Urticaria pigmentosa
Ko te Urticaria pigmentosa te āhua o te mastocytosis kiri. He mate onge na te nui rawa o ngā mast cell i roto i te kiri ka puta he hiwi, he whiu rānei kei runga i te kiri ina whakaparahako. Ka kitea ngā wāhi whero, parauri rānei i runga i te kiri, i te kapa, te rae, me te tuara. Ko ēnei pūtau mast, ina riri (hei tauira mā te mirimiri i te kiri, te wera), ka nui rawa te whakaputa histamine, ka puta he whiu i te wāhi o te kiri, i ētahi wā ka kīia ko “Darier’s sign”.
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ReferencesKo te Mastocytosis he ahuatanga kei reira te taikaha o ngā pūtau mast, he maha ngā wā ka kitea i roto i ngā momo tinana pērā i te kiri, te pūpū, me te pūnaha pāpū. E ai ki te World Health Organization (WHO), cutaneous mastocytosis ka taea te whakarōpūtia ki ngā momo matua e toru. Ko te momo tuatahi (mastocytomas) he mea mokemoke, he torutoru rānei (≤3) ngā whara. Ko te momo tuarua (urticaria pigmentosa) he maha ngā whiu, mai i te neke atu i te 10 ki te iti iho i te 100. Ko te momo whakamutunga e whakaatu ana i te whakauru whānui puta noa i te kiri. Ko te urticaria pigmentosa te āhua o te mastocytosis kiri i roto i ngā tamariki, engari ka puta anō i ngā pakeke. Ko te tikanga he āhua pai, ka pai ake i te wā o te taiohi. Kaore i te mastocytosis pakeke, urticaria pigmentosa he iti te pānga ki ngā whekau o roto. He āhuatanga motuhake o te urticaria pigmentosa ko tōna kaha ki te whakaputa i ngā wāhi iti, te piri, te whero‑parauri, te kowhai‑parauri rānei, ngā wharanga i runga i te kiri, e mōhiotia ana he hives. Ko ēnei wāhi ka puta i te wā o te tamaiti, ka mau tonu i te wā katoa.
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.
I tae mai tetahi kotiro 6 tau te pakeke me etahi wahi pouri ka puta tuatahi ki runga i tona mahunga ka horapa ki tona mata me tona tinana i nga marama e ono kua hipa. I kii ia ka piki ake, ka whero, ka patito i te wa e pehia ana. Karekau ia i pa ki te rewharewha, te ruaki, te mate korere, te wheehe ranei, a, ko nga hitori o te hauora whaiaro me te whanau kaore i te whakaatu tohu e tika ana. I runga i te tirotiro, he maha nga wahi pouri i kitea i runga i tona mahunga, te rae, te kanohi, me te kaki, me nga wahi pouri kua ara ake i runga i tona uma me tona tuara. Ko te mirimiri ngawari i nga wahi ka pupuhi me te patito i roto i te 2 meneti, engari ka memeha nga tohu i roto i te 15-20 meneti (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.
