Urticaria pigmentosa - 색소성 두드러기
색소성 두드러기 (Urticaria pigmentosa) 은 피부 비만세포증의 가장 흔한 형태입니다. 피부에 비만세포가 너무 많아 자극을 받았을 때 피부에 두드러기나 병변이 생기는 희귀질환이다. 붉은색 또는 갈색 반점이 피부, 일반적으로 가슴, 이마, 등 주위에 흔히 나타납니다. 이러한 비만 세포는 자극을 받으면(예: 피부를 문지르거나 열에 노출됨) 너무 많은 히스타민을 생성하여 자극 부위에 국한된 두드러기를 유발하는 알레르기 반응을 유발하며 때로는 "다리에 징후"라고도 합니다.
References
Mastocytosis 은 비만세포가 과잉 존재하는 질환으로 피부, 골수, 소화기관 등 다양한 신체 부위에서 흔히 발견됩니다. 세계보건기구 (WHO) 에 따르면 cutaneous mastocytosis 은 (는) 크게 세 가지 유형으로 분류할 수 있습니다. 첫 번째 유형 (mastocytomas) 은 단독 또는 소수 (3개 이하) 의 병변으로 구성됩니다. 두 번째 유형 (urticaria pigmentosa) 은 일반적으로 10개 이상에서 100개 미만 범위의 여러 병변을 포함합니다. 마지막 유형은 피부 전반에 걸쳐 광범위한 병변을 나타냅니다. Urticaria pigmentosa 은 어린이에게 가장 흔한 형태의 피부 비만세포증이지만 성인에게도 발생할 수 있습니다. 일반적으로 청소년기에 개선되는 경우가 많은 무해한 상태입니다. 성인 비만세포증과 달리 urticaria pigmentosa 은 내부 장기에 거의 영향을 미치지 않습니다. Urticaria pigmentosa 의 독특한 특징 중 하나는 일반적으로 두드러기라고 알려진 피부에 작고 가려우며 적갈색 또는 황갈색 반점이나 병변을 일으키는 경향이 있다는 것입니다. 이러한 반점은 대개 어린 시절에 나타나며 평생 동안 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 여아가 처음에는 두피에 여러 개의 짙은 색 반점이 나타났다가 지난 6개월 동안 얼굴과 몸으로 퍼지면서 내원했습니다. 그녀는 압력을 가하면 피부가 붓고, 붉어지고, 가려워지는 느낌을 받았다고 말했습니다. 그녀는 홍조, 구토, 설사 또는 천명을 경험하지 않았으며 개인 및 가족 병력은 관련 단서를 제공하지 않았습니다. 검사 결과, 그녀의 두피, 이마, 얼굴, 목에 여러 개의 검은 반점이 발견되었으며, 가슴과 등에 약간 솟아오른 검은 반점이 발견되었습니다. 가볍게 문지르면 2분 안에 그 부위가 붓고 가려워졌지만, 15~20분 안에 증상이 사라졌습니다 (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.
