Urticaria pigmentosa
Urticaria pigmentosa er den mest almindelige form for kutan mastocytose. Det er en sjælden sygdom, der skyldes et for stort antal mastceller i huden, som ved irritation kan forårsage nældefeber eller læsioner. Røde eller brune pletter ses ofte på huden, typisk omkring brystet, panden og ryggen. Når mastcellerne er irriterede (f.eks. ved at gnide huden eller ved varme), frigiver de for meget histamin, hvilket udløser en allergisk reaktion med nældefeber i irritationsområdet – nogle gange kaldet Darier's tegn.
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Det har en tendens til at forekomme på torsoen hos små børn.
relevance score : -100.0%
ReferencesMastocytosis er en tilstand, hvor der er et overskud af mastceller, som ofte findes i forskellige kropsdele såsom huden, knoglemarven og fordøjelsessystemet. Ifølge Verdenssundhedsorganisationen (WHO) kan cutaneous mastocytosis kategoriseres i tre hovedtyper. Den første type (mastocytomas) består af solitære eller få (≤ 3) læsioner. Den anden type (urticaria pigmentosa) involverer flere læsioner, typisk fra mere end 10 til mindre end 100. Den tredje type viser udbredt hudinvolvering. Urticaria pigmentosa er den mest almindelige form for kutan mastocytose hos børn, men den kan også forekomme hos voksne. Det er som regel en harmløs tilstand, som ofte forbedres i ungdomsårene. I modsætning til voksen mastocytose påvirker urticaria pigmentosa sjældent de indre organer. Et karakteristisk træk ved urticaria pigmentosa er dens tendens til at forårsage små, kløende, rødbrune eller gulbrune pletter på huden, ofte kaldet nældefeber. Disse pletter opstår normalt i barndommen og kan vare hele livet.
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‑årig pige kom ind med flere mørke pletter, der først dukkede op på hovedbunden og derefter spredte sig til ansigtet og kroppen i løbet af de sidste seks måneder. Hun bemærkede, at pletterne rejste sig, blev røde og kløede, når der blev udøvet tryk. Hun havde ingen rødme, opkastning, diarré eller hvæsende vejrtrækning, og hendes personlige og familiære sygehistorie gav ingen relevante spor. Ved undersøgelsen fandt vi flere mørke pletter på hovedbunden, panden, ansigtet og halsen samt nogle let hævede mørke pletter på brystet og ryggen. Let gnidning af pletterne fik dem til at svulme og klø inden for 2 minutter, men symptomerne forsvandt igen efter 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.
