Urticaria pigmentosa - Urtikaria Pigmentosa
Urtikaria Pigmentosa (Urticaria pigmentosa) nyaéta bentuk mastositosis kulit anu paling umum. Ieu kasakit langka disababkeun ku jumlah kaleuleuwihan sél mast dina kulit nu ngahasilkeun hives atawa lesions dina kulit nalika jengkel. Bintik beureum atawa coklat mindeng katempo dina kulit, ilaharna sabudeureun dada, dahi, jeung tonggong. Sél mast ieu, nalika jengkel (misalna ku ngagosok kulit, paparan panas), ngahasilkeun seueur teuing histamin, nyababkeun réaksi alérgi anu nyababkeun gatal-gatal dilokalkeun ka daérah iritasi, sok disebut "tanda Darier".
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References
Mastocytosis nyaéta kaayaan dimana aya kaleuwihan sél mast, mindeng kapanggih dina sagala rupa bagian awak kawas kulit, sungsum tulang, jeung sistem pencernaan. Numutkeun Organisasi Kaséhatan Dunia (WHO) , cutaneous mastocytosis tiasa digolongkeun kana tilu jinis utama. Tipe kahiji (mastocytomas) diwangun ku lesi tunggal atawa sababaraha (≤3) . Tipe kadua (urticaria pigmentosa) ngalibatkeun sababaraha lesions, ilaharna mimitian ti leuwih ti 10 mun kirang ti 100. Tipe panungtungan nembongkeun involvement nyebar sakuliah kulit. Urticaria pigmentosa mangrupikeun bentuk mastositosis kulit anu paling umum di murangkalih, tapi ogé tiasa lumangsung dina déwasa. Biasana mangrupikeun kaayaan anu teu bahaya anu sering ningkat nalika rumaja. Teu kawas mastositosis sawawa, urticaria pigmentosa jarang mangaruhan organ internal. Salah sahiji ciri has tina urticaria pigmentosa nyaéta kacenderunganna ngabalukarkeun bintik-bintik leutik, gatal, semu beureum-coklat, atawa semu konéng-coklat atawa lesi dina kulit, ilaharna katelah hives. Bintik-bintik ieu biasana muncul dina budak leutik sareng tiasa tahan sapanjang hirup.
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.
Budak awéwé umur 6 taun sumping kalayan sababaraha bintik warna poék anu mimiti muncul dina kulit sirahna teras sumebar ka raray sareng awakna salami genep bulan kapengker. Anjeunna nyebatkeun perasaan aranjeunna naék, beureum, sareng gatal nalika tekanan diterapkeun. Anjeunna henteu ngalaman flushing, utah, diare, atanapi wheezing, sareng sajarah médis pribadi sareng kulawargana henteu masihan petunjuk anu relevan. Saatos pamariksaan, urang mendakan sababaraha bintik poék dina kulit sirah, dahi, raray, sareng beuheung, sareng bintik-bintik poék anu rada diangkat dina dada sareng tonggongna. Digosok énténg éta bintik-bintik nyababkeun aranjeunna ngabareuhan sareng gatal dina 2 menit, tapi gejalana luntur dina 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.
