Urticaria pigmentosa
Is é Urticaria pigmentosa an fhoirm is coitianta de mastocytosis craicinn. Is galar neamhchoitianta é de bharr líon iomarcach crann cealla sa chraiceann a tháirgeann coirceoga nó loit ar an gcraiceann nuair a bhíonn greannú orthu. Is minic a fheictear spotaí dearga nó donn ar an gcraiceann, go hiondúil timpeall an chliabhraigh, an mhaoil, agus an chúl. Táirgeann na crannchealla seo, nuair a bhíonn greannú orthu (m.sh. an craiceann a chuimilt, nochtadh teasa), an iomarca histamine, rud a chuireann tús le frithghníomhú ailléirgeach logánta, ar a dtugtar “comhartha Darier”.
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ReferencesIs coinníoll é Mastocytosis ina bhfuil barraíocht cealla mast, a fhaightear go minic i gcodanna éagsúla den chorp ar nós an craicinn, an trácht gastaireach, agus an córas limfhí. De réir na hEagraíochta Domhanda Sláinte (WHO), is féidir cutaneous mastocytosis a chatagóiriú i dtrí phríomhchineál. Is éard atá sa chéad chineál (mastocytomas) lúb aonaracha nó cúpla (≤3). Baineann an dara cineál (urticaria pigmentosa) le lúb iolracha, idir níos mó ná 10 go níos lú ná 100 go hiondúil. Léiríonn an cineál deiridh rannpháirtíocht fhorleathan ar fud an chraicinn. Is é Urticaria pigmentosa an fhoirm is coitianta de mastocytosis craicinn i leanaí, ach is féidir go dtarlódh sé i ndaoine fásta freisin. De ghnáth is riocht neamhdhíobhálach é a imíonn go minic le linn na hóige. Mura ionann mastocytosis do dhaoine fásta, is annamh a bhíonn tionchar ag urticaria pigmentosa ar orgáin inmheánacha. Gné shainiúil amháin de urticaria pigmentosa ná an claonadh atá ann le spotaí nó lúbanna beaga, pruinnteach, donn dearg, nó donn buí a chur faoi deara ar an gcraicinn, ar a dtugtar coirceoga (hives) go coitianta. Is iondúil go bhfeictear na spotaí seo le linn óige agus maireann siad ar feadh an tsaoil.
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.
Tháinig cailín 6 bliana d'aois isteach le roinnt spotaí dorcha‑daite a bhí le feiceáil den chéad uair ar a scalp agus a scaipeadh ar a aghaidh agus ar a corp le sé mhí anuas. Luaigh sí go raibh siad ag mothú go n-ardóidh, go n-iompaíonn siad dearg, agus go n-éireodh leo nuair a cuireadh brú. Ní raibh taithí aici ar shruthlú, urlacan, buinneach ná wheezing, agus níor thug a stair leighis phearsanta agus teaghlaigh leideanna ábhartha. Tar éis scrúdaithe, fuarthas go leor spotaí dorcha ar a scalp, a mhullach, a aghaidh agus a muineál, chomh maith le plaicí dorcha beagán ardaithe ar a chroí agus ar a chúl. Nuair a chuimiltear na spotaí go héadrom, tháinig urticáir agus pruritus laistigh de 2 nóiméad, agus d'imigh sé i dtaobh laistigh de 15‑20 nóiméad (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.
