Urticaria pigmentosa
Ang Urticaria pigmentosa ay ang pinakakaraniwang anyo ng cutaneous mastocytosis. Ito ay isang bihirang sakit na sanhi ng labis na bilang ng mga mast cell sa balat, na nagdudulot ng mga pantal o sugat kapag naiirita. Karaniwan, makikita ang mga pulang o kayumangging batik sa balat, lalo na sa paligid ng dibdib, noo, at likod. Kapag nairita (hal., sa pamamagitan ng pagkuskos o pagkakalantad sa init), naglalabas ang mga mast cell ng labis na histamine, na nagdudulot ng reaksiyong alerdyi at nagiging sanhi ng mga pantal na nakatuon sa lugar ng pangangati; minsan ito ay tinatawag na “Darier’s sign.”
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ReferencesAng Mastocytosis ay isang kondisyon kung saan may labis na mga mast cell, kadalasang matatagpuan sa iba't ibang bahagi ng katawan tulad ng balat, bone marrow, at digestive system. Ayon sa World Health Organization (WHO), ang cutaneous mastocytosis ay maaaring ikategorya sa tatlong pangunahing uri. Ang unang uri (mastocytomas) ay binubuo ng isang o ilang (≤3) lesyon. Ang pangalawang uri (urticaria pigmentosa) ay nagsasangkot ng maraming sugat, karaniwang higit sa 10 ngunit mas mababa sa 100. Ang huling uri ay nagpapakita ng malawakang pagkakasangkot sa balat. Ang Urticaria pigmentosa ay ang pinakakaraniwang anyo ng cutaneous mastocytosis sa mga bata, ngunit maaari rin itong mangyari sa mga matatanda. Karaniwan itong hindi nakakapinsalang kondisyon at kadalasang bumubuti sa panahon ng pagdadalaga. Hindi tulad ng pang‑adultong mastocytosis, ang urticaria pigmentosa ay bihirang nakakaapekto sa mga panloob na organo. Ang isang natatanging katangian ng urticaria pigmentosa ay ang paglitaw ng maliliit, makati, mapula‑pula‑kayumanggi o madilaw‑dilaw na batik o sugat sa balat, na karaniwang tinatawag na pantal. Ang mga batik na ito ay karaniwang lumilitaw sa pagkabata at maaaring tumagal sa buong buhay.
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.
Isang 6 na taong gulang na batang babae ang pumasok na may ilang madilim na kulay na mga batik na unang lumitaw sa kanyang anit at pagkatapos ay kumalat sa kanyang mukha at katawan sa nakalipas na anim na buwan. Binanggit niya na ang mga ito ay tumataas, namumula, at nangangati kapag nilalapat ang presyon. Hindi siya nakaranas ng pamumula, pagsusuka, pagtatae, o kahirapan sa paghinga, at ang kanyang personal at pamilyang medikal na kasaysayan ay walang kaugnay na pahiwatig. Sa pagsusuri, nakita namin ang maraming madidilim na batik sa kanyang anit, noo, mukha, at leeg, pati na rin ang bahagyang nakataas na madidilim na patak sa kanyang dibdib at likod. Ang bahagyang pagkuskos sa mga batik ay nagdulot ng pamamaga at pangangati sa loob ng 2 minuto, ngunit nawala ang mga sintomas matapos ang 15–20 minuto (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.
