Urticaria pigmentosa - 色素性蕁麻疹
色素性蕁麻疹 (Urticaria pigmentosa) 是皮膚肥大細胞增多症最常見的表現形式。此罕見疾病是因皮膚內肥大細胞過多所致，受到刺激時會在皮膚上形成蕁麻疹或其他病變。患者常見紅色或棕色斑點，主要分布於胸部、前額及背部。當肥大細胞受到刺激（如摩擦皮膚、受熱）會釋放過量組織胺，引發過敏反應，使刺激部位出現蕁麻疹，亦稱「達裡爾氏徵」。
relevance score : -100.0%
ReferencesMastocytosis 是一種肥大細胞過多的病症，通常累及皮膚、骨髓和消化系統等部位。根據世界衛生組織 (WHO) 的定義，cutaneous mastocytosis 可分為三種主要類型。第一類（mastocytomas）由孤立或少數（≤3）個病變組成。第二種（urticaria pigmentosa）涉及多個病變，數量通常介於 10 余個至不到 100 個。urticaria pigmentosa 是兒童皮膚肥大細胞增多症最常見的形式，但亦可出現在成人身上。此病大多為良性，常在青春期逐漸改善。與成人肥大細胞增多症不同，urticaria pigmentosa 很少累及內臟器官。其顯著特徵是皮膚上易出現小而發癢的紅棕色或黃棕色斑點或病變，常稱為蕁麻疹。這些斑點通常於童年出現，且可能持續一生。
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.
一名 6 歲女孩前來就診，身上出現了幾個深色斑點。這些斑點最初出現在頭皮，之後在過去六個月逐漸擴散至臉部和身體。她提到，受到壓迫時，斑點會隆起、發紅、發癢。她沒有潮紅、嘔吐、腹瀉或喘息等症狀，個人及家族病史亦未提供相關線索。檢查發現，她的頭皮、額頭、臉部和頸部有多處黑斑，胸部和背部則有輕微凸起的黑斑。輕輕摩擦斑點約 2 分鐘後會使其腫脹並發癢，但症狀於 15–20 分鐘內自行消退（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.
