Urticaria pigmentosa - I-Urticaria Pigmentosa
I-I-Urticaria Pigmentosa (Urticaria pigmentosa) iwuhlobo oluvame kakhulu lwe-cutaneous mastocytosis. Kuyisifo esingavamile esibangelwa izinombolo eziningi zama-mast cells esikhumbeni ezikhiqiza imifantu noma izilonda lapho kuqala. Amachashaza abomvu noma ansundu avame ukubonakala esikhumbeni, ngokuvamile azungeze esifubeni, ebunzini nangemuva. Lawa ma-mast cells, lapho ecasukile (isb. ngokuhlikihla isikhumba, ukuchayeka kokushisa), akhiqiza i-histamine eningi, edala ukushayisana okuholela emifundeni ebekwe endaweni yokucasuka, ngezinye izikhathi ebizwa ngokuthi “uphawu luka-Darier”.
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ReferencesI-Mastocytosis yisimo lapho kunokwanda kwama‑mast cells, ngokuvamile atholakala ezingxenyeni ezihlukahlukene zomzimba njengokuthi isikhumba, umnkantsha, kanye nesistimu yokugaya ukudla. Ngokwe‑World Health Organization (WHO), i‑cutaneous mastocytosis ingahlukaniswa ngezinhlobo ezintathu eziyinhloko. Uhlobo lokuqala (mastocytomas) luqukethe izilonda ezodwa noma ezimbalwa (≤3). Uhlobo lwesibili (urticaria pigmentosa) lubandakanya izilonda eziningi, ngokuvamile ezisukela kokungaphezu kuka‑10 kuya ngaphansi kuka‑100. Uhlobo lokugcina lubonisa ukubandakanyeka okusabalele esikhumbeni sonke. Urticaria pigmentosa iwuhlobo oluvame kakhulu lwe‑cutaneous mastocytosis ezinganeni, kodwa lungenzeka nakubantu abadala. Ngokuvamile kuyisimo esingenangozi esivame ukuthuthuka ngokuhamba kwesikhathi. Ngokungafani ne‑mastocytosis yabantu abadala, urticaria pigmentosa ayivamisile ukuthinta izitho zangaphakathi. Isici esisodwa esihlukile se‑urticaria pigmentosa ukuthambekela kwayo ukudala amabala amancane, aluhlaza, abomvu, noma ansundu, ngokuvamile ebomvu, noma izilonda esikhumbeni, ezivame ukubizwa ngokuthi ama‑hives. Lawa machashaza ngokuvamile avela ebuntwaneni futhi angahlala impilo yonke.
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.
Intombazane eneminyaka engu-6 ubudala ifikile namabala amnyama aqala ukuvela ekhanda layo, asakazeka ebusweni nasemzimbeni ezinyangeni ezingu-6 ezedlule. Ubalule ukuthi uzizwa ekhuphuka, ebomvu, futhi alume lapho kufakwa ingcindezi. Akazange abe nokushaywa umoya, ukuhlanza, isifo sohudo, noma ukugula, futhi umlando wakhe siqu kanye nomndeni wezokwelapha awuzange unikeze izinkomba ezifanele. Lapho sihlolwa, sathola amabala amnyama amaningi ekhanda lakhe, esiphongweni, ebusweni, nasentanyeni, kanye namabala amnyama aphakeme kancane esifubeni nasemhlane. Ukuhlikihla kancane kwamabala kubangele ukuthi avuvuke futhi alume phakathi nemizuzu emi-2, kodwa izimpawu zanyamalala phakathi kwemizuzu eyi-15–20 (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.
