Urticaria pigmentosa - 色素性荨麻疹
色素性荨麻疹 (Urticaria pigmentosa) 是皮肤肥大细胞增多症最常见的形式。这是一种罕见的疾病，由皮肤中过多的肥大细胞引起，当受到刺激时，会在皮肤上产生荨麻疹或病变。皮肤上经常出现红色或棕色斑点，通常在胸部、前额和背部周围。这些肥大细胞在受到刺激时（例如摩擦皮肤、受热）会产生过多的组胺，引发过敏反应，导致刺激区域出现荨麻疹，有时称为“达里尔氏征”。
References
Mastocytosis 是一种肥大细胞过多的病症，通常存在于皮肤、骨髓和消化系统等身体各个部位。根据世界卫生组织 (WHO) 的说法， cutaneous mastocytosis 可分为三种主要类型。第一类（ mastocytomas ）由孤立或少数（≤3）个病变组成。第二种类型 (urticaria pigmentosa) 涉及多个病变，通常范围从 10 多个到不到 100 个。最后一种类型显示整个皮肤广泛受累。 Urticaria pigmentosa 是儿童皮肤肥大细胞增多症最常见的形式，但也可能发生在成人中。这通常是一种无害的病症，通常会在青春期期间得到改善。与成人肥大细胞增多症不同， urticaria pigmentosa 很少影响内脏器官。 urticaria pigmentosa 的一个显着特征是它容易在皮肤上引起小、发痒、红棕色或黄棕色斑点或病变，通常称为荨麻疹。这些斑点通常出现在童年时期，并可能持续一生。
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 岁女孩前来就诊，身上出现了几个深色斑点，这些斑点首先出现在她的头皮上，然后在过去的六个月里扩散到她的脸部和身体。她提到，当施加压力时，感觉它们会上升、变红、发痒。她没有出现潮红、呕吐、腹泻或喘息的症状，她的个人和家庭病史也没有提供相关线索。经检查，我们发现她的头皮、额头、面部和颈部有多处黑斑，胸部和背部有轻微凸起的黑斑。轻轻摩擦斑点会在 2 分钟内使斑点肿胀并发痒，但症状会在 15-20 分钟内消失 (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.
