Urticaria pigmentosa - Urtikaria Pigmentosa
Urtikaria Pigmentosa (Urticaria pigmentosa) nyaéta bentuk mastositosis kulit anu paling umum. Ieu kasakit langka disababkeun ku kaleuwihan sél mast dina kulit nu ngahasilkeun hives atawa lesi nalika dirangsang. Bintik beureum atawa coklat mindeng katempo dina kulit, ilaharna sabudeureun dada, dahi, jeung tonggong. Sél mast ieu, nalika dirangsang (misalna ku ngagosok kulit, paparan panas), ngahasilkeun teuing histamin, nu nyababkeun réaksi alérgi jeung gatal‑gatal di daérah iritasi, sok disebut “tanda Darier”.
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ReferencesMastocytosis 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 lesi, ilaharna mimitian ti leuwih ti 10 nepi ka kirang ti 100. Tipe panungtungan nembongkeun involvement nyebar sakuliah kulit. Urticaria pigmentosa mangrupikeun bentuk mastocytosis kulit anu paling umum di murangkalih, tapi ogé tiasa lumangsung dina déwasa. Biasana éta mangrupa kaayaan anu teu bahaya anu sering ningkat nalika rumaja. Béda jeung mastocytosis sawawa, urticaria pigmentosa jarang mangaruhan organ internal. Salah sahiji ciri has urticaria pigmentosa nyaéta kacenderungan ngabalukarkeun bintik‑bintik leutik, gatal, semu beureum‑coklat, atawa semu konéng‑coklat, anu 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 jeung awakna salami genep bulan kapengker. Anjeunna nyebatkeun yén bintik-bintikna beureum, ngagurilap, jeung gatal nalika diteken. Anjeunna henteu ngalaman flushing, utah, diare, atawa wheezing, sarta sajarah médis pribadi jeung kulawargana henteu masihan petunjuk anu relevan. Saatos pamariksaan, urang mendakan sababaraha bintik poék dina kulit sirah, dahi, raray, jeung beuheung, sarta bintik-bintik poék anu rada terangkat dina dada jeung tonggongna. Digosok sacara lemes, bintik-bintik éta jadi beureum jeung gatal dina 2 menit, tapi gejala 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.
