Urticaria pigmentosa - 색소성 두드러기
색소성 두드러기 (Urticaria pigmentosa)은 피부 비만세포증의 가장 흔한 형태입니다. 피부에 비만세포가 과다하게 존재하여 자극을 받으면 두드러기나 병변이 나타나는 희귀질환입니다. 붉은색 또는 갈색 반점이 피부에, 주로 가슴, 이마, 등 부위에 흔히 나타납니다. 이러한 비만세포는 자극을 받으면(예: 피부를 문지르거나 열에 노출될 때) 과도한 히스타민을 방출하여 자극 부위에 국한된 두드러기를 일으키는 알레르기 반응을 일으키며, 때로는 “다리 징후”라고도 합니다.
relevance score : -100.0%
ReferencesMastocytosis은 비만세포가 과다하게 존재하는 질환으로, 피부, 골수, 소화기관 등 다양한 신체 부위에서 흔히 발견됩니다. 세계보건기구(WHO)에 따르면 cutaneous mastocytosis은 크게 세 가지 유형으로 분류됩니다. 첫 번째 유형인 mastocytomas는 단일 병변이거나 3개 이하의 소수 병변으로 나타납니다. 두 번째 유형인 urticaria pigmentosa는 일반적으로 10개 이상, 100개 미만의 다수 병변을 포함합니다. 세 번째 유형은 피부 전체에 걸쳐 광범위한 병변이 나타나는 형태입니다. Urticaria pigmentosa는 어린이에게 가장 흔한 형태의 피부 비만세포증이지만, 성인에게도 발생할 수 있습니다. 대부분은 청소년기에 자연히 호전되는 무해한 상태이며, 성인 비만세포증과 달리 내부 장기에 거의 영향을 주지 않습니다. Urticaria pigmentosa의 특징 중 하나는, 흔히 두드러기라고 불리는 작고 가려우며 적갈색 또는 황갈색의 반점(또는 병변)이 피부에 나타나는 경향이 있다는 점입니다. 이러한 반점은 대개 어린 시절에 나타나고, 평생 지속될 수 있습니다.
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.
6세 여아는 처음에 두피에 여러 개의 짙은 색 반점이 나타났고, 지난 6개월 동안 그 반점이 얼굴과 몸으로 퍼져 내원했습니다. 압력을 가하면 피부가 붓고 붉어지며 가려워지는 느낌을 받았다고 합니다. 홍조, 구토, 설사, 천명을 경험하지 않았으며, 개인 및 가족 병력에서도 관련 단서는 찾지 못했습니다. 검사 결과 두피, 이마, 얼굴, 목에 여러 개의 검은 반점이 관찰되었고, 가슴과 등에도 약간 솟아오른 검은 반점이 보였습니다. 가볍게 문지르면 2분 이내에 해당 부위가 붓고 가려워지지만, 15~20분 정도 지나면 증상이 사라집니다 (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.
