Urticaria pigmentosa
Urticaria pigmentosa bụ ụdị ọrịa mastocytosis a na-ahụkarị. Ọ bụ ọrịa na-adịghị ahụkebe nke ọnụọgụ mkpụrụ ndụ mast dị na akpụkpọ ahụ na-akpata na-emepụta hives ma ọ bụ ọnya na akpụkpọ ahụ mgbe iwe na-ewe. A na-ahụkarị ntụpọ uhie ma ọ bụ aja aja na akpụkpọ ahụ, ọ na-adịkarị n'akụkụ igbe, ọkpọiso, na azụ. Mkpụrụ ndụ mast ndị a, mgbe iwe (dịka ọmụmaatụ site na ịcha akpụkpọ ahụ, ikpo ọkụ ọkụ), na-emepụta histamine dị ukwuu, na-ebute mmeghachi omume nfụkasị ahụ nke na-eduga na hives na mpaghara nke mgbakasị ahụ, mgbe ụfọdụ a na-akpọ "Akara Darier".
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References
Mastocytosis bụ ọnọdụ ebe enwere oke mkpụrụ ndụ mast, nke a na-ahụkarị n'akụkụ ahụ dị iche iche dịka akpụkpọ ahụ, ụmị ọkpụkpụ, na sistem nri nri. Dị ka Òtù Ahụ Ike Ụwa (WHO) si kwuo, cutaneous mastocytosis nwere ike ekewa n'ime ụdị atọ bụ isi. Ụdị nke mbụ (mastocytomas) nwere ọnya naanị ma ọ bụ ole na ole (≤3) . Ụdị nke abụọ (urticaria pigmentosa) gụnyere ọtụtụ ọnya, na-abụkarị site na ihe karịrị 10 ruo ihe na-erughị 100. Ụdị ikpeazụ na-egosi ntinye aka zuru oke n'ofe akpụkpọ ahụ. Urticaria pigmentosa bụ ụdị ọrịa mastocytosis a na-ahụkarị na ụmụaka, mana ọ nwekwara ike ime na ndị okenye. Ọ na-abụkarị ọnọdụ na-adịghị emerụ ahụ nke na-akawanye mma n'oge uto. N'adịghị ka mastocytosis okenye, urticaria pigmentosa adịghị emetụta akụkụ ahụ dị n'ime. Otu ihe pụrụ iche nke urticaria pigmentosa bụ àgwà ya nke ime ka ọ dị obere, na-egbuke egbuke, na-acha ọbara ọbara-agba aja aja, ma ọ bụ na-acha odo odo-agba aja aja n'ahụ akpụkpọ ahụ, nke a na-akpọkarị hives. Ebe ndị a na-apụtakarị n'oge ọ bụ nwata ma nwee ike ịdịru ogologo ndụ.
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.
Otu nwa agbọghọ dị afọ 6 batara nwere ọtụtụ ntụpọ gbara ọchịchịrị nke mbụ pụtara n'isi ya wee gbasaa n'ihu na ahụ ya n'ime ọnwa isii gara aga. O kwuru na ọ na-enwe mmetụta ka ha na-ebili, na-acha uhie uhie, na ihe mgbu mgbe etinyere nrụgide. Ọ hụghị nhịahụ, ịgba agbọ, afọ ọsịsa, ma ọ bụ iku ume, yana akụkọ ahụike nke onwe ya na nke ezinụlọ enyeghị nkọwa dị mkpa. Mgbe anyị nyochachara, anyị hụrụ ọtụtụ ntụpọ gbara ọchịchịrị n'isi ya, ọkpọiso, ihu, na olu ya, yana n'obi na azụ ya. Ihicha ntụpọ ndị ahụ n'ụzọ dị mfe mere ka ha zaa ma na-afụ ụfụ n'ime nkeji abụọ, mana mgbaàmà ahụ dachapụrụ n'ime nkeji 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.
