Urticaria pigmentosa - Pigmentna Urtikarija
Pigmentna Urtikarija (Urticaria pigmentosa) je najpogostejša oblika kožne mastocitoze. Gre za redko bolezen, ki jo povzroča prekomerno število mastocitov v koži, ki ob draženju povzročijo koprivnico ali izpuščaje. Rdeče ali rjave lise so pogosto vidne na koži, zlasti okoli prsi, čela in hrbta. Ko so ti mastociti razdraženi (npr. zaradi drgnjenja kože ali izpostavljenosti toploti), proizvajajo preveč histamina, kar sproži alergijsko reakcijo – koprivnico, ki se pojavi lokalno na mestu draženja, včasih imenovano “Darierjev znak”.
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ReferencesMastocytosis je stanje, pri katerem obstaja presežek mastocitov, ki jih pogosto najdemo v različnih delih telesa, kot so koža, kostni mozeg in prebavni sistem. Po podatkih Svetovne zdravstvene organizacije (WHO) lahko cutaneous mastocytosis razvrstimo v tri glavne vrste. Prvi tip (mastocytomas) je sestavljen iz posameznih ali nekaj (≤ 3) lezij. Drugi tip (urticaria pigmentosa) vključuje več lezij, ki se običajno gibljejo med več kot 10 in manj kot 100. Tretji tip kaže razširjeno prizadetost kože. Urticaria pigmentosa je najpogostejša oblika kožne mastocitoze pri otrocih, vendar se lahko pojavi tudi pri odraslih. Običajno je to neškodljivo stanje, ki se med adolescenco pogosto izboljša. Za razliko od mastocitoze pri odraslih, urticaria pigmentosa redko prizadene notranje organe. Ena značilnost urticaria pigmentosa je nagnjenost k nastanku majhnih, srbečih, rdečkasto‑rjavih ali rumenkasto‑rjavih madežev oziroma lezij na koži, ki so splošno znane kot koprivnica. Te lise se običajno pojavijo v otroštvu in lahko trajajo vse življenje.
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-letna deklica je prišla z več temnimi madeži, ki so se najprej pojavili na njenem lasišču, nato pa so se v zadnjih šestih mesecih razširili na obraz in telo. Omenila je občutek, da se ob pritisku dvignejo, pordečijo in srbe. Ni imela zardevanja, bruhanja, driske ali piskajočega dihanja; njena osebna in družinska zdravstvena zgodovina pa ni dala ustreznih namigov. Po pregledu smo opazili več temnih lis na lasišču, čelu, obrazu in vratu, skupaj z rahlo dvignjenimi temnimi lisami na prsih in hrbtu. Rahlo drgnjenje madežev je povzročilo otekanje in srbenje v 2 minutah, vendar so simptomi izginili v 15–20 minutah (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.
