Bullous Drug Eruption Staphylococcal Scalded Skin Syndrome (SSSS)
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Jordan Jamerson Evaluation of an acute Don’t be rash. bullous presentation Presentation: ❖ 68 yo M with significant skin rash ❖ Recently diagnosed with BLE cellulitis, started on bactrim and keflex ~2 weeks ago and completed them 2 days prior to presentation. ❖ Noted improvement in initial redness and tenderness. However, 3 days prior to presentation, he developed a red, nonpruritic, slightly tender rash on lower/upper extremities. Rash spread to include thighs, groin, abdomen, palms, soles, periocular. Denied oral, ocular, perianal involvement. ❖ Also reported recent cold ❖ PMH: Hepatitis C, glaucoma, h/o TBI (w/o h/o seizures) hypoglycemia, HTN, BPH ❖ Allergies: NKDA. Reports taking bactrim in past without reaction ❖ Medications: statin, tamsulosin, and a blood pressure medications. Recently stopped adderall. ❖ ROS: + subjective chills. ❖ Denies subjective fever, N, V, D, lightheadedness, loss of sensation, vision changes, chest pain, palpitations, dysuria. Physical exam ❖ Vitals: 99.7, HR 122, RR 20, BP 138/62, pulse ox 100% on RA, pain score 7. ❖ General: non-toxic, well appearing, NAD although appearing uncomfortable, AOx3 ❖ Cardiopulm: Tachycardic, breathing well on RA ❖ Skin: 1. Chest and UE: erythematous morbilliform, maculopapular eruption, appears to have progressed since initial photographs by primary team. No scale overlying with some pinpoint vesicular areas - no pustules noted 2. Feet and LE: diffuse erythema, mild edema. Upper calves bilaterally demonstrate patches of superficial bullae, easily sloughed with overlying morbilliform eruption 3. Erythematous morbilliform eruption extends up to legs to upper thighs and involved inguinal area 4. Patient declined examination of genital area 5. No sloughing or necrotic changes noted in oral mucosa, no erythema or conjunctivitis changes 6. Palms with erythematous macules, no distinct targeted or bull noted Terminology • PAPULE - Raised lesion less than 10 mm • MACULE - flat lesion. Some people use this term when the lesion is less than 10 mm and use PATCH when it is larger, while others use macule for all flat lesions. Macules can be any color: red, brown, white, etc. If you close your eyes and run your hand over the area and cannot feel it, it is likely a patch or macule. • PLAQUE - Raised lesion greater than 10 mm. • NODULE - Similar to papule or plaque, but deeper and more solid • WHEAL - Evanescent, pink, slightly elevated lesion due to edema. Also called URTICARIA or HIVES. Lesions will blanch (unlike purpura). • VESICLE - Elevated blister containing clear fluid, less than 10 mm. • BULLA (pl. BULLAE) - Elevated blister containing clear fluid, larger than10 mm. • PUSTULE - Same as vesicle, except that it contains pus instead of clear serous fluid • EROSION - Superficial defect in skin, not all the way through the epidermis. • ULCER - Defect or excavation of the skin, deeper than erosion; extends at least into the dermis. • MACULOPAPULAR - Combination of macules and papules. Usually used for non-specific drug rashes and viral exanthems. Usually used when there is no scale. (AKA morbilliform) Hand rash mnemonic ❖ MRS TECK/H ❖ Meningococcemia ❖ Rubella/RMSF ❖ Syphilis/SJS/(scabies) ❖ TSSS/TEN ❖ EM/Endocarditis ❖ Coxsackie ❖ Kawasaki’s ❖ Herpes (HSV, VZV) Labs/Imaging ❖ Hct 41.3 ❖ Glucose 83 ❖ AST 39 ❖ ESR 30 ❖ BUN 18 ❖ ALT 46 ❖ CRP 83.6 ❖ Cr 1.02 ❖ Total protein 7.6 ❖ WBC 19.7 ❖ Na 136 ❖ Albumin 3.8 ❖ N 84% ❖ K 4.6 ❖ P 4.0 ❖ L 8% ❖ Cl 105 ❖ Mg 2.3 ❖ M 5% ❖ CO2 22 ❖ troponin neg ❖ E 2% ❖ Ca 9.3 ❖ BNP 55 ❖ B 0% ❖ Tbili 0.6 ❖ LA 1.4 ❖ LE dopplers negative ❖ Alk Phos 116 ❖ Hgb: 14.1 for DVT Differential: ❖ SSSS ❖ Acute generalized exanthematous pustulosis (AGEP) ❖ Linear IgA dermatosis ❖ Toxic shock syndrome ❖ Drug hypersensitivity syndrome (DRESS) ❖ Bullous pemphigoid ❖ Pemphigus vulgaris ❖ Paraneoplastic pemphigus ❖ Dermatitis herpetiformis ❖ Sweet’s syndrome ❖ Acute graft-versus-host disease ❖ SJS/TEN ❖ EM ❖ Bullous drug eruption Staphylococcal scalded skin syndrome (SSSS) ❖ SA toxin mediated ❖ Usually newborns/infants/young children. M>F ❖ Can be seen in adults with impaired immune system or renal function. ❖ prodrome of F, sore throat, malaise, irritability, purulent rhinorrhea, conjunctivitis ❖ Facial erythema -> scarlatiniform eruption generalizes to body -> Flaccid bullae (24-48h) -> positive nikolsky -> exfoliated skin is superficial (subcorneal - toxins against desmoglein 1) ❖ Widespread ❖ Can see circumolar erythema and mild facial edema, lip fissuring, purulent conjunctivitis ❖ Mucous membrane and palms/soles are spared. ❖ Desquamation for up to 5 days ❖ No scarring following reepithelization ❖ Txt: antibiotics Differential: ❖ SSSS ❖ Acute generalized exanthematous pustulosis (AGEP) ❖ Linear IgA dermatosis ❖ Toxic shock syndrome ❖ Drug hypersensitivity syndrome (DRESS) ❖ Bullous pemphigoid ❖ Pemphigus vulgaris ❖ Paraneoplastic pemphigus ❖ Dermatitis herpetiformis ❖ Sweet’s syndrome ❖ Acute graft-versus-host disease ❖ SJS/TEN ❖ EM ❖ Bullous drug eruption Acute generalized exanthematous pustulosis (AGEP) ❖ Acute febrile pustular eruption ❖ high fever (102.2 degrees F) for about a week ❖ non-follicular pustules ❖ Following medications (primarily antibiotics: penicillins, macrolides; however, possible with multiple medications), mercury ingestion, viral infection. ❖ All ages ❖ Burning, pruritus ❖ Marked neutrophilia ❖ Nikolsky sign may be positive. ❖ Mucous membrane involvement with erosions of mouth/ tongue can appear. ❖ Facial edema occurs in 1/3 of patients. ❖ Spontaneous healing in 2 weeks followed by desquamation. ❖ Afebrile, no pustules. ❖ Symptomatic therapy, +/- topical mupirocin Differential: ❖ SSSS ❖ Acute generalized exanthematous pustulosis (AGEP) ❖ Linear IgA dermatosis ❖ Toxic shock syndrome ❖ Drug hypersensitivity syndrome (DRESS) ❖ Bullous pemphigoid ❖ Pemphigus vulgaris ❖ Paraneoplastic pemphigus ❖ Dermatitis herpetiformis ❖ Sweet’s syndrome ❖ Acute graft-versus-host disease ❖ SJS/TEN ❖ EM ❖ Bullous drug eruption Linear IgA Dermatosis ❖ Immune-mediated. ❖ Idiopathic or medication-induced ❖ Grouped or annular vesicles,or bullae on elbows, knees, buttocks (extensor surfaces). ❖ children: onset <5y and often perioral or perineal ❖ >4th decade ❖ May appear on edge of previous blister giving a “collarette” appearance. ❖ Tense blisters, unique histology -> DIF demonstrates linear IgA deposition at epidermal-dermal junction ❖ Favors extensor surfaces ❖ Certain medications: most commonly vancomycin ❖ others include phenytoin, amoxicillin, piroxicam, lithium, diclofenac, captopril, IL-2, IFN-gamma, and d-penicillamine ❖ In drug-induced reactions, mucosal involvement is less likely ❖ Burning, pruritus common complaints. ❖ Mucosal eruptions are common, can be severe, typically in idiopathic cases ❖ Txt: d/c medication. Dapsone, topical steroids, immunosuppressants Differential: ❖ SSSS ❖ Acute generalized exanthematous pustulosis (AGEP) ❖ Linear IgA dermatosis ❖ Toxic shock syndrome ❖ Drug hypersensitivity syndrome (DRESS) ❖ Bullous pemphigoid ❖ Pemphigus vulgaris ❖ Paraneoplastic pemphigus ❖ Dermatitis herpetiformis ❖ Sweet’s syndrome ❖ Acute graft-versus-host disease ❖ SJS/TEN ❖ EM ❖ Bullous drug eruption Toxic shock syndrome (TSS)* ❖ Exotoxin-mediated (GAS and SA) ❖ Superantigen -> massive nonspecific activation of T cells ❖ Range: mild to shock and end organ failure ❖ Erythema of palms and soles followed by desquamation in 1-3 weeks ❖ Erythema of mm (strawberry tongue and conjunctival hyperemia) ❖ SA: Diffuse scarlatiniform exanthem on the trunk -> extremities. Fever, hypotension. Tampons, surgical packing, abscesses, surgical mesh ❖ GAS: severe pain in extremity with or w/o underlying STI. Prodrome of fever, diarrhea, myalgia. Macular erythroderma not commonly seen. Often positive blood cultures. Mortality rates higher. Shock and multigrain failure occur 48-72 h after initial presentation. Risk factors include bites, lacerations, varicella infections. ❖ Both can demonstrate confusion, coma, renal impairment, liver impairment, ards, DIC ❖ supportive therapy, antibiotics Differential: ❖ SSSS ❖ Acute generalized exanthematous pustulosis (AGEP) ❖ Linear IgA dermatosis ❖ Toxic shock syndrome ❖ Drug hypersensitivity syndrome (DRESS) ❖ Bullous pemphigoid ❖ Pemphigus vulgaris ❖ Paraneoplastic pemphigus ❖ Dermatitis herpetiformis ❖ Sweet’s syndrome ❖ Acute graft-versus-host disease ❖ SJS/TEN ❖ EM ❖ Bullous drug eruption Drug Hypersensitivity syndrome (DRESS) ❖ Drug reaction with eosinophilia and systemic symptoms (fever, rash, internal organ involvement) ❖ - typically hepatitis, but can also effect kidneys and hematological system, and, more rarely, pneumonitis, pericarditis, or myocarditis ❖ Can also see pharyngitis, LAD, facial edema, nephritis ❖ Pink to dark red, symmetric morbilliform eruption +/- pustules ❖ Can also see urticarial plaques, atypical targeted lesions, vesicles, mucosal involvement ❖ Best classified as having TEN/SJS and treated accordingly ❖ Occur between 1-3 weeks after initiating new medication, but can be months later ❖ Commonly: anticonvulsants, sulfonamides, NSAIDS, minocycline, allopurinol, metronidazone, dapsone, nevirapine, clopidogrel, ticlopidine ❖ Skin biopsy: may show common drug eruption but may demonstrate keratinocyte necrosis (as seen in EM, SJS/TEN) ❖ stop medication, +/- systemic corticosteroids, supportive care ❖ No eosinophils or AST/ALT/Cr changes Differential: ❖ SSSS ❖ Acute generalized exanthematous pustulosis (AGEP) ❖ Linear IgA dermatosis ❖ Toxic shock syndrome ❖ Drug hypersensitivity syndrome (DRESS) ❖ Bullous pemphigoid