Urticaria pigmentosa
Urticaria pigmentosa o le ituaiga sili ona taatele o le mastocytosis paʻu. O se fa'ama'i e seasea tupu, e mafua mai i le tele o mast cells i totonu o le paʻu, ma e mafai ona maua fa'ama'i po'o ni manu'a i le paʻu pe a ita. E masani ona iloa i le lanu mumu po'o le enaena i luga o le paʻu, e masani lava i le fatafata, muaulu, ma tua. O nei sela mast, pe a ita (fa'ata'ita'iga e ala i le oloina o le paʻu, vevela), e tele le histamine, ma e fa'aaoso se fa'ama'i e o'o atu i sulu fa'apitonu i le vaega o le ita; i nisi taimi e ta'ua o le “Darier’s sign”.
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ReferencesMastocytosis o se tulaga e tele ai sela mast, e masani ona maua i vaega ese‑ese o le tino e pei o le pa'u, ga'o ponaivi, ma le faiga o mea'ai. E tusa ai ma le Faalapotopotoga o le Soifua Maloloina a le Lalolagi (WHO), cutaneous mastocytosis e mafai ona faavasegaina i ni ituaiga autu se tolu. O le ituaiga muamua (mastocytomas) e aofia ai na'o ni nai (≤3) fa'ama'i. O le ituaiga lona lua (urticaria pigmentosa) e aofia ai le tele o fa'ama'i, e masani lava mai le sili atu i le 10 i lalo o le 100. O le ituaiga mulimuli e faʻaalia le salalau lautele o le pa'u. Urticaria pigmentosa o le mastocytosis sili ona taatele i tamaiti, ae mafai foi ona tupu i tagata matutua. E masani lava o se tulaga le afaina, lea e masani ona faaleleia i le taimi o le talavou. I le mastocytosis matua, urticaria pigmentosa e seasea aafia ai totoga totonu. O se tasi o uiga iloga o le urticaria pigmentosa o le mea e mafua ai ni mea laiti, mageso, mūmū‑enaena, po'o le samasama‑enaena po'o ni manu'a i le pa'u, e masani ona ta'ua o sulu. O nei togitogi e masani ona aliali mai a o laiti ma e mafai ona tumau i le olaga atoa.
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.
O se teineitiiti e 6 tausaga le matua na sau i totonu ma le tele o lanu uliuli na aliali muamua i lona ulu, sosolo atu i ona foliga ma lona tino i le ono masina talu ai. Na ia taʻua le lagona o le tulaʻi, mumu, ma mageso pe a faʻafipiʻi le mamafa. Na te le'i o'o i le fufula, pua'i, manava manava, po'o le uigi, ma o lona talaaga fa'afoma'i patino ma lona aiga e le'i maua ni fa'aataataga talafeagai. I le su'esu'ega, na matou maua le tele o mea pogisa i luga o lona ulu, muaulu, foliga, ma le ua, faatasi ai ma paʻu pogisa isi i luga o lona fatafata ma lona tua. O le olo mama o ila na mafua ai ona fula ma mageso i totonu o le 2 minute, ae na mou atu auga i totonu o le 15-20 minute (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.
