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表现。这是一种罕见疾病，由皮肤中过多的肥大细胞引起。受到刺激时，肥大细胞会释放大量组胺，导致局部出现荨麻疹或皮损。患者常在胸部、前额和背部出现红色或棕色斑点。摩擦、受热等刺激可诱发症状，出现所谓的“达里尔氏征”。
relevance score : -100.0%
ReferencesMastocytosis 是一种肥大细胞过多的疾病，常累及皮肤、骨髓和消化系统等部位。根据世界卫生组织 (WHO) 的定义，cutaneous mastocytosis 可分为三种主要类型。第一类（mastocytomas）表现为孤立或少数（≤3）病变。第二类（urticaria pigmentosa）涉及多个病变，数量通常在 10 余个至不足 100 个之间。第三类则表现为全身皮肤广泛受累。Urticaria pigmentosa 是儿童皮肤肥大细胞增多症最常见的形式，也可见于成人。该病通常是良性的，多在青春期后自行改善。与成人肥大细胞增多症不同，urticaria pigmentosa 很少累及内脏器官。urticaria pigmentosa 的显著特征是皮肤上出现小而瘙痒的红棕色或黄棕色斑点或病变，常被称为荨麻疹。这些斑点多在童年出现，且可能伴随终生。
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。这些斑点最初出现在头皮上，随后在过去六个月里扩散至面部和身体。她提到，受到压力时，斑点会升高、发红、瘙痒。她没有出现潮红、呕吐、腹泻或喘息等症状，个人和家族史也未提供相关线索。检查发现，她的头皮、额头、面部和颈部有多处黑斑，胸部和背部有轻度凸起的黑斑。轻轻摩擦斑点约2分钟后会出现肿胀和瘙痒，但症状在15–20分钟内自行消失（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.
