Disama dia Stevens-Johnson (SJS), Toxic Epidermal Necrolysis (TEN), ne Severe Drug Rashes¶

Tshipupu tshikunze anyi tshia violet tshidi tshisama tshidi tshilonda kudi kapia, bipumbishi bia mukana anyi ku mishiku, mêsu makunzubile, anyi kikoba tshidi tshipatuka padibu batshisukula ntshilumbu tshia luondapu tshidi mua kunyanga muoyo tshidibu babikila ne: Severe Cutaneous Adverse Reaction (SCAR). Bishilangane na tshipupu tshia antibiotique tshidi tshitamba kusama, Stevens-Johnson Syndrome (SJS) ne Toxic Epidermal Necrolysis (TEN) bidi bienza bua se: T-cells ya mubidi ikale ne lufu lua selile ya bungi mu tshitupa tshia pa mutu ne mu mucose, biumusha dikoba bu tshitupa tshibidi tshidi pambelu pa tshipupu. Tshilumbu tshia lukasa tshia matuku a bungi tshidi tshipetangana, Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS), tshidi tshikebesha disama dia mpala, ditangalaka dia mputa, kapia kakole, ne disama dia tshiadi, dia mfyo anyi dia mutshima mbingu 2 too ne ku 8 kunyima kua dibanga dia manga mapiamapia. Wewe anyi muntu mukuabu udi pabuipi nebe mupete disama dia nsukadi ne mputa ya mukana, mêsu makunze, bilonda anyi kapia kakole panyima pa wewe mumane kunua manga, udi mua kubikila nimero wa lupitadi lua muaba unudi diakamue ku diambuluisha dia nomba ya tshimpitshimpi. Tangila malu adi atangila diambuluisha dia lukasa lukasa mu kuosha bikole ne disukula dia bintu bia tshimpitshimpi, disama dia allergie ne anaphylaxie, ne sepsis ne septic shock.

Enza nunku mpindieu¶

1. LEKELA kunua manga adibu bela meji ne: adi ne bualu diakamue. Kuangatshi tshintu tshikuabu tshimue, tshiamu tshia nzembu, anyi dose wa manga a antibiotique adibu batuadije matuku mashale aa, manga a diluisha disama dia tshimpitshimpi, manga a gout, anyi manga a dijikija nawu kanyinganyinga (kusangisha ne ibuprofen anyi naproxen udibu bapanyisha ku tshisalu, adi mua kuikala tshintu tshidi tshikebesha anyi tshinyangu tshikole tshia tshifu). Kuimanyika manga mu meba 24 a kumpala a ntuadijilu wa bipupu kudi kukepesha njiwu ya lufu ne bia pa lukama 30.
2. Bikila nimero wa lupitadi lua muaba unudi anyi ndaku diakamue ku tshibambalu tshia malu a lukasa tshia lupitadi lunene (911 mu États-Unis ne Canada, 112 mu Union européenne mujima, 999 mu Royaume-Uni) pikala disama dia mputa dienda pamue ne tshimue tshia ku bintu ebi Six SCAR Red Flags:
3. Dikoba didi disama anyi diosha (dikoba didi disama padibu badilenga anyi badilengeja ne bilamba, pamutu pa kuikala anu disama).
4. Dibuela dia mubidi mu miaba ibidi anyi mipite apu: mishiku minyanguke, mipatuke mashi; bipeta bia kanyinganyinga munda mua mukana anyi mu nshingu; mesu makunze, a nsongo, a nsongo, anyi adi atamba kumona butoke; anyi mputa idi isama padibu basukula.
5. Mabala a violet anyi a tshitoke tshifiike adi kaayi alua matoke (blanch) padibu baafina.
6. Bipeta (bullae) anyi kikoba tshidi tshipatuka tshidi tshipatuka bu dibeji dia tshilamba tshitoke padibu batshikosa bitekete bitekete (tshimanyinu tshia Nikolsky tshimpe).
7. Kapia kakole pa mutu pa 38.5 degrés Celsius (101.3 degrés Fahrenheit) anyi masama a mubidi adi bu a grippe adi kumpala anyi adi alonda tshipupu.
8. Divula dia mpala (nangananga pabuipi ne mêsu ne matama) ne bitupa bia mashi bidi bivula mu nshingu, mu nshingu, anyi mu tshiadi.
9. Sangisha manga onso audi muangate mu mbingu 8 mipita (kusangisha ne mapilu adibu bapanyisha ku tshisalu, manga a bilongo, ne tulasa tua antibiotique tudibu bajikije) ne ufunde dituku dijalame dia ntuadijilu wa tshionso tshia kudibi. Bualu SJS/TEN utu utuadija 4 too ne ku 28 kunyima kua wewe mumane kutuadija manga, ne DRESS utu utuadija **8 to ne ku ntuadijilu kua lumingu luonso panyima pa dilua dia manga, tshikondo tshia kuya kudi munganga wa tshimpitshimpi ke mushindu mutambe buimpe wa kumanya tshidi tshikebesha disama.
10. Lama kikoba tshidi tshipatuka anyi tshidi ne mputa bu mudi tshioshelu tshikole ne kubenga kupatula mputa anyi kuela tshilamba tshia kulamakaja natshi to. Lungila tshilamba tshia sterile tshidi katshiyi tshilamakaja, tshikezuke, tshidi katshiyi tshilamakaja anyi tshilamba tshia coton tshiume tshikezuke tshiunyunguluke mubedi. Lomba baminganga ne baminganga ne: KAKUENA nansha kakese bua kuela bimanyinu bia ECG, tshilamba tshia IV, anyi biamu bia kulamakaja nabi mashi pa dikoba didi ne disama, bualu diumusha dia tshimanyinu nedipatule tshitupa tshijima tshia dikoba.
11. Ela diakamue mesu makunze anyi makole ne mâyi a mukele adi kaayi ne tshintu tshidi tshilama anyi ne binsonji bia tshianana bidi kabiyi ne bintu bidi bikuba mêsu bikalaku. Kuela nansha kakese mafuta a mêsu adi ajikija dikala dikunze anyi biamu bia kumona nabi mêsu mu mêsu adi ne disama dia SJS/TEN, bualu dinyanguka dia mucose munda mua bipese bia mêsu didi mua kusangisha tshitupa tshia mêsu tshidi tshienza bua mêsu ikale ne tshiamu tshia mitaku ne tshia kashidi (**syphary) dipanga dia mêsu mu meba 24 too ne ku 48 kakuyi diyukidilangana dia lukasa dia munganga wa mêsu.
12. Lama nzubu ne ambulanse bikale ne luya (28 too ne ku 32 degre Celsius, anyi 82 too ne 90 degre Fahrenheit). Anu bu bantu badi bafue ku kapia, babedi badi ne tshifu tshidi tshipatuka badi bajimija kapia ka bungi ka mubidi ne mâyi adi afumina mu luya ku diambuluisha dia tshikoba tshidi katshiyi tshilongolola, ne lukasa luonso bidi bifikisha ku disama dia hypothermie ne tshipupu tshia lufu.

Mutshi wa mapangadika: Disungula dia lukasa dia bipupu bia manga (SJS, TEN, DRESS, ne AGEP)¶

flowchart TD
    Start["Disama dipiadipia kunyima kua dibanga dia manga (Dituku 1 too ne mbingu 8 mimane kupita)"] --> PainMucosalCheck{"Tangila bimanyinu bikunze diakamue: Dikoba didi ne kanyinganyinga/diosha, dia violet mutoke, dia bipumbishi, dia mputa, NANSHA mishiku, mukana, mêsu, anyi bitupa bia mubidi bidi bisama anyi?"}

    PainMucosalCheck -->|"EYOWA: Mputa ya mu mukosa, bipumbishi, bipatshila bia mîdima, anyi dibutuka dia dikoba (Nikolsky +)"| SJSTENPath["SUSPECT SJS / TEN SPECTRUM: 1. LEKELA manga adi ne bualu diakamue. 2. Bikila 911 / 112 / 999 bua tshibambalu tshia malu a tshimpitshimpi ne tshia dituma dia mu nzubu wa luondapu. 3. ZERO tshilamba tshia kulamakaja natshi pa dikoba; nzubu wa luya too ne ku 30 C (86 F). 4. Diyukidilangana dia lukasa dia munganga wa mêsu mu meba 24."]

    PainMucosalCheck -->|"KAKUENA bipumbishi anyi dinyanguka dia mubidi to"| FacialOrganCheck{"Tangila bimanyinu bikunze: disama dia mpala, kapia kakole (>38.5 C / 101.3 F), tuishi tua mashi tuvula, mashi mafiike, jaunisse, anyi ntuadijilu mbingu 2 too ne ku 8 kunyima kua manga?"}

    FacialOrganCheck -->|"EYOWA: Divula dia mpala + Kapia + Tujilu tua mashi (Mbingu 2 too ne ku 8 ya manga)"| DRESSPath["MUSHINDU WA KUVALA (DIHS): 1. LEKELA diakamue manga adi mabi. 2. Ndaku ku tshibambalu tshia malu a tshimpitshimpi bua Stat CBC ne dishilangana (eosinophils / atypical lymphocytes), enzymes ya mu tshiadi (AST/ALT), Creatinine, & Troponin. 3. Bidi bikengela manga a corticosteroïdes a bungi."]

    FacialOrganCheck -->|"KAKUENA disama dia mpala anyi bimanyinu bia bitupa bia mubidi to"| PustuleCheck{"Kudiku nkama ya tusoso tukese tudi katuyi ne tuishi tudi katuyi ne tuishi tudi tutoka tudi pa dikoba dikunze tudi tumueneka mu mêba 24 too ne ku 72 kunyima kua dienza dia antibiotique anyi?"}

    PustuleCheck -->|"EYOWA: Bipupu bia ku mutu + Kapia (<Matuku 3 kunyima kua manga)"| AGEPPath["BUALU BUA BUALU BUA BUALU BUA BUALU (AGEP): 1. Lekela manga a antibiotique/diltiazem/hydroxychloroquine. 2. Dikonkonona dia lukasa dia tshibambalu tshia malu a mpukapuka bua kumbusha disama dia pustule ne kutangila calcium/neutrophils."]

    PustuleCheck -->|"TSHIENA: Anu mabala a maculopapular adi ne mputa ya rose, malu a mubidi adi kaayi makane, kakuyi mputa ya mukana anyi ya mêsu, kakuyi disama dia dikoba"| SimpleExanthem["BUALU BUA KUENZA MUDIMU WA MORBILLIFORM: 1. Tangila munganga udi ukufundila manga lelu kumpala kua kuangata dose udi ulonda. ."]

Pathophysiology: Mushindu kayi udi T-cells imbula tshitupa tshia ku mutu mu SJS/TEN ne dibunda dia bitupa bia mubidi mu DRESS¶

Mushindu udi mubidi wa muntu udikuba ku masama a SJS ne TEN (Type IVc cytotoxic apoptosis)¶

Disama dia Stevens-Johnson (SJS) ne Toxic Epidermal Necrolysis (TEN) ki mmasama abidi mashilangane to; I bikondo bia bukole mu tshitupa tshimue, tshia disama didi ditungunuka didi dimanyishibua kudi tshitupa tshia Total Body Surface Area (TBSA) mudi tshitupa tshia mubidi tshidi tshipatuka anyi tshidi mua kupatuka: - Stevens-Johnson Syndrome (SJS): Ditapuluka dia tshitupa tshia mubidi didi dikuata muinshi mua 10 pa lukama wa bunene bua mubidi mujima (\(<10\%\text{ TBSA}\)). Bungi bua lufu budi pabuipi ne 5 too ne ku 10 pa lukama. - SJS / TEN Overlap: Epidermal detachment involving 10 to 30 percent of Total Body Surface Area (\(10\%\text{ to }30\%\text{ TBSA}\)). Bungi bua lufu budi pabuipi ne 20 too ne ku 25 pa lukama. - Toxic Epidermal Necrolysis (TEN, also called Lyell Syndrome): Epidermal detachment involving more than 30 percent of Total Body Surface Area (\(>30\%\text{ TBSA}\)). Bungi bua lufu budi bufika ku 30 too ne ku 50 pa lukama.

Mu SJS/TEN, manga a molecule mukese anyi tshintu tshidi tshienza mudimu tshidi tshienza mudimu buludiludi ne Human Leukocyte Antigen (HLA) proteine ​​wa mulongo wa I udi pa mutu pa keratinocytes (tshia dikoba ne tshia mubidi) ne tshia T-cell receptors. Dîba didi divulangana dia CD8+ cytotoxic T-lymphocytes ne Natural Killer (NK) cells didi dienzeka (didi diangata 4 too ne ku 28 matuku pa dimueneka dia kumpala), selile ya dikuba dia mubidi eyi idi ibuela mu tshitupa tshia kuinshi kua dikoba ne ipatula bingoma bisatu bia lufu: 1. Granulysin (proteine wa tshinyangu udi mu bungi bunene munda mua mayi a SJS/TEN). 2. Perforin ne Granzyme B (bidi bienza mabulu mu membrane ya keratinocyte ne bienza bua intracellular caspase enzymes ikale ne mudimu). 3. Fas Ligand (FasL / CD95L) udi ulamakaja ku biamu bia lufu pa keratinocytes.

Mu ma nsa makese, midiyo ya keratinocytes ya mu tshitupa tshia mubidi idi yenza apoptose (lufu lua selile mulongolola) ne necroptose. Bu mudi tshitupa tshijima tshia kuinshi kua dikoba tshifua musangu umue, tshitupa tshia pa mutu tshia dikoba (tshiadi tshia pa mutu) tshidi tshitapuluka tshishiki ne tshitupa tshia kuinshi tshia dikoba muaba udi dikoba ne dikoba dipetangana. Serum udi ubuela mu muaba eu udi kayi ne tshintu bua kuenza bipumbishi bidi kabiyi ne bukole, ne bukole bukese bua dikosa dia ku luseke budi buenza bua mabeji onso a tshitupa tshia mubidi tshidi tshifue apueke, ashiya tshitupa tshia mubidi tshidi katshiyi tshilengeja, tshidi tshidila, tshidi tshipatuka mashi tshipatuka mu lupepele.

Bua tshinyi SJS/TEN udi unyanga mêsu, njila wa luya, njila wa tshifu, ne bitupa bia lulelu¶

Bualu bua milongo ya epitelium squamous idi kayiyi anu itangila dikoba dia pambelu to, kadi idi itangila kabidi bitupa bia mubidi bidi ne mâyi, selile ya CD8+ T idi ne bukole bua kushipa mubidi idi iluisha mubidi wa munda ne wa pambelu musangu umue mu bantu bapite pa 90 pa lukama badi ne disama dia SJS/TEN: - Mucosa wa mêsu (80 pa lukama wa babedi): Dinyanguka dia tshitupa tshia mêsu ne tshia tshitupa tshia mêsu didi dibutula selile ya goblet ne ya limbal stem cells. Bipese bia fibrin bidi bilamakaja tshitupa tshia munda tshia tshiadi tshia dîsu buludiludi ku tshitupa tshia dîsu (symblepharon), bikudimuna mishiku munda bua kutapa tshitupa tshia dîsu (trichiasis), ne bibutula njila ya binsonji, bienza bua tshitupa tshia dîsu tshikale tshifofo tshia matuku onso padibu kabayi batshiondopa mu mêba 24 too ne ku 48 a kumpala. - Mucosa wa mukana, wa mu tshiadi, ne wa mu tshifu (90+ pa lukama): Bintu bifiike bidi bipatuka mashi bidi bibuikila mishiku (muelelu wa vermilion), eku bipupu bidi bikolesha munda mua mukana, ludimi ne tshifu bienza bua se: muntu kayi mua kumina mâyi to. - Tracheobronchial Tree (Respiratory Tract, 25 percent): Dinyanguka dia tshitupa tshia bronchial tshidi tshikebesha tshifu, dipanga dia mupuya, dibutuka dia tshitupa tshia bronchial tshifue, ne dipanga dia mupuya dikole didi dilomba dienza dia kapepe. - Urogenital Tract (60 to 70 percent): Dinyanguka dia kanyinganyinga ka urethra, glans penis, vulva, ne vagina didi difikisha ku dilama dia urine ne dipanga dia kashidi dia urethra anyi vaginal strictures (adhesions) pikalabu kabayi bawuela mafuta ne batapulula dituku dionso.

Mushindu udi mubidi wa muntu udikuba ku disama dia DRESS (Type IVb eosinophilic multiorgan hypersensitivity)¶

Mu Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS), udibu babikila kabidi ne: Drug-Induced Hypersensitivity Syndrome (DIHS), diandamuna dia T-cell didi dienda dikola didi dilomba matuku a bungi bua kukola (2 too ne ku 8 lumingu kunyima kua dibanga dia manga) ne didi dienza bua **Th2 T-cell ** idi yambuluisha bua kupatula bungi bua bungi bua Interleukin-5 (IL-5). - IL-5 udi usaka eosinophils mu mifuba ne mu mashi, pamue ne dijadikulula dia herpesvirus ya bantu, nangananga Human Herpesvirus-6 (HHV-6), HHV-7, Epstein-Barr Virus (EBV), ne Cytomegalovirus (CMV). - Pamutu pa kumbusha tshitupa tshia pa mutu mu mabeji, eosinophiles ne T-cells eyi idi ibuela mu bitupa bia munda: - Tshifu (tshidi tshinyanguka mu 70 too ne ku 80 pa lukama tshia bilumbu bia DRESS): Tshidi tshikebesha disama dia hepatite cytolytique anyi cholestatique didi mua kuya kumpala too ne ku dipanga dia tshifu didi dilomba dipanda dia tshifu dia lukasa. - Mifuba (idi minyanguka mu 15 too ne ku 30 pa lukama): Ifila disama dia nsukadi ne dipanga dia mifuba. - Mapunga (adi anyanguka mu 15 pa lukama): Adi akebesha disama dia pneumonite eosinophilique. - Mutshima (mushipianganyi udi ne njiwu ya bungi mu DRESS): Udi ukebesha myocardite eosinophilique anyi giant-cell necrotizing, udi mua kufikisha ku disama dia tshimpitshimpi dia lufu anyi dia cardiogenic shock mbingu mikese panyima pa disama dia nsukadi dimane kutuadija.

Difuanyikija dia malu a luondapu dia bipeta binayi bia manga ne disama dia manga didi kadiyi dikole¶

Maluvu adi ne njiwu mikole ne tshikebelu tshia HLA tshia pharmacogenomic¶

Malu mapite pa 80 pa lukama a SJS/TEN ne DRESS adi afumina ku kasumbu kakese ka manga adi ne njiwu mikole. Dîba dionso diudi utangila mubedi udi ne disama dia mputa anyi kapia + mputa, keba diakamue mishindu ya manga eyi:

1. Maluvu adi ambuluisha bua kujikija tunyinganyinga (malu adi ambuluisha bua kujikija tshiji ne adi akolesha lungenyi)¶

• Lamotrigine (Lamictal): Umue wa ku malu adi atamba kufikisha ku SJS/TEN kudi bansonga ne kudi babedi ba mu lungenyi/ba disama dia tshiadi pa buloba bujima. Njiwu ya SJS/TEN ne lamotrigine idi ivula bikole pikala:
• Dose wa ku ntuadijilu udi ubandishibua lukasa (upita ndongamu wa dipima dia bitekete bitekete ku mbingu ibidi).
• Lamotrigine udi musangisha ne valproic acid / divalproex (Depakote), udi ukandika glucuronidation ne udi uvudija tshitupa tshia matuku a lamotrigine mu mashi misangu ibidi.
• Mubedi udi ulekela manga a lamotrigine matuku mapite pa 5 ne pashishe udi utuadija kabidi ne manga ende onso a kumpala pamutu pa kuenza kabidi manga a bungi ku ntuadijilu.
• Carbamazepine (Tegretol) ne Oxcarbazepine (Trileptal): Bidi bipetangana bikole ne SJS/TEN mu babedi badi ne HLA-B*15:02 allele (bitamba kuikala mu Han Chinese, Thai, Philippines, Malaysian, Vietnamese, ne bena Asia wa ku Sud) ne bidi bipetangana ne SJS/TEN mu babedi badi ne DRES/STEN HLA-A*31:01 allele (udibu bapeta mu matunga a ku Mputu, a mu Japon ne a pa buloba bujima).
• Phenytoin (Dilantin), Fosphenytoin, ne Phenobarbital: Bintu bia mupuya muimpe bidi ne njiwu mikole bidi ne cross-reactivity ne carbamazepine. (Mukenji wa luondapu: Shi mubedi mupeta SJS/TEN nansha DRESS ku carbamazepine, phenytoin, nansha phenobarbital, udi ne tshia kuepuka manga onso adi ne mupuya muimpe bualu cross-reactivity udi upita 40 too ne ku 60 pa lukama; manga makuabu adi kaayi ne mupuya muimpe bu mudi levetiracetam anyi valproate adibu benza nawu mudimu kudi baminganga ba mitu).

2. Buanga bua tshipupu: Allopurinol¶

• Allopurinol (Zyloprim): Tshintu tshimuepele tshidi tshikebesha SJS/TEN ne DRESS kudi bantu bakulumpe pa buloba bujima ne ku Mputu ne ku Azia.
• Bidi bipetangana bikole ne HLA-B*58:01 allele (bitamba kuikala mu Han Chinese, Corée, Thai, ne bena Afrike-Amerika) ne bidi bienda bikola bua disama dia tshiadi didi dienda dikola, didi difikisha ku disangisha dia tshintu tshidi tshienza mudimu tshia allopurinol, oxypurinol.

3. Sulfonamide Antibiotics ne Anti-Infectives¶

• Sulfamethoxazole-Trimethoprim (TMP-SMX / Bactrim / Septra): Tshintu tshia kumpala tshidi tshijula SJS/TEN pa buloba bujima.
• Sulfasalazine (udibu benza nende mudimu bua disama dia rheumatoid arthritis ne ulcerative colitis) ne Dapsone (udi ukebesha disama dia DRESS dia kale didibu babikila ne: Dapsone Hypersensitivity Syndrome, didi dipetangana ne HLA-B*13:01). (Note: Non-arylamine sulfonamides bu mudi furosemide anyi thiazide diuretics rarely cross-react to cause SJS/TEN).
• Nevirapine (luondapu lua diluisha disama dia nsukadi), Abacavir (ludi lufikisha ku disama dia hypersensitivity syndrome didi dipangisha kudi HLA-B*57:01 dikonkonona dia malu a mubidi kumpala kua difunda dia manga), Vancomycin (tshintu tshinene tshidi tshikebesha DRESS Syndrome ne Liarmatosis), Dermatosis Minocycline (tshintu tshidi tshijula DRESS mu bansonga ne bakulumpe badibu bondopa bua mputa).

4. Oxicam NSAIDs ne Non-Drug Infectious Triggers (Mycoplasma)¶

• Oxicam NSAIDs (Piroxicam, Meloxicam, Tenoxicam) idi ne njiwu mikole ya SJS/TEN munkatshi mua manga adi ajikija kanyinganyinga. Buteyi bua mushinga (budi buleja): Bu mudi SJS/TEN utuadija misangu yonso ne tshipupu tshia matuku 1 too ne ku 3 tshia disama dia luya, disama dia mu mikolo, ne mêsu adi asama kumpala kua disama dia nsukadi kupatuka, babedi ba bungi batu bangata **ibuprofen anyi acopétaminol) udibu bapana ku tshisalu tshia kumpala bua kuondopa tshipupu tshia prodromal. Padi bipupu bipatuka kunyima kua meba 24, ibuprofen anyi acetaminophen uvuabu bangate malaba batu misangu ya bungi bafila tshilumbu tshia dishima padi tshilema tshilelela tshivua lamotrigine, allopurinol anyi sulfa antibiotic uvuabu batuadije matuku 18 kumpala.
• Mycoplasma pneumoniae ne Herpes Simplex Virus (HSV): Nangananga kudi bana ne bansonga, disama dia pneumoniae dia pa tshibidilu didi difumina ku Mycoplasma pneumoniae didi mua kufikisha ku dinyanguka dia mukana, dia mêsu ne dia mu bitupa bia lulelu ne mputa mikese ya dikoba (**Mycoplasma-Induced Rash ne Mucosi [RMMI) Erythema Multiforme Major.

Dikonkonona dia mubidi: Mushindu kayi wa kumanya SJS/TEN pa kumbusha Erythema Multiforme ne Staphylococcal Scalded Skin¶

1. Bilonda bia kale bia 3-Ring Target vs. Atypical 2-Ring Dusky Targets¶

Mu disama dia Erythema Multiforme (EM Minor/Major) (didi difumina ku disama dia mashika / Herpes Simplex Virus pamutu pa manga), mputa idi mibandile, milenga, mputa ya 3-zone "bullseye" target lesions (tshitupa tshifiike tshia munkatshi, tshijengu tshia munkatshi tshia rose tshitoke, ne tshijengu tshikunze tshitangalake) pa maboko, maboko, maboko, ne maulu (diabanya dia acral).

Mu Stevens-Johnson Syndrome ne TEN, mputa idi mibalakane (makula), kayiyi milongolola, kayiyi mimanyike bimpe "bipatshila bidi kabiyi bia pa tshibidilu" ne anu bitupa 2: tshitupa tshia violet tshifiike, tshia gris dusky, anyi tshia munda tshidi ne bilonda bia lufu tshidi tshinyunguluka kudi muelelu mutoke wa macula. Bidi bituadija ku mpala, nshingu, tshiadi, ne tshitupa tshia mubidi, bidi biumvuika bibi menemene padibu babilenga, ne bidi bibuela lukasa mu mabeji manene a dikoba dia violet.

2. Kuteta tshimanyishilu tshia Nikolsky ne tshia Asboe-Hansen mu bukubi¶

• Direct Nikolsky Sign: Padi munganga wenza mudimu ne tshianza tshinene tshidi ne tshianza pa dikoba didi ne tshipupu, tshitupa tshia pa mutu tshia dikoba tshidi tshinyanguka ne tshikosa diakamue pa dikoba didi muinshi mua dikoba bu dibeji dia tshilamba tshiume.
• Tshimanyinu tshia Asboe-Hansen (Tshimanyinu tshia Nikolsky / Bulla-Spread tshidi katshiyi tshinyanguke): Kufina bitekete bitekete pa mutu pa tshipupu tshidi katshiyi tshinyanguke kudi kuenzeja mâyi adi munda bua kupatuka ku luseke, ne kualabaja tshitupa tshia tshimpumpu pambelu mu dikoba didi pabuipi bualu bintu bidi bitshinyunguluke bidi bimane kufua.
• (Mukenji wa diambuluisha dia kumpala: Paudi umona dikoba dia mpukapuka anyi muaba umue udi muimpe, lekela kuela dikoba, bualu mateta adi kaayi ne mushinga adi anyanga dikoba didi dikuba dikoba).

3. Dishilangana dia SJS/TEN ne Staphylococcal Scalded Skin Syndrome (SSSS) mu bana¶

Mu bana batyetye ne bana batyetye badi ne luya, dikoba dikunze, ne dikoba didi dipatuka ne tshimanyinu tshia Nikolsky, ba munganga badi ne tshia kusunguluja SJS/TEN ne Staphylococcal Scalded Skin Syndrome (SSSS) (difumina ku toxins A ne B idi ifumina ku Staphylococcus): - Mukenji wa dikoba: Mu Staphylococcal Scalded Skin Syndrome (SSSS), toxin udi ukosolola desmoglein-1 muulu mu tshitupa tshia dikoba, tshidi katshiyi mu mucosa; nunku, munda mua mukana, mêsu, ne tshitupa tshia mubidi tshia masandi bidi 100 pa lukama SPARED mu SSSS (nansha mudi dikoba dia pabuipi ne mukana dienzeka). Mu SJS/TEN, ditapuluka didi dienzeka muinshi mua tshitupa tshia kuinshi, didi difikisha ku bipumbishi bia bungi bia mashi munda mua mukana, mu mêsu ne mu bitupa bia lulelu.

Dibala dia malu adi mua kuenzeka mu tshibambalu tshia malu a mpukapuka: tshipiminu tshia SCORTEN¶

Padibu bafika mu lupitadi (Dituku dia 1) ne kabidi mu Dituku dia 3, baminganga ba disama dia kapia ne dia bantu badi ne disama dikole badi babala SCORTEN (Score of Toxic Epidermal Necrolysis) bua kumanyisha lufu ne kulombola dituma dia lukasa mu nzubu wa luondapu wa pa buawu wa disama dia kapia. Tshintu tshimue tshidi tshipeshibua bua tshionso tshia ku bintu 7 bidi mua kuikala ne njiwu**:

SCORTEN Mortality Interpretation & Triage Mandate: - 0 to 1 points: pabuipi ne 3.2 pa lukama bavua bamanyishe lufu. - 2 points: approximately 12.1 percent predicted mortality (Transfer to Burn Unit / ICU obligated for any score \(\ge 2\) or any detachment \(\ge 10\%\text{ TBSA}\)). - 3 points: pabuipi ne 35.3 pa lukama bavua bamanyishe lufu. - 4 points: pabuipi ne 58.3 pa lukama bavua bamanyishe lufu. - \(\ge 5\) points: \(> 90\text{ percent}\) predicted mortality.

Mikenji ya dilombola dia malu a tshimpitshimpi ya lupitadi ne ya Burn Center¶

1. Dilengeja dia mayi (Mushindu wa kuosha, ki mmushindu wa Parkland mujima to)¶

Dishilangana dinene dia mubidi didi ditapulula SJS/TEN ne kapia ka kapia ka tshitupa tshisatu: bualu SJS/TEN udi unyanga anu tshitupa tshitekete tshia mubidi kayi ukolesha mishipa ya muinshi mua dikoba, edema wa tshitupa tshisatu udi mukese kupita wa kapia ka kapia, ne udi wenza mudimu bikole ne tshiamu tshia kapia tshijima tshia Parkland (\(4x mL/kg/\%TBSA}\)) udi ukebesha disama dia lufu lua mu mikolo. - UK BAD ne Burn Center Guideline: Kufila tshiamu tshia isotonic crystalloid (Lactated Ringer's anyi Plasmalyte) mu mishipa ne tshiamu tshia **Modified SJS/TEN Resuscitation Formula tshia \(2\text{ mL/kg/\%TBSA}\) tshia dikoba didi dipatuke mu mêba 24 a kumpala, bua kulama dipatuka dia mashi dia \(0.5\text{ to }1.0\text{ mL/kg/dîba}\).

2. Dilama dia mputa: Dilamba dia bintu bidi ne muoyo ne dilama dia mputa didi kadiyi dikosa¶

• Lekela tshitupa tshia mubidi tshidi tshitapuluke pa muaba watshi bu tshilamba tshia mubidi: Bishilangane ne bipupu bia kapia mudibu bumbusha eschar mu kapia, mu SJS/TEN tshitupa tshia mubidi tshidi tshipatuke tshia tshitupa tshia mubidi tshidi katshiyi ne disama. Muinshi mua British Association of Dermatologists (BAD) mikenji ya malu a kale, bipumbishi bidi kabiyi binyanguke bidi mua kupatuka ku nshindamenu yabi eku bashiya tshisasa tshia dikoba tshitekete pa mutu pa dikoba bua kuenza mudimu bu tshintu tshidi tshikuba mubidi tshidi tshikepesha kanyinganyinga, tshipangisha dijimija dia mâyi, ne tshikolesha dipingana dia tshipupu.
• Ela 50:50 wa parafine mutoke / mafuta a parafine mu mayi pa dikoba dionso ne pa mishiku kunyima kua mêba 2 anyi 4, mubuikila ne tshilamba tshia silicone tshidi katshiyi tshilamakaja (bu mudi Mepitel) anyi tshilamba tshia nanocrystalline tshidi ne arjan (Acticoat) tshidi tshishala muaba watshi dituku dionso kakuyi dishintulula dia tshilamba tshia dituku dionso.
• Zero Prophylactic Systemic Antibiotics: Kokufila nansha kakese manga a prophylactic intravenous antibiotics mu SJS/TEN; antibiotiques idi mua kujula tshienzedi tshibidi tshia manga ne kusungula Pseudomonas anyi Staphylococcus aureus sepsis udi kayi mua kuitaba manga a bungi (sepsis ke tshintu tshia kumpala tshidi tshikebesha lufu mu TEN). Ela bilonda bia pa dikoba kunyima kua meba 48 ne utuadije manga a antibiotique IV anu padiku bimanyinu bia disama (dipueka dia tshimpitshimpi dia luya lua mubidi, dibandila dia CRP/procalcitonin, anyi dipeta dia mashi mimpe).

3. Dididishi dia mêba 24 dia diondopangana dia mêsu bua kuepula bufofo bua kashidi¶

Mubedi yense wa SJS/TEN udi ne tshia kuikala mukonkonona ku luseke lua bulalu kudi munganga wa mêsu mu meba 24 a dibuela mu lupitadi: - Ela mafuta a lubricant adi kaayi ne tshintu tshidi tshilama ne mafuta a corticosteroïde adi kaayi ne tshintu tshidi tshilama kunyima kua mêba 1 anyi 2 bua kupangisha dibutuka dia selile ya T ya mu mêsu. - Enza mudimu wa dilengeja dia mâyi a mukele ne dilengeja dia tshiamu tshia glass (anyi bimpa, diteka dia tshiamu tshia amniotic Membrane Transplant [AMT] / Prokera ring ku luseke lua bulalu mu mêba 72 a kumpala a ntuadijilu wa bimanyinu). Mateta a mu lupitadi adi aleja ne: kuela tshiamu tshia amniotic pa mutu pa mêsu ne pa mêsu a munda mu tshikondo tshia disama didi dipangisha symblepharon scarring ne dilama dimona dia 20/20.

4. Dishintulula dia mubidi mu SJS/TEN vs. DRESS¶

• Mu SJS/TEN: Nansha mudi Intravenous Immunoglobulin (IVIG) nkayende muleje dikuatshisha dikese mu meta-analyses ya lelu, nzubu ya pa buayi idi yenza mudimu ne Cyclosporine (\(3\text{ to }5\text{ mg/kg/day}\)) (udi ukandika buludiludi granulysin release) anyi TNF-alpha inhibitors (Etanercept \(50\text{ mg}\) subcutaneous injection), udi mateta a tshimpitshimpi aleja ne udi ukolesha dikoba dia lukasa ne ukepesha lufu padibu bamupesha mu matuku makese kunyima kua dibanga dia bipupu.
• Mu disama dia DRESS: Bishilangane ne SJS/TEN, malu adibu babikila ne: corticosteroïdes adi ne mushinga wa bungi bua kusungila muoyo bua DRESS Syndrome (\(1\text{ too ne ku }2\text{ mg/kg/day}\) wa prednisone wa ku mishiku anyi wa methylprednisolone wa mu mishiku). Mukenji wa DRESS: Steroïdes mu DRESS udi ne tshia kuikala mupueka bitekete bitekete mu mbingu 8 too ne ku 12+; kuimanyika manga a steroïdes kunyima kua matuku 5 anyi 10 a tshimpitshimpi kudi mua kufikisha ku disama dia hepatite, dia myocardite anyi dia CMV/HHV-6 dia tshipupu tshikole, misangu ya bungi tshidi mua kushipa muntu.

Nomba ya tshimpitshimpi ya mu tshijengu ne mikenji ya bibambalu¶

• États-Unis ne Canada: Ela 911. Muinshi mua American Burn Association (ABA) mikenji ya dituma, babedi badi ne SJS/TEN idi itangila \(>10\%\text{ TBSA}\) anyi disama dia mucosal/airway dia mushinga mukole badi bakumbaja mikenji ya dilomba bua kubatuma diakamue ku ABA-Verified Regional Burn Center. Umanyishe bipeta bibi bia manga kudi US FDA MedWatch program anyi Health Canada Vigilance Program.
• Bukalenge bua ditunga: Ela 999 (anyi 112). Londa mikenji ya British Association of Dermatologists (BAD) ne NICE bua SJS/TEN kudi bantu bakole ne bana, idi ilomba diambuluisha dia lukasa dia ICU/Burn Center, dibala dia SCORTEN, ne difila dia luapolo ku diambuluisha dia MHRA Yellow Card Scheme.
• Buobumue bua matunga a ku Mputu: Ela 112. Bibambalu bia European Reference Network for Rare Skin Disorders (ERN-Skin) ne RegiSCAR bidi bilombola dilama dia pa buadi dia disama dia dikoba mu matunga adi mu UE.
• Australie ne Nouvelle-Zélande: Ela 000 mu Australie anyi 111 mu Nouvelle-Zélande. Tuma bilumbu bikola ku Adult or Pediatric Burn Service ya mu ditunga dijima ne funda mamanyisha a kashidi a manga mu mukanda wa malu a bukola bua mubidi wa ditunga.
• India ne Asia-Pacifique: Ela 112 mu Inde, 119 mu Japon ne mu Corée du Sud, anyi 995 mu Singapour. Bua bungi bua HLA-B*15:02 (carbamazepine/phenytoin) ne HLA-B*58:01 (allopurinol) alleles mu Asie wa ku Sud ne wa ku Est, manyisha baminganga ba lupitadi diakamue bua manga onso adi ambuluisha bua kujikija tshipupu anyi a gout adibu banua mu matuku 60 adi mapite.

Tangila nomba ya tshimpitshimpi bua nomba yonso ya dituma dia njiwu ya pa buloba bujima.

Nkonko itubu bela misangu ya bungi¶

Mmunyi mundi mua kumanya dishilangana pankatshi pa disama dia tshiadi tshidi katshiyi ne njiwu ne disama dia Stevens-Johnson?¶

Disama dia maculopapular dia manga didi kadiyi ne njiwu didi ne mabala a rose adi alua matoke padibu baafina, adi asama kakese, kaayi asama padibu baalenga, ne kaayi alenga mukana, mêsu anyi bitupa bia lulelu to. Disama dia Stevens-Johnson dia kumpala didi difikisha ku dikoba didi dikola anyi diosha padibu badilenga, mabala a violet anyi a mu tshimfuanyi tshia tshintu tshidi katshiyi tshitoka padibu baafina, kapepe kakole, ne mputa mikole idi minyanguke ku mishiku, ku mishiku, ku mêsu anyi ku bitupa bia lulelu; tshimue tshia ku bimanyinu ebi tshidi tshikengela kuya diakamue mu nzubu wa lupitadi lua lukasa.

Bua tshinyi disama dianyi dia lamotrigine, allopurinol, anyi Bactrim diakatuadija mbingu isatu kunyima kua meme mumane kutuadija kunua manga?¶

Disama dia Stevens-Johnson, Toxic Epidermal Necrolysis, ne DRESS Syndrome i disama dia Type IV didi dienda dikola dia T-cell pamutu pa disama dia anticorps dia lukasa. Paudi upeta disama dia kumpala, bidi bilomba tshiluilu tshiebe tshia mubidi matuku 7 too ne ku 28 (ne too ne ku mbingu 8 mu DRESS) bua kuvudija selile ya T idi ne buanga bua kutuadija dibunda didi dimueneka pa dikoba diebe ne pa bitupa biebe bia mubidi.

Bua tshinyi bidi ne njiwu bua kuela bilamba bia kulamakaja nabi, tshilamba tshia luondapu anyi bimanyinu bia ECG pa muntu udi ne SJS anyi TEN?¶

Mu SJS ne TEN, tshitupa tshia dikoba tshidi tshilamakaja tshitupa tshia pa mutu tshia dikoba ku dikoba dia kuinshi tshidi tshifua nansha pa bitupa bia dikoba dikunze bidi kabiyi bianji kupatuka bipumbishi (tshimanyinu tshimpe tshia Nikolsky). Kufina tshilamba tshia kulamakaja natshi, tshiamu tshia IV, anyi tshiamu tshia kulamakaja natshi muoyo pa dikoba adi ne pashishe kutshikoka kudi kupatula tshitupa tshijima tshia dikoba, kuenza mputa wa kapia udi mua kufikisha ku lufu lua sepsis.

Kunua ibuprofen anyi acetaminophen bua disama dia kapia kudi mua kufikisha ku disama dia Stevens-Johnson anyi?¶

Nansha mudi SJS udi kayi umueneka bikole udi ufumina ku NSAID (nangananga ne NSAID ya oxicam), misangu ya bungi idibu banua ibuprofen anyi acetaminophen matuku 1 anyi 2 kumpala kua bipumbishi kumueneka bidi bileja dipanga dia lungenyi. Bu mudi SJS/TEN utuadija ne matuku 1 anyi 3 a kapia, disama dia mu mikolo, ne disama dia mubidi kumpala kua bipumbishi kupatuka, babedi badi banua manga adi akepesha kapia bua bimanyinu bia kumpala bia SJS; baminganga badi ne tshia kutangila panyima mbingu 1 anyi 4 bua kupeta manga malelela adi ne bualu.

Bua tshinyi babedi ba SJS ne ba TEN badi ne tshia kuondopibua mu nzubu wa lupitadi wa Burn Unit pikalabu kabayi baoshibue kudi kapia?¶

Padi 10 too ne ku 90 pa lukama ya tshitupa tshia pambelu tshia mubidi wa mubedi tshipatuka mu SJS/TEN, mubedi udi ujimija mudimu wa dikuba dia dikoba dia muomumue ne wa muntu udibu bashipe bikole: udi ujimija litre ya mâyi adi ne proteyine ya bungi ku diambuluisha dia dikoba didi kadiyi dilengeja, kena mua kukokesha luya lua mubidi to, udi umvua kanyinganyinga kakole padibu bashintulula bilamba, ne udi upeta njiwu mikole ya disama dia mashi adi afua, udi ukengela Burn Dilama dia ICU.

Meme mupanduke ku SJS/TEN ku carbamazepine anyi phenytoin, manga kayi makuabu a disama dia tshiadi adi ne njiwu kundi?¶

Carbamazepine, oxcarbazepine, phenytoin, fosphenytoin, phenobarbital, ne primidone badi ne tshimuenekelu tshia benzene-ring tshia mupuya muimpe, ne lamotrigine udi ne njiwu ya dienza dia T-cell. Bantu batue ku tshitupa tshia babedi badi ne SJS/TEN anyi DRESS ku diambuluisha dia manga kampanda a mupuya muimpe nebapete disama dikole padibu babapesha manga makuabu mu kasumbu aku, nunku udi ne bua kuepuka bua kashidi manga onso a mupuya muimpe.

Malu adi apetangana¶

• Kuosha bikole ne disukula dia bintu bia tshimpitshimpi
• Disama dia allergie ne anaphylaxie
• Sepsis ne septic shock
• Disama dia mêsu ne diambuluisha dia lukasa

Miaba ne buludiki bua mbulamatadi¶

• British Association of Dermatologists (BAD, United Kingdom) and National Institute for Health and Care Excellence (NICE): UK Guidelines for the Management of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis in Adults and Children (Creamer et al. / McPherson et al. SCORTEN triage, modified \(2\text{ mL/kg/\%TBSA}\) fluid resuscitation, conservative biological dressing care, and 24-hour ophthalmology protocols).
• American Burn Association (ABA, United States): Burn Center Referral Criteria ne Clinical Practice Guidelines for Non-Thermal Exfoliative Skin Diseases (SJS/TEN).
• European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR Consortium) and European Academy of Dermatology and Venereology (EADV): Diagnostic Criteria and Management Guidelines for SJS/TEN, DRESS Syndrome (Kardaun RegiSCAR Score), and AGEP.
• United States Food and Drug Administration (FDA) and Clinical Pharmacogenetics Implementation Consortium (CPIC): Pharmacogenomic Boxed Warnings and Guidelines for HLA-B*15:02, HLA-A*31:01 (Carbamazepine/Phenytoin), ne HLA-B185: Dikonkonona*.
• Tshilongelu tshia bena Amerike tshia malu a mêsu (AAO, États-Unis): Dilama dia mêsu dia lukasa ne dishintulula dia tshitupa tshia munda tshia difu mu disama dia Stevens-Johnson ne dinyanguka dia tshitupa tshia mubidi tshidi ne buanga.