Urticaria pigmentosa
Ko te Urticaria pigmentosa te ahua o te mastocytosis kiri (cutaneous mastocytosis). He mate onge na te nui rawa o nga mast cells i roto i te kiri ka puta he hiwi, he whiu ranei kei runga i te kiri ina whakaparahako. Ka kitea nga waahi whero, parauri ranei i runga i te kiri, i te taha o te uma, te rae, me te tuara. Ko enei pūtau mast, ina riria (hei tauira ma te mirimiri i te kiri, te wera o te wera), ka nui rawa te whakaputa histamine, ka puta he mate mate ka puta ki nga hives i te waahi o te irirangi, i etahi wa ka kiia ko "Darier's sign".
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Ka puta ki runga i te tinana o nga tamariki nohinohi.
relevance score : -100.0%
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 hinu wheua, me te pūnaha nakunaku. E ai ki te World Health Organization (WHO), mastocytosis kiri (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 kino kore ka pai ake i te wā o te taiohitanga. Kāore i rite ki te mastocytosis pakeke, urticaria pigmentosa he uaua ka pā ki ngā whekau o roto. Ko tētahi ahuatanga motuhake o te urticaria pigmentosa ko tōna kaha ki te whakaputa i ngā wāhi iti, te tito, te whero‑parauri, te kowhai‑parauri rānei, he mate rānei i runga i te kiri, e mōhiotia ana he pōwhiri (hives). Ko ēnei wāhi ka puta i te wā o te tamarikitanga, 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.
He kōtiro 6‑tau te pakeke i whakaatu i te Urticaria pigmentosa, ā, i puta he wāhi pouri tuatahi ki runga i tana mahunga, ka horapa ki tana mata me tana tinana i ngā marama e ono kua hipa. I kii ia ka piki ake, ka whero, ka patito i te wā e pehia ana. Kāore ia i pā ki te rewharewha, te ruaki, te mate korere, te wheehe rānei, ā, kāore ngā hitori o te hauora whaiaro me te whānau i whakaatu tohu e tika ana. I runga i te tirotiro, he maha ngā wāhi pouri i kitea i runga i tana mahunga, te rae, te kanohi, me te kaki, me ngā wāhi pouri kua ara ake i runga i tana umu me tana tuara. Ko te mirimiri ngawari i ngā wāhi ka pupuhi me te patito i roto i te 2 meneti, engari ka memeha ngā 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.
