Urticaria pigmentosa
Urticaria pigmentosa je najbežnejšou formou kožnej mastocytózy. Ide o zriedkavé ochorenie spôsobené nadmerným počtom žírnych buniek v koži, ktoré pri podráždení vytvárajú žihľavku alebo lézie na koži. Červené alebo hnedé škvrny sú často viditeľné na koži, zvyčajne okolo hrudníka, čela a chrbta. Tieto žírne bunky, keď sú podráždené (napr. trením kože, vystavením teplu), produkujú príliš veľa histamínu, čo spúšťa alergickú reakciu, ktorá vedie k žihľavke lokalizovanej v oblasti podráždenia, niekedy označovanej ako „Darierov príznak“.
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Mastocytosis je stav, pri ktorom je nadbytok žírnych buniek, ktoré sa často nachádzajú v rôznych častiach tela, ako je koža, kostná dreň a tráviaci systém. Podľa Svetovej zdravotníckej organizácie (WHO) možno cutaneous mastocytosis rozdeliť do troch hlavných typov. Prvý typ (mastocytomas) pozostáva zo solitárnych alebo niekoľkých (≤3) lézií. Druhý typ (urticaria pigmentosa) zahŕňa viaceré lézie, typicky v rozsahu od viac ako 10 do menej ako 100. Posledný typ vykazuje rozsiahle postihnutie kože. Urticaria pigmentosa je najbežnejšou formou kožnej mastocytózy u detí, ale môže sa vyskytnúť aj u dospelých. Je to zvyčajne neškodný stav, ktorý sa často zlepšuje počas dospievania. Na rozdiel od mastocytózy dospelých urticaria pigmentosa zriedkavo postihuje vnútorné orgány. Jednou z charakteristických čŕt urticaria pigmentosa je jej tendencia spôsobovať malé, svrbiace, červenohnedé alebo žltohnedé škvrny alebo lézie na koži, bežne známe ako žihľavka. Tieto škvrny sa zvyčajne objavujú v detstve a môžu pretrvávať po celý život.
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-ročné dievča prišlo s niekoľkými tmavými škvrnami, ktoré sa najskôr objavili na pokožke hlavy a potom sa za posledných šesť mesiacov rozšírili na tvár a telo. Spomenula, že pociťuje, ako sa dvíhajú, sčervenajú a svrbia, keď bol aplikovaný tlak. Nezaznamenala návaly tepla, vracanie, hnačku ani sipot a jej osobná a rodinná anamnéza neposkytovala relevantné stopy. Pri vyšetrení sme našli viacero tmavých škvŕn na jej temene, čele, tvári a krku spolu s mierne vyvýšenými tmavými škvrnami na hrudi a chrbte. Ľahké trenie škvŕn spôsobilo, že do 2 minút napuchli a svrbeli, ale symptómy vymizli do 15–20 minút (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.
