Urticaria pigmentosa
Urticaria pigmentosa yeyona ndlela ixhaphakileyo ye-cutaneous mastocytosis. Sisifo esinqabileyo esibangelwa ngamanani amaninzi eeseli zemast esikhumbeni ezivelisa imidumba okanye izilonda esikhumbeni xa zicaphukile. Amabala abomvu okanye amdaka ahlala ebonwa eluswini, ngokuqhelekileyo ejikeleze isifuba, ibunzi, nasemva. Ezi seli zemast, xa zicatshukiswa (umzekelo, ngokuxubha ulusu, ukuvezwa kobushushu), zivelisa i-histamine eninzi, ibangela ukusabela okungahambi kakuhle okukhokelela kwimiqolomba ehlala kwindawo yokucaphuka, ngamanye amaxesha ibizwa ngokuba yi "Darier's sign".
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References
Mastocytosis yimeko apho kukho ukugqithiswa kweeseli zemast, ezihlala zifumaneka kwiindawo ezahlukeneyo zomzimba ezifana nolusu, umongo wethambo, kunye nenkqubo yokwetyisa. Ngokutsho koMbutho wezeMpilo weHlabathi (i-WHO) , cutaneous mastocytosis ingahlelwa kwiintlobo ezintathu eziphambili. Uhlobo lokuqala (mastocytomas) luqulathe izilonda zodwa okanye ezimbalwa (≤3) . Uhlobo lwesibini (urticaria pigmentosa) lubandakanya izilonda ezininzi, ngokuqhelekileyo ukusuka ngaphezu kwe-10 ukuya ngaphantsi kwe-100. Uhlobo lokugqibela lubonisa ukubandakanyeka okubanzi kulo lonke ulusu. Urticaria pigmentosa yeyona ndlela ixhaphakileyo ye-cutaneous mastocytosis ebantwaneni, kodwa ingenzeka nakubantu abadala. Idla ngokuba yimeko engenabungozi edla ngokuphucuka ngexesha lokufikisa. Ngokungafaniyo ne-mastocytosis yabantu abadala, urticaria pigmentosa ayifane ichaphazele amalungu angaphakathi. Olunye uphawu olwahlukileyo lwe urticaria pigmentosa kukuthanda ukubangela amabala amancinci, arhawuzelela, abomvu-mdaka, okanye amabala atyheli-mdaka esikhumbeni, aziwa ngokuba ziihives. La mabala aqhele ukuvela ebuntwaneni kwaye anokuhlala ubomi bonke.
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.
Intombazana eneminyaka emi-6 ubudala yeza namachokoza amnyama aqala ukuvela entloko emva koko asasazeka ebusweni nasemzimbeni kwezi nyanga zintandathu zidlulileyo. Ukhankanye ukuziva bephakama, bajike babebomvu, kunye nokurhawuzelelwa xa kusenziwa uxinzelelo. Akazange abe nangxaki yokugungxulwa, ukugabha, urhudo, okanye ukuphefumla, kwaye imbali yakhe yobuqu kunye neyosapho zonyango ayizange ibonelele ngemikhondo efanelekileyo. Ekuxilongeni, safumana amabala amnyama amaninzi entloko, ebunzi, ebusweni, nasentanyeni, kunye namabala amnyama anyuswe kancinci esifubeni nasemqolo. Ukuhlikihla kancinci amabala kubangele ukuba adumbe kwaye arhawuzelelwe ngemizuzu emi-2, kodwa iimpawu ziye zaphela 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.
