Urticaria pigmentosahttps://en.wikipedia.org/wiki/Urticaria_pigmentosa
Urticaria pigmentosa is de meast foarkommende foarm fan kutane mastocytose. It is in seldsume sykte dy't feroarsake wurdt troch tefolle oantallen mastcellen yn 'e hûd, dy't histamine frijlitte by stimulatie, wat netels of lesjes feroarsaakje. Reade of brune flekken ferskine faak, benammen op de boarst, foarholle en rêch. As dizze mastcellen irritearre wurde (bgl. troch wriemjen fan de hûd of hitte), frijlitte se grutte hoemannichten histamine, wat in lokale allergyske reaksje útluit, bekend as Darier’s sign.

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  • It hat de neiging om te foarkommen op 'e romp fan jonge bern.
  • It hurd wriuwen fan de lesion kin in swelling feroarsaakje.
References Urticaria Pigmentosa 29494109 
NIH
Mastocytosis is in betingst wêryn d'r in oerskot is oan mastsellen, faak fûn yn ferskate lichemsdielen lykas de hûd, bonkenmurg en spijsverteringssysteem. Neffens de Wrâldsûnensorganisaasje (WHO) kin cutaneous mastocytosis wurde yndield yn trije haadtypen. It earste type (mastocytomas) bestiet út ien of in pear (≤3) lesjes. It twadde type (urticaria pigmentosa) omfettet meardere bywenningen, typysk fariearjend fan mear as 10 oant minder as 100. It lêste type toant wiidferspraat belutsenens oer de hûd. Urticaria pigmentosa is de meast foarkommende foarm fan kutane mastozytosis by bern, mar it kin ek foarkomme by folwoeksenen. It is in normaal harmlike tastân dy’t faak ferbetteret yn de adolesinsje. Oars as folwoeksen mastozytosis, hat urticaria pigmentosa komselden ynfloed op ynterne organen. By stimulatie litte de mastcellen histamine frij, wat netten (hives) of lesjes (lesions) op de hûd feroarsaket. Ien ûnderskiedend skaaimerk fan urticaria pigmentosa is de oanstriid om lytse, jeukende, readbrún of gielbrún flekken of lesjes (lesions) op de hûd te feroarsaakjen, ornaris bekend as net (hives). It ferskinen normaal yn de jeugd en kinne it libben duorje. Darier's teken (Darier's sign) wurdt aktivearre troch it fyn rûmen fan de lesjes, wêrtroch binnen twa minuten urtikaasje en jeuk optrint, dy't binnen 15–20 minuten ferbynt.
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.
 Urticaria pigmentosa - Case reports 26752589 
NIH
In famke fan 6 jier kaam binnen mei ferskate donkere flekken dy't earst op har hoofdhuid ferskynden en dêrnei oer har gesicht en lichem oer de ôfrûne seis moanne ferspriede. Se neamde it gefoel dat se opstean, read wurde en jeuken as druk waard tapast. Se belibbe gjin spoelen, braken, diarree, of piepende piepen, en har persoanlike en famylje-medyske histoarjes levere gjin relevante oanwizings. By ûndersyk fûnen wy meardere donkere plakken op har hoofdhuid, foarholle, gesicht en nekke, tegearre mei wat ferhege donkere plakken op har boarst en rêch. Licht wrijven fan 'e flekken feroarsake dat se binnen 2 minuten swollen en jeuken, mar de symptomen ferdwûnen binnen 15-20 minuten (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.