Urticaria pigmentosa - Urtikaria Pigmentosa
Urtikaria Pigmentosa (Urticaria pigmentosa) ialah bentuk mastositosis kulit yang paling biasa. Ia merupakan penyakit yang jarang berlaku, disebabkan oleh bilangan sel mast yang berlebihan dalam kulit, yang menghasilkan sarang atau luka apabila teriritasi. Tompok merah atau coklat sering kelihatan pada kulit, biasanya di sekitar dada, dahi, dan belakang. Sel mast ini, apabila teriritasi (contohnya dengan menggosok kulit atau pendedahan haba), menghasilkan terlalu banyak histamin, mencetuskan tindak balas alahan yang menyebabkan gatal‑gatal pada kawasan yang teriritasi, kadangkala dirujuk sebagai “tanda Darier”.
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Ia cenderung berlaku pada batang tubuh kanak-kanak kecil.
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ReferencesMastocytosis ialah keadaan di mana terdapat lebihan sel mast, sering ditemui di pelbagai bahagian badan seperti kulit, sumsum tulang dan sistem pencernaan. Menurut Pertubuhan Kesihatan Sedunia (WHO), cutaneous mastocytosis boleh dikategorikan kepada tiga jenis utama. Jenis pertama (mastocytomas) terdiri daripada lesi tunggal atau beberapa (≤ 3). Jenis kedua (urticaria pigmentosa) melibatkan pelbagai lesi, biasanya antara lebih daripada 10 hingga kurang daripada 100. Jenis ketiga menunjukkan penglibatan yang meluas di seluruh kulit. Urticaria pigmentosa ialah bentuk mastocytosis kulit yang paling biasa pada kanak‑kanak, tetapi ia juga boleh berlaku pada orang dewasa. Ia biasanya merupakan keadaan yang tidak berbahaya dan sering bertambah baik semasa remaja. Tidak seperti mastocytosis dewasa, urticaria pigmentosa jarang menjejaskan organ dalaman. Satu ciri khas urticaria pigmentosa ialah kecenderungan untuk menghasilkan bintik‑bintik kecil, gatal, berwarna coklat kemerahan atau coklat kekuningan, atau luka pada kulit, yang biasanya dikenali sebagai sarang. Tompok‑tompok ini biasanya muncul pada zaman kanak‑kanak dan boleh bertahan sepanjang hayat.
Mastocytosis is a disorder characterized by mast cell accumulation, commonly in the skin, bone marrow, gastrointestinal (GI) tract, liver, spleen, and lymphatic tissues. The World Health Organization (WHO) divides cutaneous mastocytosis into 3 main presentations. The first has solitary or few (≤3) lesions called mastocytomas. The second, urticaria pigmentosa (UP), involves multiple lesions ranging from >10 to <100 lesions. The last presentation involves diffuse cutaneous involvement. UP is the most common cutaneous mastocytosis in children, but it can form in adults as well. It is considered a benign, self-resolving condition that often remits in adolescence. Unlike adult forms of mastocytosis, there is rarely any internal organ involvement in UP. What makes UP particularly distinctive is its tendency to manifest as small, itchy, reddish-brown, or yellowish-brown spots or lesions on the skin, commonly referred to as urticaria or hives. These spots typically appear in childhood and can persist throughout a person's life.
Mastocytosis is a disorder characterized by mast cell accumulation, commonly in the skin, bone marrow, gastrointestinal (GI) tract, liver, spleen, and lymphatic tissues. The World Health Organization (WHO) divides cutaneous mastocytosis into 3 main presentations. The first has solitary or few (≤3) lesions called mastocytomas. The second, urticaria pigmentosa (UP), involves multiple lesions ranging from >10 to <100 lesions. The last presentation involves diffuse cutaneous involvement. UP is the most common cutaneous mastocytosis in children, but it can form in adults as well. It is considered a benign, self-resolving condition that often remits in adolescence. Unlike adult forms of mastocytosis, there is rarely any internal organ involvement in UP. What makes UP particularly distinctive is its tendency to manifest as small, itchy, reddish-brown, or yellowish-brown spots or lesions on the skin, commonly referred to as urticaria or hives. These spots typically appear in childhood and can persist throughout a person's life.
Seorang kanak‑kanak perempuan berusia 6 tahun hadir dengan beberapa bintik berwarna gelap yang mula‑mula muncul di kulit kepala dan kemudian merebak ke muka serta badan sejak enam bulan yang lalu. Dia melaporkan bahawa bintik‑bintik tersebut naik, menjadi merah, dan gatal apabila tekanan dikenakan. Dia tidak mengalami pembilasan, muntah, cirit‑birit atau berdehit, dan sejarah perubatan peribadi serta keluarga tidak memberikan petunjuk yang berkaitan. Selepas pemeriksaan, kami mendapati banyak bintik hitam pada kulit kepala, dahi, muka, dan leher, serta tompokan gelap yang sedikit terangkat di dada dan belakang. Menggosok sedikit bintik menyebabkan ia membengkak dan gatal dalam masa 2 minit, tetapi gejala pudar dalam masa 15–20 minit (Darier's sign).
A 6-year-old female, presented with multiple dark-colored lesions, which started over the scalp and further progressed to involve the face and trunk since past six months. She gave a history of elevation, redness, and itching on the lesions on application of pressure. There was no associated flushing, vomiting, diarrhoea, or wheeze. The personal and family history was not contributory. On examination, there were multiple hyperpigmented macules over the scalp, forehead, face, and neck in addition to minimally elevated hyperpigmented plaques over the chest and the back. Gentle rubbing of the lesions elicited urtication and itching within 2 min and it resolved within 15–20 minutes, suggestive of the Darier's sign.
A 6-year-old female, presented with multiple dark-colored lesions, which started over the scalp and further progressed to involve the face and trunk since past six months. She gave a history of elevation, redness, and itching on the lesions on application of pressure. There was no associated flushing, vomiting, diarrhoea, or wheeze. The personal and family history was not contributory. On examination, there were multiple hyperpigmented macules over the scalp, forehead, face, and neck in addition to minimally elevated hyperpigmented plaques over the chest and the back. Gentle rubbing of the lesions elicited urtication and itching within 2 min and it resolved within 15–20 minutes, suggestive of the Darier's sign.
