Urticaria pigmentosa
Ko te Urticaria pigmentosa te āhua tino noa o te mastocytosis kiri. He mate te raruraru nā te nui rawa o ngā pūtau mast i te kiri, e tuku ana i te histamine ina whakaaweawe, ka hua ake ngā hives, ko ngā pākau rānei. Ka puta ngā wheti whero, parauri rānei, i te nuinga o te wā i runga i te puku, te mata, me te tuara. Ina riri ngā pūtau mast (hei tauira mā te mirimiri i te kiri, te whakamātao wera), ka tuku nui te histamine, ka whakaoho i tētahi whakautu ārai ā-rohe e kīia ana ko te “Darier’s sign”.
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Ko te Urticaria pigmentosa (Urticaria pigmentosa) te āhua tino noa o te mastocytosis ā‑kiri. He hēkona iti tēnei nā te nui o ngā pūtau mast i te kiri, e tuku ana i te histamine ina whakaaweawe, ka hua ngā pupuhi, ngā peka rānei. Ka puta ngā whewhe whero, parauri, pērā i te whewhe whero‑parauri, i te puku, i te upoko, me te tuara. Ina whakaaweawe ngā pūtau mast (hei tauira, mā te mirimiri te kiri, te wera), ka tuku rahi rātou i te histamine, ka whakaoho i tētahi whakautu ā‑matea ā‑ringa e kīia ana ko te tohu a Darier (Darier’s sign). Ka pā tēnei i te tinana o ngā tamariki i te wā o te kōpū, ā, ka kitea i te torso. Ka taea te rerekē i te peka mā te mirimiri kaha, ka puku, ka whewhe. He āhua pai, ka whakamutua i te rangatahi, ā, kāore i te nuinga o te wā e pā ana ki ngā wāhi o roto i te nuinga o ngā tamariki. E wehewehe ana te WHO i te mastocytosis ā‑kiri ki ngā āhua toru: (1) ko te mastocytoma kotahi, (2) ko te Urticaria pigmentosa (UP) – he maha ngā peka (i te nui atu i te 10, i raro i te 100), (3) ko te urticaria diffuse. Ko te UP te āhua tino noa i ngā tamariki, engari ka kitea anō i ngā pakeke. He huringa pai, ka taea te whakaora i te rangatahi, ā, kāore i te nuinga o te wā e pā ana ki ngā wāhi o roto. He tauira: i tētahi kōtiro 6‑tau te pakeke, he maha ngā peka pango i te upoko, te puku, te tuara, ā, ka whewhe te peka i te wā ka pēhia, ka ngau i te 2 meneti, ka ngau i te 15–20 meneti, e tohu ana i te tohu a Darier (Darier’s sign).
relevance score : -100.0%
ReferencesKo te Mastocytosis he āhua e pā ana ki te nui o ngā pūtau mast, ā, ka kitea i ngā wāhanga tinana pērā i te kiri, te ate (liver), te wheua (spleen), me te pūnaha rākau (lymphatic system). E ai ki te World Health Organization (WHO), ka taea te whakarōpūtia te cutaneous mastocytosis 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 whara, 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. He āhua pai, e whakakāhore ana, ka rongo i te taiohitanga (benign, self‑resolving condition that often remits in adolescence). Kaore i te mastocytosis pakeke e pā ana ki ngā whekau o roto, ā, i te urticaria pigmentosa he iti noa te pā ki ngā wāhi o roto (rare internal organ involvement). He āhuatanga motuhake o te urticaria pigmentosa ko tōna kaha ki te whakaputa i ngā wāhi iti, te tito, te whero‑parauri, te kowhai‑parauri rānei, arā, ngā macules (lesions) i runga i te kiri, e mōhiotia ana hei hives (urticaria). Ka puta ēnei wāhi i te wā o te tamarikitanga, ā, ka mau tonu i te roanga o te ao (throughout life). Ka puta te tohu a Darier (Darier’s sign) ina whakakāhorehia ngā macules, ka piri te urticaria.
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, he wāhi e whakaatu ana i te Urticaria pigmentosa (Urticaria pigmentosa), 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 whānau 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.
