Urticaria pigmentosa
Urticaria pigmentosa é a forma mais comum de mastocitose cutânea. É uma doença rara causada por um número excessivo de mastócitos na pele, que produzem urticária ou lesões cutâneas quando são irritados. Manchas vermelhas ou marrons são frequentemente observadas, geralmente no peito, na testa e nas costas. Quando esses mastócitos são estimulados (por exemplo, por fricção da pele ou exposição ao calor), liberam grande quantidade de histamina, desencadeando uma reação alérgica que provoca urticária localizada na área irritada, às vezes chamada de “sinal de Darier”.
☆ Nos resultados do Stiftung Warentest de 2022 da Alemanha, a satisfação do consumidor com o ModelDerm foi apenas ligeiramente inferior do que com as consultas pagas de telemedicina.
relevance score : -100.0%
ReferencesMastocytosis é uma condição em que há excesso de mastócitos, frequentemente encontrados em várias partes do corpo, como pele, medula óssea e sistema digestivo. De acordo com a Organização Mundial da Saúde (OMS), cutaneous mastocytosis pode ser categorizada em três tipos principais. O primeiro tipo (mastocytomas) consiste em lesões solitárias ou poucas (≤ 3). O segundo tipo (urticária pigmentosa) envolve múltiplas lesões, normalmente variando de mais de 10 a menos de 100. O último tipo apresenta envolvimento generalizado em toda a pele. Urticária pigmentosa é a forma mais comum de mastocitose cutânea em crianças, mas também pode ocorrer em adultos. É geralmente uma condição inofensiva que melhora durante a adolescência. Ao contrário da mastocitose em adultos, urticária pigmentosa raramente afeta órgãos internos. Uma característica distintiva de urticária pigmentosa é sua tendência a causar manchas ou lesões pequenas, com coceira, marrom‑avermelhadas ou marrom‑amareladas na pele, conhecidas como urticária. Essas manchas geralmente aparecem na infância e podem durar toda a vida.
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.
Uma menina de 6 anos chegou com várias manchas escuras que apareceram primeiro no couro cabeludo e, depois, se espalharam pelo rosto e pelo corpo nos últimos seis meses. Ela relatou senti‑las subir, ficarem vermelhas e coçarem quando a pressão era aplicada. Não apresentou rubor, vômito, diarreia ou respiração ofegante, e seu histórico médico pessoal e familiar não revelou pistas relevantes. No exame, observamos múltiplas manchas escuras no couro cabeludo, testa, rosto e pescoço, além de lesões levemente elevadas no peito e nas costas. Ao esfregar levemente as manchas, estas incham e coçam em cerca de 2 minutos, mas os sintomas desaparecem em 15–20 minutos (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.
