Urticaria pigmentosa
Urticaria pigmentosa er algengasta form júgurfrumna í húð. Þetta er sjaldgæfur sjúkdómur sem orsakast af of miklum fjölda mastfrumna í húðinni sem framkallar ofsakláði eða sár á húðinni við ertingu. Rauðir eða brúnir blettir sjást oft á húðinni, venjulega í kringum brjóst, enni og bak. Þessar mastfrumur, þegar þær eru pirraðar (t.d. með því að nudda húðina, útsetning fyrir hita), framleiða of mikið histamín, sem kallar fram ofnæmisviðbrögð sem leiða til ofsakláða sem er staðsett á ertingarsvæðinu, stundum nefnt „Dariers merki“.
☆ Í niðurstöðum Stiftung Warentest 2022 frá Þýskalandi var ánægja neytenda með ModelDerm aðeins minni en með greiddum fjarlækningaráðgjöfum.
Það hefur tilhneigingu til að koma fram á bol ungra barna.
References
Mastocytosis er ástand þar sem ofgnótt er af mastfrumum sem finnast oft í ýmsum líkamshlutum eins og húð, beinmerg og meltingarvegi. Samkvæmt Alþjóðaheilbrigðismálastofnuninni (WHO) er hægt að flokka cutaneous mastocytosis í þrjár megingerðir. Fyrsta gerð (mastocytomas) samanstendur af eintómum eða nokkrum (≤3) sárum. Önnur gerð (urticaria pigmentosa) felur í sér margar skemmdir, venjulega á bilinu fleiri en 10 til færri en 100. Síðasta gerðin sýnir víðtæka þátttöku í húðinni. Urticaria pigmentosa er algengasta form júgurfrumna í húð hjá börnum, en það getur einnig komið fram hjá fullorðnum. Það er venjulega skaðlaust ástand sem batnar oft á unglingsárum. Ólíkt mastocytosis hjá fullorðnum hefur urticaria pigmentosa sjaldan áhrif á innri líffæri. Einn sérkenni urticaria pigmentosa er tilhneiging þess til að valda litlum, kláða, rauðbrúnum eða gulbrúnum blettum eða sárum á húðinni, almennt þekktur sem ofsakláði. Þessir blettir koma venjulega fram í æsku og geta varað alla ævi.
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 ára stúlka kom inn með nokkra dökklita bletti sem komu fyrst í hársvörðinn og dreifðust síðan í andlit og líkama síðastliðið hálft ár. Hún nefndi að finna þær rísa, verða rauðar og klæja þegar þrýstingur var beitt. Hún fann ekki fyrir roða, uppköstum, niðurgangi eða önghljóði og persónuleg og fjölskyldusaga hennar gaf ekki viðeigandi vísbendingar. Við skoðun fundum við marga dökka bletti á hársvörð hennar, enni, andliti og hálsi, ásamt örlítið upphækkuðum dökkum blettum á brjósti og baki. Létt nudda blettanna olli því að þeir bólgnuðu og kláðaðu innan 2 mínútna, en einkennin dofnuðu innan 15–20 mínútna (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.
