Urticaria pigmentosa
Urticaria pigmentosa är den vanligaste formen av kutan mastocytos. Det är en sällsynt sjukdom som orsakas av ett för stort antal mastceller i huden, vilket ger nässelutslag eller lesioner när de irriteras. Röda eller bruna fläckar syns ofta på huden, särskilt på bröstet, pannan och ryggen. När dessa mastceller irriteras (t.ex. vid gnuggning av huden eller exponering för värme) frisätts för mycket histamin, vilket utlöser en allergisk reaktion med nässelutslag på det irriterade området, ibland kallat ”Dariers tecken”.
☆ I Stiftung Warentest-resultaten 2022 från Tyskland var konsumentnöjdheten med ModelDerm bara något lägre än med betalda telemedicinkonsultationer.
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ReferencesMastocytos är ett tillstånd där det finns ett överskott av mastceller, som ofta återfinns i olika delar av kroppen såsom huden, benmärgen och matsmältningssystemet. Enligt Världshälsoorganisationen (WHO) kan cutaneous mastocytosis kategoriseras i tre huvudtyper. Den första typen (mastocytomas) består av enstaka eller några få (≤ 3) lesioner. Den andra typen (urticaria pigmentosa) involverar flera lesioner, vanligtvis fler än 10 men färre än 100. Den tredje typen visar en utbredd hudinvolvering. Urticaria pigmentosa är den vanligaste formen av kutan mastocytos hos barn, men den kan även förekomma hos vuxna. Det är vanligtvis ett ofarligt tillstånd som ofta förbättras under tonåren. Till skillnad från mastocytos hos vuxna påverkar urticaria pigmentosa sällan inre organ. En utmärkande egenskap hos urticaria pigmentosa är dess tendens att ge små, kliande, rödbruna eller gulbruna fläckar eller lesioner på huden, allmänt kallade nässelfeber. Dessa fläckar uppträder ofta i barndomen och kan bestå hela 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 flicka kom in med flera mörka fläckar som först dök upp på hårbotten och sedan spred sig till ansikte och kropp under de senaste sex månaderna. Hon uppgav att fläckarna känns när de reser sig, blir röda och kliar vid tryck. Hon hade ingen rodnad, kräkningar, diarré eller väsande andning, och hennes personliga samt familjemedicinska historia gav inga relevanta ledtrådar. Vid undersökning fann vi flera mörka fläckar på hårbotten, panna, ansikte och hals samt lätt upphöjda mörka fläckar på bröst och rygg. Lätt gnuggning av fläckarna gjorde att de svullnade och kliade inom två minuter, men symtomen försvann inom 15–20 minuter (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.
