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ʻO ka pane ʻana i ka lāʻau me ka eosinophilia a me nā hōʻailona ʻōnaehana (DRESS), i ʻike ʻia hoʻi ʻo ka drug-induced hypersensitivity syndrome, he hopena koʻikoʻi ʻo T-cell-mediated cutaneous i hōʻike ʻia e ka hū, ke kuni, ke komo ʻana o nā ʻōpū o loko, a me nā hōʻailona ʻōnaehana ma hope o ka hoʻohana lōʻihi ʻana i kekahi mau lāʻau.
Loaʻa ka DRESS ma kahi o 1 i 1,000 a i 1 i 10,000 mau maʻi e loaʻa ana i ka lāʻau lapaʻau, ma muli o ke ʻano o ka lāʻau hoʻoulu. ʻO ka hapa nui o nā hihia DRESS ma muli o nā lāʻau lapaʻau ʻelima, ma ke ʻano iho o ka hanana: allopurinol, vancomycin, lamotrigine, carbamazepine, a me trimethopridine-sulfamethoxazole. ʻOiai he kakaikahi ka DRESS, hiki i ka 23% o ka hopena o ka lāʻau ʻili i nā poʻe maʻi. Ma hope o kēia pae, hoʻomohala pinepine ka poʻe maʻi i kahi ʻōpala e like me ka maʻi maʻi e hoʻomaka ana ma ka ʻōpū a me ka maka a palahalaha mālie, ma hope o ka uhi ʻana ma mua o 50% o ka ʻili ma ke kino. ʻO ka edema maka kekahi o nā hiʻohiʻona o DRESS a hiki ke hōʻeha a alakaʻi paha i ka ʻāʻī oblique ear lobe hou, e kōkua ana i ka hoʻokaʻawale ʻana i ka ʻaʻahu mai ka lāʻau lapaʻau like ʻole.

Hiki i nā poʻe maʻi me ka DRESS ke hōʻike mai i nā ʻano maʻi like ʻole, e like me ka urticaria, eczema, nā loli lichenoid, exfoliative dermatitis, erythema, nā maʻi i hoʻohālikelike ʻia, purpura, blisters, pustules, a i ʻole ka hui pū ʻana o ia mau mea. Hiki ke loaʻa nā ʻili ʻili he nui i ka maʻi hoʻokahi i ka manawa like a i ʻole e loli i ka wā e piʻi ai ka maʻi. I nā poʻe maʻi me ka ʻili ʻeleʻele, ʻaʻole ʻike ʻia ka erythema mua, no laila pono e nānā pono ʻia ma lalo o nā kūlana kukui maikaʻi. He mea maʻamau ka pustules ma ka maka, ka ʻāʻī a me ka ʻāpana o ka umauma.

I ka prospective, valided European Registry of Serious Cutaneous Adverse Reactions (RegiSCAR) study, 56% o DRESS maʻi hoʻomohala mucosal mumū a me ka erosion, me 15% o na maʻi i mucosal mumū e pili ana i na wahi he nui, ka hapanui o ka oropharynx. ʻo ka hoʻonui ʻana ma mua o nā hōʻailona ʻili. ʻOi aku ka maʻamau ma mua o ʻelua pule a ʻoi aku ka lōʻihi o ka hoʻōla ʻana, ʻo ka desquamation superficial ka hiʻohiʻona nui. Eia kekahi, ʻoiai kakaʻikahi loa, aia kekahi helu liʻiliʻi o nā mea maʻi me ka DRESS ʻaʻole hiki ke hele pū ʻia me kahi maʻi a i ʻole eosinophilia.

ʻO nā maʻi maʻamau o DRESS ka mea maʻamau e pili ana i ke koko, ate, puʻupaʻa, akemāmā, a me nā ʻōnaehana puʻuwai, akā aneane hiki ke hoʻopili ʻia nā ʻōnaehana o ke kino (e like me ka endocrine, gastrointestinal, neurological, ocular, and rheumatic system). Ma ke aʻo ʻana RegiSCAR, 36 pakeneka o nā maʻi i loaʻa i hoʻokahi okana ʻokoʻa ʻē aʻe i komo, a ʻo 56 pākēneka i loaʻa i ʻelua a ʻoi aʻe nā kino. ʻO ka lymphocytosis atypical ka maʻi maʻamau a me ka mua loa o ka hematological abnormality, akā ʻo ka eosinophilia ka mea maʻamau i nā pae hope o ka maʻi a hiki ke hoʻomau.
Ma hope o ka ʻili, ʻo ke ake ka mea maʻamau i hoʻopilikia pinepine ʻia. Hiki ke piʻi aʻe nā pae enzyme o ka ate ma mua o ka ʻike ʻia ʻana o ka ʻūhū, ma ke ʻano he haʻahaʻa haʻahaʻa, akā hiki i kekahi manawa ke piʻi i ka 10 manawa o ka palena kiʻekiʻe o ka maʻamau. ʻO ke ʻano maʻamau o ka ʻeha ate ʻo ka cholestasis, a ukali ʻia e ka cholestasis hui ʻia a me ka ʻeha hepatocellular. I nā hihia koʻikoʻi, ʻoi aku ka paʻakikī o ka hāʻule ʻana o ke akepaʻa e koi ai i kahi transplant ate. I nā hihia o DRESS me ka hana ʻino o ka ate, ʻo ka papa lāʻau pathogenic maʻamau ka antibiotic. Ua nānā ʻia kahi loiloi ʻōnaehana i nā maʻi 71 (67 mau mākua a me nā keiki 4) me DRES pili i ka renal sequelae. ʻOiai ʻo ka hapa nui o nā poʻe maʻi i hōʻino ʻia i ka ate, 1 i loko o 5 mau mea maʻi e hōʻike nei me ke komo ʻana o nā puʻupaʻa kaʻawale. ʻO nā lāʻau antibiotic nā lāʻau lapaʻau maʻamau e pili ana i ka pōʻino o ke kīkī i ka poʻe maʻi DRESS, me ka vancomycin e hoʻoulu ai i ka 13 pākēneka o ka pōʻino kīkī, a ukali ʻia e allopurinol a me nā anticonvulsants. Ua hōʻike ʻia ka hōʻeha renal acute e ka piʻi ʻana o ka pae creatinine serum a i ʻole ka emi ʻana o ka helu kānana glomerular, a ʻo kekahi mau hihia i hele pū ʻia me ka proteinuria, oliguria, hematuria a i ʻole nā ​​​​ʻekolu. Eia hou, aia paha he hematuria kaawale a i ʻole proteinuria, a ʻaʻohe mimi. ʻO 30% o nā maʻi i hoʻopilikia ʻia (21/71) i loaʻa i ka lāʻau lapaʻau renal replacement therapy, a ʻoiai he nui nā mea maʻi i loaʻa hou i ka hana kīkī, ʻaʻole maopopo inā loaʻa nā sequelae lōʻihi. ʻO ke komo ʻana o ka māmā, ʻike ʻia e ka pōkole o ka hanu, ka ʻū maloʻo, a i ʻole nā ​​​​mea ʻelua, ua hōʻike ʻia ma 32% o nā maʻi DRESS. ʻO nā maʻi like ʻole o ka pulmonary ma ka hoʻokolokolo kiʻi, ʻo ia ka infiltration interstitial, acute respiratory distress syndrome a me pleural effusion. ʻO nā pilikia e pili ana i ka pneumonia interstitial acute, lymphocytic interstitial pneumonia, a me ka pleurisy. No ka mea, ua kuhi hewa pinepine ʻia ka ʻaʻahu pulmonary e like me ka maʻi maʻi maʻi, pono ke kiʻekiʻe o ka makaʻala. Aneane nā hihia a pau me ke komo ʻana o ka māmā i hele pū ʻia me nā hana ʻino ʻē aʻe. Ma kahi loiloi hou aʻe, a hiki i ka 21% o nā maʻi DRESS i loaʻa i ka myocarditis. Hiki ke lohi ka myocarditis no nā mahina ma hope o ka emi ʻana o nā hōʻailona ʻē aʻe o DRESS, a i ʻole e hoʻomau. Aia nā ʻano mai ka myocarditis eosinophilic acute (ke kala ʻana me ka mālama ʻana i ka immunosuppressive no ka manawa pōkole) a i ka myocarditis necrotizing eosinophilic acute (ke make ma mua o 50% a me ke ola ma waena o 3 a 4 mau lā wale nō). ʻO ka poʻe maʻi me ka myocarditis e hōʻike pinepine ʻia me ka dyspnea, ka ʻeha o ka umauma, ka tachycardia, a me ka hypotension, i hui pū ʻia me ke kiʻekiʻe o ka myocardial enzyme kiʻekiʻe, nā loli electrocardiogram, a me nā mea ʻino echocardiographic (e like me ka pericardial effusion, systolic dysfunction, ventricular septal hypertrophy, a me ka biventricular failure). Hiki i ka cardiac magnetic resonance imaging ke hōʻike i nā ʻeha endometrial, akā ʻo ka maʻi maʻamau e koi pinepine i kahi biopsy endometrial. ʻAʻole maʻamau ka komo ʻana o ka māmā a me ka myocardial i ka DRESS, a ʻo ka minocycline kekahi o nā mea hoʻoulu maʻamau.

Ua hōʻoia ʻia ka ʻōnaehana helu RegiSCAR ʻEulopa a hoʻohana nui ʻia no ka ʻike ʻana o DRESS (Papa 2). Hoʻokumu ʻia ka ʻōnaehana helu ma nā ʻano ʻehiku: ka wela o ke kino ma luna o 38.5°C; Hoʻonui ʻia nā puʻupuʻu lymph i ʻelua mau wahi; Eosinophilia; Atypical lymphocytosis; Rash (e uhi ana ma mua o 50% o ka ʻili o ke kino, nā hiʻohiʻona morphological hiʻohiʻona, a i ʻole nā ​​ʻike histological e kūlike me ka hypersensitivity lāʻau); Ke komo ʻana o nā ʻāpana ʻokoʻa-cutaneous; A me ke kala lōʻihi (ʻoi aku ma mua o 15 mau lā).
Mai ka −4 a hiki i ka 9, a hiki ke puunaueia ka maopopo o ka diagnostics i eha mau pae: o ka helu ma lalo o ka 2 he ma'i ole, 2 a hiki i ka 3 ke hoike mai i ka ma'i, 4 a hiki i ka 5 ke hoike mai i ka ma'i malia loa, a oi aku mamua o 5 e hoike ana i ka ma'i o ka LAHU. He mea maikaʻi loa ka helu RegiSCAR no ka hōʻoia hope ʻana o nā hihia i hiki ke hoʻokō ʻia no ka mea ʻaʻole i hoʻokō pono nā mea maʻi i nā koina diagnostic āpau i ka hoʻomaka ʻana o ka maʻi a ʻaʻole i loaʻa kahi loiloi piha e pili ana i ka helu.

Pono e ho'oka'awale 'ia ka LOLE mai kekahi mau hopena ko'iko'i o ka ili, e like me SJS a me na ma'i pili, toxic epidermal necrolysis (TEN), a me acute generalized exfoliating impetigo (AGEP) (Figure 1B). ʻOi aku ka lōʻihi o ka manawa incubation no DRESS ma mua o nā hopena ʻino ʻē aʻe o ka ʻili. Hoʻomaka koke ʻo SJS a me TEN a hoʻoholo maʻamau iā lākou iho i loko o 3 a 4 mau pule, ʻoiai ʻoi aku ka mau o nā hōʻailona DRESS. ʻOiai pono e hoʻokaʻawale ʻia ke komo ʻana o ka mucosal i ka poʻe maʻi DRESS mai SJS a i ʻole TEN, ʻoi aku ka maʻalahi o nā ʻeha mucosal waha ma DRESS a emi ke koko. Hiki ke alakaʻi i ka ʻili ʻili edema ʻano o DRESS i ka catatonic secondary blisters a me ka erosion, ʻoiai ʻo SJS a me TEN i hōʻike ʻia e ka exfoliation epidermal piha piha me ka ʻāʻī ʻana, e hōʻike pinepine ana i ka hōʻailona maikaʻi o Nikolsky. I ka hoʻohālikelike ʻana, ʻike pinepine ʻia ka AGEP i nā hola a i nā lā ma hope o ka ʻike ʻana i ka lāʻau lapaʻau a hoʻoholo wikiwiki i loko o 1 a 2 mau pule. ʻO ka ʻūhā o AGEP ua ʻōwili ʻia a ua haku ʻia me nā pustules ākea ʻaʻole i hoʻopaʻa ʻia i nā follicles lauoho, he ʻano ʻokoʻa mai nā ʻano o DRESS.
Ua hōʻike ʻia kahi noiʻi e hiki mai ana he 6.8% o nā mea maʻi DRESS i loaʻa nā hiʻohiʻona o SJS, TEN a i ʻole AGEP, a ʻo 2.5% ka mea i manaʻo ʻia ua loaʻa nā hopena koʻikoʻi o ka ʻili. ʻO ka hoʻohana ʻana i nā koina hōʻoia RegiSCAR e kōkua i ka ʻike pololei ʻana i kēia mau kūlana.
Eia kekahi, ʻike pinepine ʻia nā lāʻau lapaʻau e like me ka maʻi ʻaʻai maʻamau i loko o 1 a 2 mau pule ma hope o ka hoʻolaha ʻana i ka lāʻau (ʻoi aku ka wikiwiki o ka hoʻolaha hou ʻana), akā ʻaʻole like me DRESS, ʻaʻole i hui pū ʻia kēia mau ʻōpala me ka transaminase kiʻekiʻe, piʻi eosinophilia, a i ʻole ka lōʻihi o ka manawa hoʻōla mai nā hōʻailona. Pono e ho'oka'awale 'ia ka LOLE mai nā wahi ma'i 'ē a'e, e like me ka hemophagocytic lymphohistiocytosis, vascular immunoblastic T-cell lymphoma, a me ka ma'i acute graft-versus-host.

ʻAʻole i hoʻomohala ʻia ka manaʻo a i ʻole nā ​​alakaʻi no ka mālama DRESS; Hoʻokumu ʻia nā ʻōlelo aʻoaʻo lapaʻau e kū nei ma luna o ka ʻikepili nānā a me ka manaʻo loea. Loaʻa ʻole nā ​​haʻawina hoʻohālikelike e alakaʻi i ka mālama ʻana, no laila ʻaʻole like nā ala lapaʻau.
Hoʻomaʻemaʻe i ka lāʻau lapaʻau maʻi
ʻO ka hana mua a koʻikoʻi loa ma DRESS, ʻo ia ke ʻike a hoʻopau i ka lāʻau kumu kumu. ʻO ka hoʻomohala ʻana i nā palapala lāʻau lapaʻau kikoʻī no nā maʻi hiki ke kōkua i kēia kaʻina hana. Me ka hoʻopaʻa ʻana i ka lāʻau lapaʻau, hiki i nā kauka ke hoʻopaʻa pono i nā lāʻau lapaʻau a pau e hiki ai ke hoʻoulu i ka maʻi a me ka nānā ʻana i ka pilina kino ma waena o ka hoʻolaha ʻana i ka lāʻau a me ka ʻūhū, eosinophilia, a me ke komo ʻana o ke kino. Me ka hoʻohana ʻana i kēia ʻike, hiki i nā kauka ke nānā i ka lāʻau e hoʻomaka ai i ka DRESS a hoʻōki i ka hoʻohana ʻana i kēlā lāʻau i ka manawa. Eia kekahi, hiki i nā kauka ke kuhikuhi i nā algorithm i hoʻohana ʻia e hoʻoholo i ka hopena o ka lāʻau no nā hopena ʻino ʻē aʻe.

ʻO ka lāʻau lapaʻau - glucocorticoids
ʻO nā glucocorticoids pūnaewele ka mea mua o ka hoʻoulu ʻana i ke kala ʻana o ka DRESS a me ka mālama ʻana i ka hoʻi hou ʻana. ʻOiai he 0.5 a hiki i ka 1 mg/d/kg ka helu hoʻomaka maʻamau i kēlā me kēia lā (ana ʻia i ka prednisone equivalent), aia ka nele o nā hoʻokolohua lapaʻau e loiloi ana i ka pono o nā corticosteroids no DRESS, a me nā haʻawina e pili ana i nā ʻano like ʻole a me nā regimens lapaʻau. ʻAʻole pono e hoʻemi ʻia ka nui o nā glucocorticoids a hiki i ka wā e ʻike ʻia ai ka hoʻomaikaʻi ʻana o ke kino, e like me ka hoʻēmi ʻana o ka ʻūhū, eosinophil penia, a me ka hoʻihoʻi ʻana i ka hana o ke kino. No ka hōʻemi ʻana i ka hopena o ka hoʻi hou ʻana, ua ʻōlelo ʻia e hoʻemi mālie i ka nui o nā glucocorticoids ma luna o 6 a 12 mau pule. Inā ʻaʻole hana ka maʻamau maʻamau, a laila hiki ke noʻonoʻo ʻia ka "shock" glucocorticoid therapy, 250 mg i kēlā me kēia lā (a i ʻole like) no 3 mau lā, a laila e hoʻemi iki ʻia.
No nā poʻe maʻi me ka ʻaʻahu haʻahaʻa, ʻoi aku ka maikaʻi o ka corticosteroid topical he koho lapaʻau kūpono. No ka laʻana, ʻo Uhara et al. Ua hōʻike ʻia he 10 mau maʻi DRESS i hoʻōla maikaʻi me ka ʻole o nā glucocorticoids systemic. Eia nō naʻe, no ka mea ʻaʻole maopopo i ka poʻe maʻi hiki ke pale palekana i ka mālama ʻōnaehana, ʻaʻole ʻōlelo ʻia ka hoʻohana nui ʻana o nā lāʻau lapaʻau ma ke ʻano he koho.

Hōʻalo i ka lāʻau glucocorticoid a me ka lāʻau lapaʻau
No nā poʻe maʻi DRESS, ʻoi aku ka nui o ka poʻe i pilikia i nā pilikia (e like me nā maʻi) mai ka hoʻohana ʻana i nā kiʻekiʻe kiʻekiʻe o nā corticosteroids, hiki ke noʻonoʻo ʻia nā lāʻau pale corticosteroid. ʻOiai ua loaʻa nā hōʻike e hiki ke hoʻohana pono i ka immunoglobulin intravenous (IVIG) i kekahi mau hihia, ua hōʻike ʻia kahi haʻawina ākea he nui ka hopena o ka lāʻau i nā hopena ʻino, ʻoi aku ka thromboembolism, e alakaʻi ana i nā maʻi he nui e hoʻololi i ka ʻōnaehana glucocorticoid therapy. Hiki ke pili ka pono o IVIG i kona hopena hoʻomaʻemaʻe antibody, e kōkua ana i ka pale ʻana i ka maʻi viral a i ʻole ka hoʻāla hou ʻana o ka maʻi. Eia nō naʻe, ma muli o ka nui o ka IVIG, ʻaʻole kūpono ia no nā poʻe maʻi me ka puʻuwai congestive, hōʻino ʻole ʻana, a i ʻole ka ate.
ʻO nā koho lapaʻau ʻē aʻe he mycophenolate, cyclosporin a me cyclophosphamide. Ma ka pale ʻana i ka hoʻoulu ʻana o ka cell T, cyclosporine blocks gene transcription o cytokines e like me interleukin-5, ma laila e hōʻemi ai i ka eosinophilic recruitment a me ka hoʻōla ʻana o ka cell T cell activation. ʻO kahi haʻawina e pili ana i ʻelima mau maʻi i mālama ʻia me ka cyclosporine a me nā maʻi 21 i mālama ʻia me nā glucocorticoids systemic i hōʻike ʻia ua pili ka hoʻohana ʻana i ka cyclosporine me nā haʻahaʻa haʻahaʻa o ka piʻi ʻana o ka maʻi, hoʻomaikaʻi ʻia nā ana lapaʻau a me nā keʻena hoʻokolohua, a me ka noho pōkole o ka haukapila. Eia naʻe, ʻaʻole i manaʻo ʻia ka cyclosporine i kēia manawa he lāʻau lapaʻau mua no DRESS. Hoʻohana nui ʻia ʻo Azathioprine a me mycophenolate no ka mālama mālama ʻana ma mua o ka induction therapy.
Ua hoʻohana ʻia nā antibodies monoclonal e mālama i ka DRESS. ʻO kēia me Mepolizumab, Ralizumab, a me benazumab e ālai ana i ka interleukin-5 a me kona axis receptor, Janus kinase inhibitors (e like me tofacitinib), a me nā anti-CD20 monoclonal antibodies (e like me rituximab). Ma waena o kēia mau lāʻau lapaʻau, ua manaʻo ʻia nā lāʻau anti-interleukin-5 ʻoi aku ka maʻalahi o ka maʻi induction. Pili paha ke ʻano o ka pono i ka piʻi mua ʻana o nā pae interleukin-5 i DRESS, ka mea maʻamau i hoʻoulu ʻia e nā pūnaewele T kūikawā. ʻO Interleukin-5 ka mea hoʻoponopono nui o nā eosinophils a nona ke kuleana no ka ulu ʻana, ka ʻokoʻa, ka hoʻoulu ʻana, ka hoʻoulu ʻana a me ke ola. Hoʻohana mau ʻia nā lāʻau anti-interleukin-5 no ka mālama ʻana i nā maʻi e loaʻa mau ana ka eosinophilia a i ʻole ka hana ʻino o ke kino ma hope o ka hoʻohana ʻana i nā glucocorticoids systemic.

Ka lōʻihi o ka mālama ʻana
Pono e hoʻoponopono pono ʻia ka mālama ʻana i ka DRESS e like me ka piʻi ʻana o ka maʻi a me ka pane lapaʻau. ʻO ka poʻe maʻi me DRESS e koi maʻamau i ka hale maʻi, a ma kahi o ka hapahā o kēia mau hihia e koi i ka mālama mālama koʻikoʻi. I ka wā o ka hale maʻi, loiloi ʻia nā hōʻailona o ka mea maʻi i kēlā me kēia lā, hana ʻia kahi hoʻokolohua kino piha, a nānā mau ʻia nā hōʻailona laboratory e loiloi i ke komo ʻana o ke kino a me nā loli i nā eosinophils.
Ma hope o ka hoʻokuʻu ʻia ʻana, pono ka loiloi ʻana i kēlā me kēia pule e nānā i nā loli o ke kūlana a hoʻoponopono i ka hoʻolālā lapaʻau i ka manawa. Hiki ke hoʻi hou i ka manawa i ka emi ʻana o ka dosis glucocorticoid a i ʻole ma hope o ke kala ʻia ʻana, a hiki ke hōʻike ʻia ma ke ʻano he hōʻailona hoʻokahi a i ʻole lesion organ kūloko, no laila pono nā mea maʻi e nānā lōʻihi a piha.