Urticaria pigmentosa - Urtikaria Pigmentosa
Urtikaria Pigmentosa (Urticaria pigmentosa) adalah bentuk mastositosis kulit yang paling umum. Penyakit ini langka dan disebabkan oleh kelebihan sel mast di kulit, yang menimbulkan gatal atau lesi ketika teriritasi. Bintik‑bintik merah atau coklat biasanya terlihat di daerah dada, dahi, dan punggung. Sel mast yang teriritasi (misalnya akibat menggosok kulit atau paparan panas) melepaskan terlalu banyak histamin, memicu reaksi alergi yang menyebabkan gatal pada area tersebut, kadang‑kadang disebut “tanda Darier”.
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Umumnya terjadi pada batang tubuh anak kecil.
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ReferencesMastocytosis adalah suatu kondisi di mana terdapat kelebihan sel mast, yang sering ditemukan di berbagai bagian tubuh seperti kulit, sumsum tulang, dan sistem pencernaan. Menurut Organisasi Kesehatan Dunia (WHO), cutaneous mastocytosis dapat dikategorikan menjadi tiga jenis utama. Tipe pertama (mastocytomas) terdiri dari lesi soliter atau beberapa (≤3) lesi. Tipe kedua (urticaria pigmentosa) melibatkan banyak lesi, biasanya berkisar antara lebih dari 10 hingga kurang dari 100 lesi. Tipe terakhir menunjukkan keterlibatan luas di seluruh kulit. Urticaria pigmentosa adalah bentuk mastocytosis kulit yang paling umum terjadi pada anak‑anak, namun juga dapat terjadi pada orang dewasa. Kondisi ini biasanya tidak berbahaya dan sering membaik selama masa remaja. Berbeda dengan mastocytosis pada orang dewasa, urticaria pigmentosa jarang menyerang organ dalam. Salah satu ciri khas urticaria pigmentosa adalah kecenderungannya menimbulkan bercak atau lesi kecil, gatal, berwarna coklat kemerahan, atau coklat kekuningan pada kulit, yang biasa disebut gatal‑gatal. Bintik‑bintik ini biasanya muncul pada masa kanak‑kanak dan dapat bertahan sepanjang hidup.
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 anak perempuan berusia 6 tahun datang dengan beberapa bintik berwarna gelap yang pertama kali muncul di kulit kepalanya, kemudian menyebar ke wajah dan tubuh selama enam bulan terakhir. Ia melaporkan bahwa bintik‑bintik tersebut terasa naik, memerah, dan gatal ketika ditekan. Ia tidak mengalami kemerahan, muntah, diare, atau mengi, dan riwayat kesehatan pribadi serta keluarga tidak memberikan petunjuk yang relevan. Pada pemeriksaan ditemukan beberapa bintik hitam di kulit kepala, dahi, wajah, dan leher, serta bercak hitam sedikit menonjol di dada dan punggung. Menggosok titik tersebut secara ringan menyebabkan pembengkakan dan gatal dalam waktu 2 menit, namun gejalanya menghilang dalam 15–20 menit (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.
