Urticaria pigmentosa
Urticaria pigmentosa er den vanligste formen for kutan mastocytose. Det er en sjelden sykdom som skyldes et overdreven antall mastceller i huden, og som gir elveblest eller lesjoner når huden blir irritert. Røde eller brune flekker ses ofte på huden, vanligvis rundt brystet, pannen og ryggen. Disse mastcellene produserer for mye histamin når de blir irritert (f.eks. ved å gni huden eller ved varmeeksponering), og utløser en allergisk reaksjon som fører til elveblest lokalisert til irritasjonsområdet, noen ganger kalt «Dariers tegn».
☆ I 2022 Stiftung Warentest-resultatene fra Tyskland var forbrukertilfredsheten med ModelDerm bare litt lavere enn med betalte telemedisinkonsultasjoner.
Det har en tendens til å forekomme på overkroppen til små barn.
relevance score : -100.0%
ReferencesMastocytose er en tilstand med et overskudd av mastceller, som ofte finnes i ulike deler av kroppen, som hud, benmarg og fordøyelsessystemet. I følge Verdens helseorganisasjon (WHO) kan cutaneous mastocytosis deles inn i tre hovedtyper. Den første typen (mastocytomas) består av én eller noen få (≤ 3) lesjoner. Den andre typen (urticaria pigmentosa) omfatter flere lesjoner, vanligvis fra mer enn 10 til færre enn 100. Den siste typen viser en omfattende involvering av hele huden. Urticaria pigmentosa er den vanligste formen for kutan mastocytose hos barn, men den kan også forekomme hos voksne. Tilstanden er som regel ufarlig og forbedres ofte i ungdomsårene. I motsetning til voksen mastocytose påvirker urticaria pigmentosa sjelden indre organer. Et særtrekk ved urticaria pigmentosa er at den kan gi små, kløende, rødbrune eller gulbrune flekker eller lesjoner på huden, ofte kalt elveblest. Disse flekkene dukker vanligvis opp i barndommen og kan vare livet ut.
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.
En 6 år gammel jente kom inn med flere mørke flekker som først dukket opp på hodebunnen og deretter spredte seg til ansiktet og kroppen i løpet av de siste seks månedene. Hun opplevde at de hevet seg, ble røde og kløet når trykk ble påført. Hun hadde ingen rødme, oppkast, diaré eller hvesing, og hennes personlige og familiehistorie ga ingen relevante ledetråder. Ved undersøkelse fant vi flere mørke flekker på hodebunnen, pannen, ansiktet og nakken, samt noen hevede mørke flekker på brystet og ryggen. Lett gnidning av flekkene førte til at de hovnet opp og kløet innen 2 minutter, men symptomene avtok etter 15–20 minutter (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.
