Urticaria pigmentosa - Urtikaaria Pigmentosa
Urtikaaria Pigmentosa (Urticaria pigmentosa) on kõige levinum naha mastosütoosi vorm. See on haruldane haigus, mis on põhjustatud liigsest mastosüütide arvust nahas, mis ärrituse korral tekitavad nõgestõbi või kahjustusi. Nahal on sageli näha punaseid või pruune laike, tavaliselt rinnal, otsmikul ja seljal. Kui need mastosüütid on ärritunud (nt nahka hõõrudes või kuumusega kokkupuutel), toodavad nad liiga palju histamiini, põhjustades allergilise reaktsiooni, mis viib ärrituspiirkonnas lokaliseeritud nõgestõbini, mida mõnikord nimetatakse Darieri märgiks.
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ReferencesMastocytosis on seisund, kus nuumrakke on liiga palju; neid leidub sageli erinevates kehaosades, näiteks nahas, luuüdis ja seedesüsteemis. Maailma Terviseorganisatsiooni (WHO) andmetel võib cutaneous mastocytosis liigitada kolme põhitüüpi. Esimene tüüp (mastocytomas) koosneb üksikutest või mõnest (≤ 3) kahjustusest. Teine tüüp (urticaria pigmentosa) hõlmab mitut kahjustust, tavaliselt 10–100 kahjustust. Viimane tüüp hõlmab laialdast nahakahjustust. Urticaria pigmentosa on kõige levinum naha mastotsütoosi vorm lastel, kuid see võib esineda ka täiskasvanutel. See on tavaliselt kahjutu seisund, mis sageli paraneb noorukieas. Erinevalt täiskasvanute mastotsütoosist mõjutab urticaria pigmentosa harva siseorganeid. Üks urticaria pigmentosa eripära on kalduvus tekitada nahal väikeseid, sügelevaid, punakaspruune või kollakaspruune laike, mida tavaliselt nimetatakse nõgestõveks. Need laigud ilmuvad tavaliselt lapsepõlves ja võivad püsida kogu elu.
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.
6-aastane tüdruk tuli esile mitme tumedate värvi laiguga, mis tekkisid esmalt peanahale ja levisid viimase kuue kuu jooksul näole ja kehale. Ta mainis, et tundis, kuidas need tõusevad, muutuvad punaseks ja sügelevad, kui surve avaldati. Ta ei kogenud õhetust, oksendamist, kõhulahtisust ega vilistavat hingamist ning tema isiklik ja perekondlik haiguslugu ei andnud asjakohaseid vihjeid. Uurimisel avastasime peanahal, otsmikul, näol ja kaelal mitu tumedat laiku ning rinnal ja seljal veidi kõrgemad tumedad laigud. Kerge hõõrumine tekitas nende turse ja sügeluse 2 minuti jooksul, kuid sümptomid kadusid 15–20 minutiga (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.
