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 nantu à a pelle quandu irritata. I punti rossi o marroni sò spessu vistuti nantu à a pelle, tipicamenti intornu à u pettu, a fronte è u spinu. Issi mastociti, quandu sò irritati (per esempiu, frottendu a pelle, esposizione à u calore), pruducianu troppu istamina, inducendu una reazione allergica chì porta à l'urticaria localizzata à l'area di l'irritazione, qualchì volta chjamata "signu di Darier".
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References
Mastocytosis 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) , cutaneous mastocytosis pò esse categurizatu in trè tippi principali. U primu tipu (mastocytomas) hè custituitu di lesioni solitarie o uni pochi (≤3) . U sicondu tipu (urticaria pigmentosa) implica parechje lesioni, tipicamenti chì varienu da più di 10 à menu di 100. L'ultimu tipu mostra una implicazione generalizata in tutta a pelle. Urticaria pigmentosa hè a forma più cumuna di mastocitosi cutanea in i zitelli, ma pò ancu esse in adulti. Hè di solitu una cundizione innocu chì spessu migliurà durante l'adolescenza. A cuntrariu di a mastocitosi adulta, urticaria pigmentosa raramente affetta l'organi internu. Una caratteristica distintiva di urticaria pigmentosa hè a so tendenza à causà spots o lesioni chjuchi, pruriginosi, rossi-marroni o gialli-marroni nantu à a pelle, cumunimenti cunnisciuti cum'è urticaria. Sti spots sò generalmente apparsu in a zitiddina è ponu durà in 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 apparsu prima nantu à u so scalp è poi si sparse à a so faccia è u corpu in l'ultimi sei mesi. Ella hà dettu chì sentenu chì si alzanu, diventanu rossi è prurigineghjanu quandu a pressione hè stata applicata. Ùn hà micca sperimentatu vaghjime, vomitu, diarrea o sibilanti, è a so storia medica persunale è famigliali ùn hà micca furnitu indizi pertinenti. À l'esaminazione, avemu trovu parechje macchie scure nantu à u so scalp, a fronte, a faccia è u collu, inseme cù petti scuri ligeramente elevati nantu à u so pettu è u spinu. Un strofinamentu ligeru di e macchie li fece 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.
