Urticaria pigmentosa
Urticaria pigmentosa ke mofuta o atileng haholo oa mastocytosis ea letlalo. Ke lefu le sa tloaelehang le bakoang ke palo e feteletseng ea lisele tsa mast letlalong tse hlahisang mahlahahlaha kapa liso letlalong ha le hloekile. Hangata matheba a khubelu kapa a sootho a bonoa letlalong, hangata a pota-potile sefuba, phatleng le mokokotlong. Lisele tsena tsa mast, ha li halefisitsoe (mohlala, ka ho senya letlalo, ho pepesehela mocheso), li hlahisa histamine e ngata haholo, e leng se etsang hore ho be le tšoaetso e lebisang ho li-hives sebakeng sa ho teneha, ka linako tse ling ho thoe ke "letšoao la Darier".
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References
Mastocytosis ke boemo boo ho nang le lisele tse ngata tsa mast, tse fumanehang likarolong tse fapaneng tsa 'mele joalo ka letlalo, moko oa masapo le tsamaiso ea lijo. Ho latela Mokhatlo oa Lefatše oa Bophelo (WHO) , cutaneous mastocytosis e ka aroloa ka mefuta e meraro e meholo. Mofuta oa pele (mastocytomas) o na le maqeba a le mong kapa a seng makae (≤3) . Mofuta oa bobeli (urticaria pigmentosa) o kenyelletsa maqeba a mangata, hangata ho tloha ho feta 10 ho ea ho tlase ho 100. Mofuta oa ho qetela o bontša ho ameha ho pharaletseng ho pholletsa le letlalo. Urticaria pigmentosa ke mofuta o atileng haholo oa mastocytosis ea letlalo ho bana, empa e ka etsahala le ho batho ba baholo. Hangata ke boemo bo se nang kotsi bo atisang ho ntlafala nakong ea bocha. Ho fapana le mastocytosis ea batho ba baholo, urticaria pigmentosa ha se hangata e ama litho tsa ka hare. Ntho e 'ngoe e ikhethang ea urticaria pigmentosa ke tšekamelo ea eona ea ho baka matheba a manyane, a ho hlohlona, a bofubelu bo sootho, kapa a bosootho bo bosehla letlalong, a tsejoang ka hore ke hives. Hangata matheba ana a hlaha bongoaneng 'me a ka tšoarella bophelo bohle.
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.
Ngoanana ea lilemo li 6 o ile a kena a e-na le matheba a ’maloa a ’mala o motšo a ileng a qala ho hlaha letlalong la hlooho eaba a namela sefahlehong le ’meleng likhoeling tse tšeletseng tse fetileng. O boletse hore ba ikutloa ba phahama, ba fetoha bofubelu, 'me ba hlohlona ha khatello e sebelisoa. Ha aa ka a hlatsa, a hlatsa, letšollo, kapa ho hema, 'me nalane ea hae ea bongaka le ea lelapa ha e fane ka lintlha tse nepahetseng. Ha re mo hlahloba, re ile ra fumana matheba a matšo a mangata letlalong la hlooho, phatleng, sefahlehong le molaleng, hammoho le matheba a matšo a phahamisitseng hanyenyane sefubeng le mokokotlong. Ho hohla matheba hanyenyane ho ile ha etsa hore a ruruhe le ho hlohlona nakong ea metsotso e 2, empa matšoao a ile a fela nakong ea metsotso e 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.
