Urticaria pigmentosa - Wrticaria Pigmentosa
Wrticaria Pigmentosa (Urticaria pigmentosa) yw'r math mwyaf cyffredin o fastocytosis croenol. Mae'n afiechyd prin a achosir gan ormodedd o gelloedd mast yn y croen sy'n cynhyrchu cychod gwenyn neu friwiau ar y croen pan fyddant yn llidiog. Mae smotiau coch neu frown i'w gweld yn aml ar y croen, fel arfer o amgylch y frest, y talcen a'r cefn. Mae'r celloedd mast hyn, o'u llidio (e.e. trwy rwbio'r croen, amlygiad gwres), yn cynhyrchu gormod o histamin, gan ysgogi adwaith alergaidd sy'n arwain at gychod gwenyn wedi'u lleoli yn yr ardal o lid, y cyfeirir ato weithiau fel "arwydd Darier".
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References
Mae Mastocytosis yn gyflwr lle mae gormodedd o gelloedd mast, a geir yn aml mewn gwahanol rannau o'r corff fel y croen, mêr esgyrn, a'r system dreulio. Yn ôl Sefydliad Iechyd y Byd (WHO) , gellir categoreiddio cutaneous mastocytosis yn dri phrif fath. Mae'r math cyntaf (mastocytomas) yn cynnwys briwiau unigol neu ychydig (≤3) . Mae'r ail fath (urticaria pigmentosa) yn cynnwys briwiau lluosog, fel arfer yn amrywio o fwy na 10 i lai na 100. Mae'r math olaf yn dangos ymglymiad eang ar draws y croen. Urticaria pigmentosa yw'r math mwyaf cyffredin o fastocytosis croenol mewn plant, ond gall hefyd ddigwydd mewn oedolion. Fel arfer mae'n gyflwr diniwed sy'n aml yn gwella yn ystod llencyndod. Yn wahanol i fastocytosis oedolion, anaml y mae urticaria pigmentosa yn effeithio ar organau mewnol. Un nodwedd nodedig o urticaria pigmentosa yw ei duedd i achosi smotiau neu friwiau bach, coslyd, coch-frown, neu felyn-frown ar y croen, a elwir yn gyffredin fel cychod gwenyn. Mae'r smotiau hyn fel arfer yn ymddangos yn ystod plentyndod a gallant bara gydol oes.
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.
Daeth merch 6 oed i mewn gyda sawl smotyn lliw tywyll a ymddangosodd gyntaf ar ei chroen pen ac yna'n lledu i'w hwyneb a'i chorff dros y chwe mis diwethaf. Soniodd am deimlo eu bod yn codi, yn troi'n goch, ac yn cosi pan roddwyd pwysau. Ni chafodd brofiad o fflysio, chwydu, dolur rhydd na gwichian, ac nid oedd ei hanes meddygol personol a theuluol yn rhoi cliwiau perthnasol. Ar ôl ei harchwilio, canfuom nifer o smotiau tywyll ar groen y pen, ei thalcen, ei hwyneb a'i gwddf, ynghyd â chlytiau tywyll ychydig yn uwch ar ei brest a'i chefn. Roedd rhwbio'r smotiau'n ysgafn yn achosi iddynt chwyddo a chosi o fewn 2 funud, ond pylu'r symptomau o fewn 15-20 munud (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.
