Iktiosis Harlequin (IH) merupakan kelompok iktiosis kongenital autosomal resesif yang sangat jarang terjadi, dengan prevalensi 1 kasus setiap 300.000 orang. IH memiliki manifestasi klinis tipikal, dilahirkan dengan hiperkeratosis masif yang disebabkan pewarisan gen autosomal resesif mutasi gen adenosine triphosphate (ATP) - binding cassette sub-family A member 12 (ABCA12) yang terlibat dalam sekresi granula lamelar, serta transport seramid dan lipid epidermal. Kemungkinan hidup pasien IH meningkat seiring perbaikan fasilitas perawatan neonatus dan terapi retinoid. Prinsip utama perawatan pasien IH adalah mengupayakan deskuamasi, terapi suportif, dan penanganan kecacatan fisik yang mengancam vitalitas organ. Konseling genetik dan pemeriksaan diagnostik prenatal perlu untuk deteksi dini pada keluarga yang memiliki riwayat IH. Harlequin Ichthyosis (HI) is a rare autosomal recessive congenital ichthysosis that occurs in about 1 in 300.000 people. HI is characterized by typical skin manifestation with massive hyperkeratosis due to adenosine triphosphate (ATP) - binding cassette sub-family A member 12 (ABCA12) gene mutation inheritance. The ABCA12 gene is involved in lamellar granule secretion, ceramide, and epidermal lipid transport. Survival rate of HI patient increased along with improvement of neonatal care facilities and administration of systemic retinoid. The main principles of care are desquamation, supportive therapy, and treatment for physical disabilities which threatens organ vitality. Genetic counseling and prenatal diagnosis could benefit family with HI history.

Background: Cutaneous amyloidosis is characterized by the deposition of amyloid material in the dermis or epidermis, usually limited to the dermal papillae in local cutaneous amyloidosis. Dermal involvement could lead to skin thickening with a waxy surface. The prevalence of cutaneous amyloidosis is relatively rare, only 0.2 – 0.3 % and is more common in individuals with darker skin phototype. The aim of this study is to report a case of lichen amyloidosis associated with its treatment using a combination of desoxymethasone and salicylic acid.Case Report: We report a 27-year-old woman who came with multiple hyperpigmented papules, which were based on sharp-bordered brown macules with scales and lichenification that felt very itchy on both of her lower legs since 5 years ago. The same lesions also appeared on both her arms 2 years ago. She had skin phototype IV. She denied any history of chronic friction in those areas. On histopathological examination with hematoxylin and eosin (H&E) staining, amorphous deposits were found in the papillae dermis surrounded by melanophages. The patient was treated with topical therapy of 0.25% desoximetasone and 3% salicylic acid and was educated to avoid friction on the lesions. The patient admitted that the itchiness was reduced, but the lesion did not improve.Result: Based on the patient’s age and sex, she belonged to a less common group affected by lichen amyloidosis. Phototype skin IV was the risk factor identified in this case. She denied any history of chronic friction on her skin. Lichen amyloidosis was diagnosed based on a typical lesion found at the predilection sites and was confirmed with histopathological examination with H&E staining. Topical desoximetasone and salicylic acid didn’t result in clinical improvement.Conclusion: The diagnosis of lichen amyloidosis was confirmed with histopathological examination. H&E staining could demonstrate amyloid deposit clearly. Treatment of cutaneous amyloidosis is generally unsatisfactory and may require a combination of therapeutic modalities.  Latar Belakang: Amiloidosis kutaneus merupakan deposisi material amiloid pada lapisan dermis atau epidermis, umumnya terbatas hingga papilla dermis pada kasus amiloidosis kutaneus lokal. Keterlibatan dermis dapat menyebabkan penebalan kulit dengan permukaan seperti lilin. Prevalensi amiloidosis kutaneus relatif jarang yaitu hanya 0,2-0,3 % dan lebih sering terjadi pada individu dengan fototipe kulit lebih gelap. Tujuan penelitian ini untuk melaporkan kasus liken amiloidosis terkait dalam penatalaksanaannya menggunakan kombinasi desoksimetason dan asam salisilat.  Laporan Kasus: Dilaporkan pasien perempuan berusia 27 tahun memiliki papul multipel dengan hiperpigmentasi, dasar kulit makula kecoklatan berbatas tegas disertai skuama dan likenifikasi yang terasa sangat gatal pada area kedua tungkai bawah sejak 5 tahun yang lalu. Lesi yang sama juga muncul pada kedua lengan sejak 2 tahun yang lalu. Pasien memiliki fototipe kulit IV. Pasien menyangkal riwayat gesekan kronis pada area tersebut. Pada pemeriksaan histopatologis dengan pewarnaan hematoxylin dan eosin (H&E), didapatkan deposit amorf di dalam papilla dermis dikelilingi melanofag. Pasien mendapatkan terapi krim topikal desoksimetason 0,25 % dan asam salisilat 3 %, serta diedukasi untuk menghindari gesekan pada lesi. Pasien mengaku gatal berkurang, namun tidak didapatkan perbaikan pada lesi.Hasil: Berdasarkan usia dan jenis kelamin, pasien termasuk kelompok yang jarang terjadi liken amiloidosis. Kulit pasien tergolong dalam fototipe IV merupakan faktor risiko pada pasien. Pasien menyangkal adanya riwayat gesekan kronis. Diagnosis liken amyloidosis didasarkan lesi yang khas dengan lokasi sesuai predileksi dan diagnosis ditegakkan melalui pemeriksaan histopatologis dengan pewarnaan H&E. Terapi desoksimetason dan asam salisilat topikal tidak menunjukkan perbaikan secara klinis.Kesimpulan: Pewarnaan H&E dapat mendemonstrasikan deposit amiloid dengan baik. Terapi amiloidosis kutaneus umumnya tidak memuaskan dan mungkin membutuhkan kombinasi berbagai modalitas terapi.

Syphilis is a systemic infection caused by Treponema pallidum subspecies pallidum, which has a major and various manifestations on the skin (the great imitator). Its prevalence is frequently associated with Human Immunodeficiency Virus (HIV) prevalence. Atypical and aggressive presentation of syphilis is more commonly found in persons with syphilis and coinfection of HIV. We report a case of a 30-yearold male with erythematous plaques and desquamation on his palms and soles with neither pain nor pruritus, and also Beau's line on the hand and toe nails. HIV infection was detected. The diagnosis of the patient was relapse late latent syphilis with HIV co-infection. The diagnosis of syphilis was established based on clinical and serological testing. The clinical manifestations of syphilis in immunosuppressed patients are often atypical. In order to treat, the patient was prescribed with doxycycline 100 mg two times a day for a month and showed clinical improvement after three weeks course of medication. Serologic testing interpretation and treatment do not differ between syphilis patients with and without HIV coinfection.

Background:Vitiligo is an autoimmune disease that causes progressive skin melanocyte destruction, clinically seen as asymptomatic depigmentation macule and poliosis. CD8+ T cell is the primary effector of melanocyte destruction, and the activities of CD8+ T cell are driven by interferon-γ (IFN-γ). The current vitiligo treatment options are often not satisfied with many limitations and a high recurrence rate. Janus Kinase (JAK) inhibitor is one of the emerging vitiligo therapies which has a more specific target with a direct effect on CD8+ T cell cytotoxicity and IFN-γ. Methods: In writing this article, the literature review method was used, and sources consist of relevant Latar belakang: Vitiligo merupakan penyakit autoimun yang menyebabkan destruksi melanosit secara progresif pada kulit, menimbulkan makula depigmentasi asimtomatik dan poliosis. Sel T CD8+ merupakan efektor primer yang menyebabkan kematian melanosit dan aktivitasnya ditingkatkan oleh interferon-γ (IFN-γ). Pilihan terapi vitiligo saat ini seringkali tidak memuaskan, memiliki keterbatasan, dan memiliki tingkat kekambuhan yang tinggi. Inhibitor Janus Kinase (JAK) merupakan salah satu terapi baru vitiligo yang memiliki target lebih spesifik dengan efek langsung terhadap sitotoksisitas sel T CD8+ dan IFN-γ. Metode: Dalam penulisan artikel ini digunakan metode tinjauan pustaka dengan bersumber pada journals obtained through online search engines. Result: Several studies show that repigmentation in the facial area increases between 51-92%. Repigmentation occurs better in areas exposed to sunlight or a combination of phototherapy because repigmentation in vitiligo lesions requires suppression of the autoimmune process and melanocyte regeneration. Conclusion:Many studies showed the results of vitiligo therapy using JAK inhibitors were very promising and safe. The vitiligo therapy using JAK inhibitor showed higher efficacy on the facial area and combined with ultraviolet exposure. artikel relevan yang didapatkan melalui pencarian secara daring. Hasil: Beberapa studi menunjukkan adanya repigmentasi pada area wajah meningkat antara 51-92%. Repigmentasi terjadi lebih baik pada area terpapar sinar matahari atau kombinasi fototerapi disebabkan karena repigmentasi pada lesi vitiligo membutuhkan supresi dari proses autoimun dan regenerasi melanosit. Simpulan: Berbagai studi menunjukkan hasil terapi inhibitor JAK terhadap vitiligo sangat menjanjikan dan cukup aman. Terapi inhibitor JAK menunjukkan efektivitas lebih tinggi pada area wajah dan dengan kombinasi paparan ultraviolet.

<p>Iktiosis Harlequin (IH) merupakan kelompok iktiosis kongenital autosomal resesif yang sangat jarang terjadi, dengan prevalensi 1 kasus setiap 300.000 orang. IH memiliki manifestasi klinis tipikal, dilahirkan dengan hiperkeratosis masif yang disebabkan pewarisan gen autosomal resesif mutasi gen adenosine triphosphate (ATP) - binding cassette sub-family A member 12 (ABCA12) yang terlibat dalam sekresi granula lamelar, serta transport seramid dan lipid epidermal. Kemungkinan hidup pasien IH meningkat seiring perbaikan fasilitas perawatan neonatus dan terapi retinoid. Prinsip utama perawatan pasien IH adalah mengupayakan deskuamasi, terapi suportif, dan penanganan kecacatan fisik yang mengancam vitalitas organ. Konseling genetik dan pemeriksaan diagnostik prenatal perlu untuk deteksi dini pada keluarga yang memiliki riwayat IH.</p><p>Harlequin Ichthyosis (HI) is a rare autosomal recessive congenital ichthysosis that occurs in about 1 in 300.000 people. HI is characterized by typical skin manifestation with massive hyperkeratosis due to adenosine triphosphate (ATP) - binding cassette sub-family A member 12 (ABCA12) gene mutation inheritance. The ABCA12 gene is involved in lamellar granule secretion, ceramide, and epidermal lipid transport. Survival rate of HI patient increased along with improvement of neonatal care facilities and administration of systemic retinoid. The main principles of care are desquamation, supportive therapy, and treatment for physical disabilities which threatens organ vitality. Genetic counseling and prenatal diagnosis could benefit family with HI history.</p><p> </p>