Urticaria pigmentosa - Urtikaria Pigmentosa
Urtikaria Pigmentosa (Urticaria pigmentosa) adalah bentuk mastositosis kulit yang paling umum. Ini adalah penyakit langka yang disebabkan oleh jumlah sel mast yang berlebihan di kulit yang menimbulkan gatal-gatal atau lesi pada kulit saat teriritasi. Bintik merah atau coklat sering terlihat pada kulit, biasanya di sekitar dada, dahi, dan punggung. Sel mast ini, ketika teriritasi (misalnya dengan menggosok kulit, paparan panas), menghasilkan terlalu banyak histamin, memicu reaksi alergi yang menyebabkan gatal-gatal di area iritasi, kadang-kadang disebut sebagai "tanda Darier".
☆ Pada hasil Stiftung Warentest tahun 2022 dari Jerman, kepuasan konsumen terhadap ModelDerm hanya sedikit lebih rendah dibandingkan dengan konsultasi telemedis berbayar.
Cenderung terjadi pada batang tubuh anak kecil.
References
Mastocytosis adalah suatu kondisi dimana terdapat kelebihan sel mast, 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) . Tipe kedua (urticaria pigmentosa) melibatkan banyak lesi, biasanya berkisar antara lebih dari 10 hingga kurang dari 100. Tipe terakhir menunjukkan keterlibatan luas di seluruh kulit. Urticaria pigmentosa adalah bentuk mastositosis kulit yang paling umum terjadi pada anak-anak, namun juga dapat terjadi pada orang dewasa. Ini biasanya merupakan kondisi yang tidak berbahaya dan sering membaik selama masa remaja. Berbeda dengan mastositosis pada orang dewasa, urticaria pigmentosa jarang menyerang organ dalam. Salah satu ciri khas urticaria pigmentosa adalah kecenderungannya menimbulkan bercak atau lesi kecil, gatal, coklat kemerahan, atau coklat kekuningan pada kulit, yang biasa disebut gatal-gatal. Bintik-bintik ini biasanya muncul pada masa kanak-kanak dan bisa 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 dan kemudian menyebar ke wajah dan tubuhnya selama enam bulan terakhir. Dia menyebutkan perasaannya naik, memerah, dan gatal ketika ditekan. Dia tidak mengalami kemerahan, muntah, diare, atau mengi, dan riwayat kesehatan pribadi dan keluarganya tidak memberikan petunjuk yang relevan. Setelah pemeriksaan, kami menemukan beberapa bintik hitam di kulit kepala, dahi, wajah, dan lehernya, serta bercak hitam sedikit menonjol di dada dan punggungnya. Menggosok titik tersebut dengan ringan akan menyebabkan bengkak dan gatal dalam waktu 2 menit, namun gejalanya hilang dalam waktu 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.
