Urticaria pigmentosa - Urtikaria Pigmentaria
Urtikaria Pigmentaria (Urticaria pigmentosa) larruazaleko mastozitosiaren forma ohikoena da. Gaixotasun arraroa da larruazaleko masto-zelulen gehiegizko kopuruak eragiten duena, narritatuta larruazalean erlauntza edo lesioak sortzen dituztenak. Orban gorriak edo marroiak azala askotan ikusten dira, normalean bularrean, kopetan eta bizkarrean. Masto-zelula hauek, narritatuta daudenean (adibidez, larruazala igurtziz, bero-esposizioarekin), histamina gehiegi sortzen dute, eta horrek narritadura-eremuan kokatutako erlauntza eragiten du, batzuetan "Darier-en seinalea" deritzona.
☆ Alemaniako Stiftung Warentest-en 2022ko emaitzetan, ModelDerm-ekin kontsumitzaileen gogobetetasuna apur bat txikiagoa izan zen ordaindutako telemedikuntzako kontsultekin baino.
References
Mastocytosis mastozelulen gehiegizko egoera bat da, askotan gorputzeko hainbat ataletan aurkitzen dena, hala nola larruazalean, hezur-muinean eta digestio-aparatuan. Osasunaren Mundu Erakundearen (OME) arabera, cutaneous mastocytosis hiru mota nagusitan sailka daitezke. Lehenengo mota (mastocytomas) lesio bakartiek edo gutxi batzuek (≤3) osatzen dute. Bigarren motak (urticaria pigmentosa) lesio anitz dakartza, normalean 10 baino gehiago eta 100 baino gutxiago bitartekoak. Azken motak larruazaleko inplikazio zabala erakusten du. Urticaria pigmentosa haurrengan larruazaleko mastozitosi forma ohikoena da, baina helduetan ere gerta daiteke. Nerabezaroan askotan hobetzen den baldintza kaltegabea izan ohi da. Helduen mastozitosiak ez bezala, urticaria pigmentosa oso gutxitan eragiten du barne-organoetan. Urticaria pigmentosa -ren ezaugarri bereizgarri bat larruazalean orban edo lesio txikiak, azkura, marroi-gorrixkak edo marroi horixkak sortzeko joera da, erlauntza bezala ezagutzen direnak. Orban hauek haurtzaroan agertu ohi dira eta bizitza osoan iraun dezakete.
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.
6 urteko neska bat sartu zen kolore iluneko hainbat orbanekin, lehenengo azalean agertu eta gero aurpegira eta gorputzera hedatu ziren azken sei hilabeteetan. Presioa aplikatzean altxatzen, gorritzen eta azkura sentitzea aipatu zuen. Ez zuen gorritzerik, okarik, beherakoa edo txisturik izan, eta bere historia mediko pertsonalek eta familiarek ez zuten arrasto garrantzitsurik eman. Azterketa egin ondoren, azalean, bekokian, aurpegian eta lepoan orban ilun anitz aurkitu genituen, bularrean eta bizkarrean apur bat altxatutako orban ilunekin batera. Lekuak arin igurtziz gero, 2 minuturen buruan puztu eta azkura eragin zituen, baina sintomak 15-20 minututan desagertu ziren (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.
