Urticaria pigmentosa
Urticaria pigmentosa hè a forma più cumuna di mastocitosi cutanea. Hè una malatia rara causata da un numeru eccessivu di mastociti in a pelle, chì produce bugni o lesioni quandu a pelle hè irritata. I punti rossi o marroni si trovanu spessu nantu à a pelle, tipicamenti intornu à u pettu, a fronte è u spinu. Quessi mastociti, quandu sò irritati (per esempiu, per frizione di a pelle o esposizione à u calore), pruducenu troppu istamina, inducendu una reazione allergica chì porta à l’urticaria localizzata in l’area di irritazione, qualchì volta chjamata «signu di Darier».
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ReferencesMastocytosis hè una cundizione induve ci hè un eccessu di mastociti, spessu truvati in diverse parti di u corpu cum'è a pelle, a medula ossea è u sistema digestivu. Sicondu l'Organizazione Mondiale di a Salute (OMS), a mastocitosi cutanea pò esse categurizata in trè tipi principali. U primu tipu (mastocytomas) hè custituitu da lesioni solitarie o da un pocu (≤3). U sicundu tipu (urticaria pigmentosa) implica parechje lesioni, tipicamenti trà più di 10 è menu di 100. L'ultimu tipu mostra una implicazione generalizata in tutta a pelle. L'urticaria pigmentosa hè a forma più cumuna di mastocitosi cutanea in i zitelli, ma pò ancu presentassi in l'adulti. Hè di solitu una condizione innocua chì spessu migliora durante l'adolescenza. A differenza di a mastocitosi adulta, l'urticaria pigmentosa raramente affetta l'organi interni. Una caratteristica distintiva di l'urticaria pigmentosa hè a so tendenza à causà macchie o lesioni chjuchi, pruriginosi, rossi‑marroni o gialli‑marroni nantu à a pelle, cumunemente cunnisciute cum'è urticaria. Sti macchie sò generalmente apparite in l'infanzia è ponu persiste per tutta a vita.
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.
Una zitella di 6 anni hè ghjunta cù parechje macchie di culore scuru chì sò apparite prima nantu à u so scalp è poi si sò sparse à a so faccia è u corpu in l'ultimi sei mesi. Ella hà dettu chì, quandu si gratta, e macchie si alzanu, diventanu rossi è pruriginose. Ùn hà micca sperimentatu vomitu, diarrea, sibilu, né altri sintomi sistemichi, è a so storia medica persunale è famigliali ùn hà furnitu indizi pertinenti. À l'esaminazione, avemu trovu parechje macchie scure nantu à u scalp, a fronte, a faccia è u collu, inseme cù petti scuri leggermente elevati nantu à u pettu è u spinu. Un strofinamentu leggeru di e macchie li ha fattu gonfiare è prurire in 2 minuti, ma i sintomi svaniscenu in 15‑20 minuti (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.
