nevus sebaceous and usually accompanied by abnormalities of the eyes, skeletal, cardiovascular, and central nervous systems. A 2-year-old girl presented with large, hairless, yellowish-brown plaques on the scalp and face along with multiple brownish-black verrucose plaques and brownish-black macules on almost all parts of the body. The skin-colored verrucose tumors were also found on the lips and around the mouth. Histopathological examination of the lesion on the forehead revealed hyperkeratosis, acanthosis, and sebaceous gland hyperplasia supporting the diagnosis of nevus sebaceous, while histopathological examination of the lesions on the lips and abdomen demonstrated hyperkeratosis, acanthosis, and papillomatosis consistent with verrucous epidermal nevus. The pediatrician suspected that the patient had mental retardation; however, there were no neurological, cardiac, skeletal, nor ophthalmologic abnormalities. The lesions on the lips and around the mouth were excised, and it demonstrated a good result. To conclude, epidermal nevus syndrome (e.g., Schimmelpenning syndrome) should be considered in children born with nevus sebaceous.

An Unusual and Rare Case of Generalized Multiple Epidermoid Cysts with a Giant Epidermoid Cyst.

Epidermoid cyst (EC) is a cyst containing keratin and its breakdown products, that is surrounded by an epidermoid wall. EC can occur in both men and women, especially in young- and middle-aged adults. The clinical features of EC include asymptomatic, well-demarcated, and skin-colored to yellowish subcutaneous or dermal nodules with a punctum on the middle of the lesion which can occur anywhere on the body. This type of cyst is usually solitary with diameter up to 5 cm, or rarely, there are multiple with diameters of more than 5 cm, which is classified as a giant EC. Here, we report a case of generalized multiple ECs with a giant EC on the left gluteal region of a 19-year-old male with complaints of asymptomatic skin-colored nodules with central punctum on the right temple, chest, back, and gluteal regions for five years. Correlation between the clinical and histopathological findings in the form of cysts containing keratin and lined by wall composed of stratified squamous epithelia established the diagnosis of EC. The patient underwent surgical procedures for the skin-colored nodules on the chest, back, and gluteal regions. He also received triamcinolone acetonide intralesional injection for the skin-colored nodule on the right temple. After four months of follow-up, there was no recurrence, and all the skin-colored nodules were completely removed. EC should always be considered when diagnosing a skin-colored nodule even if the manifestations are generalized multiple and giant nodules, as in our case, demonstrating a rare and unusual case of EC, to the best of our knowledge.

Clinical efficacy of biocellulose, carboxymethyl cellulose and normal saline dressing in epidermolysis bullosa.

OBJECTIVE: To evaluate the efficacy of a biocellulose, a carboxymethyl cellulose and a normal saline wound dressing in the wound care management of epidermolysis bullosa (EB) skin wounds. METHODS: This was a single-blind, randomised controlled trial involving wounds from patients with EB. Wounds were divided into three groups: group I with biocellulose wound dressing, group II with carboxymethyl cellulose wound dressing and group III with normal saline wound dressing as a control. All dressing changes and wound parameters were recorded. Observations were conducted every three days until complete wound closure or up to one month. RESULTS: The outcomes of treatment of 36 wounds from four patients were evaluated in this study. Mean healing time in group I was seven days, eight days in group II and 14 days in group III. There were significant differences in healing times between group I and group III (p=0.0001) and between group II and III (p=0.001). The results showed a significant reduction in the percentage of wounds area on day three for each group: 51.7% in group I, 51.9% in group II, and 26% for group III. All wounds in groups I and II had healed at day 12 (100%) and at day 24 (100%) in group III. There were significant differences in the reduction of percentage wound area between group I and group III at day three (p=0.044) and day six (p=0.000), and between group II and III at day six (p=0.003). CONCLUSION: The study demonstrates that both the biocellulose and the carboxymethyl cellulose wound dressings significantly reduced percentage wound areas and complete healing times compared with the normal saline wound dressing in EB skin wounds, demonstrating they are both equally good for wound care management in EB patients.

Multifocal Osteomyelitic Tuberculosis at Rare Locations with Metastatic Tuberculosis Abscess.

BACKGROUND Multifocal tuberculosis (TB) with more than 1 tuberculous osteoarticular lesion is rare. Furthermore, metastatic tuberculous abscess (MTA) is also a very rare manifestation of cutaneous TB in children. A non-specific, often subtle, early clinical presentation in conjunction with a low prevalence rate constitute obstacles for diagnosis. CASE REPORT A 9-years old female patient was referred to Hasan Sadikin Pediatric Respirology Outpatient clinic from the Orthopedic Department with lumps at the left elbow joint, lateral side of the left hand, and lateral side of the left foot. Fine needle aspiration biopsy of the lumps suggested a chronic inflammation due to TB. The patient was then started with a course of anti-TB drugs consisting of rifampicin, isoniazid, pyrazinamide, and ethambutol. During the treatment course, she experienced anti-TB drug-induced hepatotoxicity (ADIH). We then switched the regimen to streptomycin and ethambutol for 2 weeks, then reintroduced treatment with the modified British Thoracic Society guidelines regimen. The nodules appeared shrunken after 3 months of treatment with anti-TB drugs. CONCLUSIONS Increased awareness of unusual manifestations of TB will likely allow for proper diagnosis and management of this common infection. Accordingly, timely diagnosis and management will prevent further debilitating sequelae.

A clinician's reference guide for the management of atopic dermatitis in Asians.

BACKGROUND: Atopic dermatitis (AD) is a common skin condition among Asians. Recent studies have shown that Asian AD has a unique clinical and immunologic phenotype compared with European/American AD. OBJECTIVE: The Asian Academy of Dermatology and Venereology Expert Panel on Atopic Dermatitis developed this reference guide to provide a holistic and evidence-based approach in managing AD among Asians. METHODS: Electronic searches were performed to retrieve relevant systematic reviews and guidelines on AD. Recommendations were appraised for level of evidence and strength of recommendation based on the U.K. National Institute for Health and Care Excellence and Scottish Intercollegiate Guidelines Network guidelines. These practice points were based on the consensus recommendations discussed during the Asia Pacific Meeting of Experts in Dermatology held in Bali, Indonesia in October 2016 and April 2017. RESULTS: The Expert Panel recommends an approach to treatment based on disease severity. The use of moisturizers is recommended across all levels of AD severity, while topical steroids are recommended only for flares not controlled by conventional skin care and moisturizers. Causes of waning efficacy must be explored before using topical corticosteroids of higher potency. Topical calcineurin inhibitors are recommended for patients who have become recalcitrant to steroid, in chronic uninterrupted use, and when there is steroid atrophy, or when there is a need to treat sensitive areas and pediatric patients. Systemic steroids have a limited role in AD treatment and should be avoided if possible. Educational programs that allow a patient-centered approach in AD management are recommended as an adjunct to conventional therapies. Recommendations on the use of phototherapy, systemic drugs, and emerging treatments are also included. CONCLUSION: The management of AD among Asians requires a holistic approach, integrating evidence-based treatments while considering accessibility and cultural acceptability.

A review on the role of moisturizers for atopic dermatitis.

Effective management of atopic dermatitis (AD) involves the treatment of a defective skin barrier. Patients with AD are therefore advised to use moisturizers regularly. To date, there are few comparative studies involving moisturizers in patients with AD, and no classification system exists to objectively determine which types of moisturizers are best suited to specific AD phenotypes. With this in mind, a group of experts from allergy and immunology, adult and pediatric dermatology, and pediatrics centers within Southeast Asia met to review current data and practice, and to develop recommendations regarding the use of moisturizers in patients with AD within the Asia-Pacific region. Chronicity and severity of AD, along with patient age, treatment compliance, and economic background should all be taken into account when selecting an appropriate moisturizer for AD patients. Other considerations include adjuvant properties of the product, cosmetic acceptability, and availability over the counter. Well-defined clinical phenotypes of AD could optimally benefit from specific moisturizers. It is hoped that future studies may identify such differences by means of filaggrin mutation subtypes, confocal microscopic evaluation, pH, transepidermal water loss or presence of allergy specific IgE. Recommendations to improve the regular use of moisturizers among AD patients include measures that focus on treatment compliance, patient and caregiver education, appropriate treatment goals, avoidance of sensitizing agents, and collaboration with other relevant specialists.