Urticaria pigmentosa - Pigmentna Urtikarija
Pigmentna Urtikarija (Urticaria pigmentosa) je najpogostejša oblika kožne mastocitoze. To je redka bolezen, ki jo povzroča prekomerno število mastocitov v koži, ki ob draženju povzročijo koprivnico ali rane na koži. Rdeče ali rjave lise so pogosto vidne na koži, običajno okoli prsi, čela in hrbta. Ti mastociti, ko so razdraženi (npr. zaradi drgnjenja kože, izpostavljenosti toploti), proizvedejo preveč histamina, kar sproži alergijsko reakcijo, ki povzroči koprivnico, lokalizirano na območju draženja, včasih imenovano "Darierjev znak".
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Ponavadi se pojavi na trupu majhnih otrok.
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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 kožna mastocitoza (cutaneous mastocytosis) razvrstimo v tri glavne vrste. Prvi tip (mastocitomi (mastocytomas)) je sestavljen iz posameznih ali nekaj (≤3) lezij. Drugi tip (urtikarija pigmentosa (urticaria pigmentosa)) vključuje več lezij, ki se običajno gibljejo od več kot 10 do manj kot 100. Zadnji tip kaže razširjeno prizadetost po koži. Urtikarija 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 urtikarija pigmentosa redko prizadene notranje organe. Ena značilnost urtikarije pigmentosa je njena nagnjenost k povzročanju majhnih, srbečih, rdečkasto‑rjavih ali rumenkasto‑rjavih madežev ali lezij na koži, splošno znanih 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 urtikario pigmentoso (Urticaria pigmentosa), ki se je najprej pojavila na njenem lasišču, nato pa se je v zadnjih šestih mesecih razširila na obraz in telo. Omenila je občutek, da se ob pritisku dvignejo, pordečijo in srbijo. Ni imela zardevanja, bruhanja, driske ali piskajočega dihanja, njena osebna in družinska zdravstvena zgodovina pa ni dala ustreznih namigov. Po pregledu smo našli več temnih lis na njenem 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.
