Dermatological Emergencies: What should be known

This is a presentation on Dermatological Emergencies.

1. DERMATOLOGICAL EMERGENCIES Sunil Timilsina 1 st year PG Resident GP and EM NAIHS 1

2. Outline Introduction Conditions resulting in dermatological emergencies References 1

3. Introduction Dermatological emergencies are those cutaneous conditions that can be life threatening if not diagnosed quickly and managed accordingly. Dermatological conditions with widespread systemic involvement and complication are the emergencies that have to be dealt with in ER. Prompt recognition for appropriate diagnosis and treatment is necessary to improve a patient’s prognosis and a single decision can make the difference between LIFE and DEATH. Multiple faculties involvement according to the emergency of the condition is required. 2

4. Conditions resulting in Dermatological Emergencies 1.Erythoderma 8. Bacterial Toxin Mediated Illnessess - Staphylococcal Scalded Skin Syndrome -Toxic Shock Syndrome 2. Anaphylaxis 9. Drugs induced Cutaneous Necrosis Warfarin/Heparin/Vasopressors 3. Severe Cutaneous Drug Reactions 10. Vesiculobullous disorders -Pemphigus Foliaceus 4. Psoriasis - Psoriatic Erythroderma - Acute generalized pustular Psoriasis 11. Vascular disorders 5. Leprosy Reactions 12. Acute Graft Vs Host Disease 6. Erythema Multiforme 13. Metabolic Disorders -Pellagra - Acrodermatitis Enteropathica 7. Infections 3

5. Erythroderma Erythroderma is an inflammatory skin disease with redness and scaling that affects 90% or more of the patients skin . Also known as Exfoliative Dermatitis Extensive skin exfoliation : 20-30g/day 18-20% mortality Males affected more (4x) 4

6. Presentation Initial lesions Erythema – rapidly progressing later scaling Established Case Erythema, Scaling and Induration involving >90% BSA Ectropion , Eclabium , Everted nostrils and flexion contracture of joints Later Exagerrated skin folds and shedding of hair, eyebrows, eyelashes and nostrils Lymphadenopathy Association : Extreme pruritus, Enteropathy,   Lesion of primary illness 5

7. Why an Emergency? Temperature Dysregulation Skin loss and hypothermia High Output Cardiac Failure Dehydration and Electrolyte imbalance Hypoalbuminemia Loss of muscle mass Thrombophlebitis/DVT Infection-both cutaneous and respiratory with pneumonia a major cause of death 6

8. Causes Diagnostic Clues Associated features Ichthyosis and related syndromes Present since or soon after birth. Adherent scales. May have positive family history Psoriasis Typical psoriasiform plaque Nail changes Pityriasis pilaris rubra Islands of Normal Skin. Salmon colored erythema Orthokeratosis , Parakeratosis Inflammatory disorders Atopic Dermatitis Fulfilling diagnostic criteria History of atopy Seborrheic Dermatitis Yellowish greasy scale involving seborrheic areas History of recurrent seborrheic dermatitis Airbrone Contact Dermatitis Prior history of contact dermatitis Causes 7

9. Causes Diagnostic Clues Associated features Pemphigus Foliaceous Superfical erosions with collarette of scales + ve Nikolsky sign Subcorneal split in HPE SSSS Skin tenderness + ve Nikolsky sign Norwegian Scabies Intense pruritus in background of immunocompromised states Mite under microscope Drug Hypersensitivity Syndrome Systemic involvement History of drug intake Sezary syndrome Leonine facies in long standing cases Sezary cells in PBS Dermatomyositis Heliotrop rash, Poikiloderma , Gottron’s papules, proximal muscle weakness Raised muscle enzymes Sarcoidosis Ichthyosiform erythroderma Sarcoidal granuloma in HPE Causes 8

10. Investigations CBC,RFT with electrolyte, LFT, blood culture, RBS Skin swab-microscopy and culture sensitivity Chest x-ray Skin biopsy-if cause uncertain 9

11. Reference: DOI: https ://doi.org/10.1016/j.mpmed.2017.04.005 10

12. 11 Condition Clinical picture Treatment Anaphylaxis Intense pruritus Flushing Stridor Hypotension Shock Adrenaline Respiratory Support Antihistaminic Steroids Utricaria Intense pruritus Localized wheals Triggerer avoidance Emollients Antihistaminic Leukotriene antagonists Steroids Immunotherapy Angioedema Swelling of face/lips/ peri orbital region

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14. Acute Generalized Pustular Psoriasis Occurs in patients with any variant of psoriasis Due to any provocating factors Most commonly after sudden withdrawal of topical or systemic steroids. 13

15. Why an Emergency? Severe hypoalbuminemia Hypocalcemia and tetany Oligemia and Acute tubular Necrosis Malabsorption Deep vein Thrombosis and Pulmonary Embolism 14

16. Clinical Presentation Generalized pustular eruptions with “lake of pus” formation Intense burning sensation, erythema Skin tenderness Fever Repeated crops of lesions May develop erythroderma 1

17. Investigations Basic laboratory profile Gram staining of smear from pustules  PMN cells without organism Skin biopsy 16

18. Management DICU admission General Measures Mild sedation Fluid and electrolytes High protein diet Systemic Therapy: Acitretin 1mg/kg/day to overcome acute stage and then 0.5 to 0.75mg/kg/day to control PUVA therapy Acetretin + PUVA Methotrexate IV/IM 0.3mg/kg/week High dose cyclosporine (9-12 mg/kg/day) Parenteral corticosteroids 17

19. Erythema Multiforme Acute, usually mild, self-limited cutaneous and/or mucocutaneous syndrome Types : EM major : mucous membrane involved EM minor : mucous membrane not involved Causes : Infection (mostly): HIV,HSV, Mycoplasma Drugs: Uncommon 18

20. Clinical features Rapid onset of lesions within few days, Mild pruritic or painful papular or urticarial lesion- Target lesion Oral mucosa : ulcers, erosion, difficulty swallowing Oesophageal stricture Eye-permanent eye damage Urethra-stricture and urinary retention Sepsis, cellulitis, permanent skin damage & scarring Inflammation of internal organs-lungs, liver 19

21. Investigation CBC, CRP, RFT with electrolytes, LFT with enzymes Biopsy : If diagnosis uncertain Swabs : HSV PCR Imaging : Chest X-ray PA view Serology : Mycoplasma (if respiratory symptoms present) 20

22. Treatment Treatment of underlying cause Withdrawl of any causative agent Symptomatic treatment-analgesics, antipyretics, topical steroids, antihistamine Xylocaine and diphenhydramine elixir for oral ulcers Consider Ophthalmology consultation if eyes involved Consider medicine consultation if systemic organ involvement Liquid diet I/V fluid therapy and electrolyte replacement Antibiotics if secondary infection suspected Tab. acyclovir 800mg 5 times a day for 7 days Consider systemic steroid use Admit for further treatment 21

23. Severe Cutaneous Drug Reactions High mortality Common in extremities of age Women more susceptible than men Includes: SJS/SJS-TEN overlap/TEN Drug Reaction with eosinophilia and systemic symptoms Serum Sickness Acute generalized Exanthematous Pustulosis 22

24. 23 Condition Mechanism Clinical Picture Treatment Remarks SJS/SJS-TEN/TEN Type IV hypersensitivity reaction Initial Prodrome Painful erythematous macules of targetoid appearance + ve Nikolsky sign Drug discontinuation Fluid Resuscitation Wound care Topical steroids IV Ig 1-2 mg/kg/day x 3-4 days SJS: <10 % BSA SJS-TEN: 10-30% BSA TEN: > 30%BSA SCORTEN scoring for severity DRESS Not fully understood Initial Prodrome Generalized  morbilliform  pruritic rash with  cephalo caudal  progression Systemic involvement Drug discontinuation Severity assessment Adequate Hydration Wound care Topical and systemic steroids Immunosuppresants 2 forms: Non serious - no organ involvement or stage 1 AKI or mild liver injury Serious - stage 2 AKI or severe liver injury or other system involvement

25. SJS TEN DRESS DRESS 24

26. 25 Condition Mechanism Clinical Picture Treatment Remarks Serum Sickness Type III hypersensitivity reaction Triad of Fever Arthralgia Skin rash Discontinuation of drug Antihistamines NSAIDS Systemic Steroids Occurs by 2 weeks after exposure to drugs Acute Generalized Exanthematous Pustulosis  T cell mediated neutrophilic inflammation rapid development dozens to hundreds nonfollicular , sterile, pinhead-sized pustules on a background of edematous erythema with flexural accentuation Drug withdrawal Supportive care Symptomatic treatment Pruritus and skin inflammation: Medium Potency topical steroid Desquamation phase: Emollients Resolves spontaneously in 1-2 weeks after stopping drug

27. 26 AGEP AGEP with flexural accentuation Serum Sickness

28. Drugs induced Cutaneous Necrosis Drug Mechanism Clinical Picture Why an Emergency? Management Warfarin  transient hypercoagulable state during initial   warfarin  administration that in turn leads to vascular occlusion and tissue infarction followed by extravasation of blood.   demarcated areas of purpura and necrosis  Starts within D1-D10 Symmetrical Extensive area of necrosis may lead to acute skin failure Discontinuation Heparin Vit . K IV FFP or Protein C concentrate transfusion Supportive Measures Heparin Antigen Antibody complex formation Incorrect injection technique Persistence on subcutaneous tissue Manifests within first two weeks Well demarcated erythema, edema, pain Bullae formation and necrosis Rapid progression Discontinuation Switch to non-heparin anticoagulant 4t score calculation and management 27

29. 28 Heparin Induced skin necrosis Necrosis along peripheral line by nor-adrenaline

30. Drugs induced Cutaneous Necrosis 29 Drug Mechanism Clinical Picture Why an Emergency? Management Vasopressor Excessive vasoconstriction with inadequate circulating volume Immunological Phenomenon Ecchymotic area which soon changes to black along the line of vasopressor infusion Extensive tissue loss Rapid progression Discontinuation Increasing preload Analgesics Topical nitroglycerine Inj. Phentolamine 5mg Surgical debridement

31. Metabolic Disorders Cause Pathophysiology Clinical Picture Management Acrodermatitis Enteropathica Intestinal absorption defect due to defective intestinal zinc transporter protein Autosomal Recessive  erythematous and vesiculobullous dermatitis Alopecia Ophthalmic manifestations Severe growth retardation Recurrent infections To measure fasting plasma zinc level < 60mcg/dl 3 mg/kg/day of elemental zinc (13.2 mg/kg/day of  zinc sulfate  or 10.1 mg/kg/day of  zinc acetate )  Lifelong zinc supplementation 30

32. Metabolic Disorders 31 Cause Pathophysiology Clinical Picture Management Pellagra Deficiency of Niacin  symmetric hyperpigmented rash, similar in color a distribution to a sunburn. present in the exposed areas of skin. " Casal necklace“ appearance Diarrhea Neuropsychiatric symptoms Food fortification Niacin supplementation Supportive care

33. Norwegian Scabies Seen in immunocompromised individuals begins with poorly defined, erythematous patches that quickly develop prominent scale   usually spreads inexorably and may eventually involve the entire integument Scales become warty, especially over bony prominences. Crusts and fissures appear. The lesions are malodorous. Nails are often thickened, discolored, and dystrophic . Pruritus may be minimal or absent. 32

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35. Why an Emergency? Associated fissures provides entry point for organism Secondary infection Septicemia Glomerulonephritis Aesthetic complications 34

36. Management United States Centers for Disease Control and Prevention's combination regimen for the treatment of crusted scabies: Permethrin  5% cream applied every two to three days for one to two weeks and Oral  ivermectin (200 mcg/kg/dose) given for three, five, or seven nonconsecutive days depending on severity of infestation Second generation antihistaminics for pruritus 35

37. Pemphigus Foliaceous Epidemiology Autoantigen C/F HPE DIF IIF ELISA Variants Middle aged adults Endemic form common in children and young adults Desmoglein 1 Fragile blisters Shallow erosions Erythematous crust Absent mucosal involvement Positive nikolsky sign Subcorneal or granular layer acantholysis Intercellular IgG deposition Circulating IgG DSG 1 autoantibodies Fogo sevalgem Senar Usher syndrome 36

38. Treatment 37 No Yes Rituximab therapy feasible? To start Prednisolone 0.5-1mg/kg/days And Rituximab To start Prednisolone 0.5-1mg/kg/days Add MMF or AZA Disease control achieved within 1 month?

39. 38 Patient treated with rituximab Disease control achieved within 1 month? Patient not treated with rituximab Increment of prednisolone upto 1.5mg/kg/day Increment of prednisolone upto 1.5mg/kg/day Switching immunosuppressant Rituximab to be considered Disease control achieved within 1 month? No MMF or AZA or treat as refractory PF Tapering prednisolone after achieving stable disease control If replase occurs: Rituximab administration if not given previously or last dose: 4-6 months MMF or AZA to be added if prednisolone or rituximab insufficient Switching immunosuppressant if already on it Yes Yes No No Ref: Joly P, et.al. Updated S2K guidelines on the management of Pemphigus Vulgaris and Pemphigus Foliaceous by EADV. J Eur Acad Dermatol Venerol 2020; 34:1900 Yes

40. Lepra Reactions   Type 1(reversal) Type 2(ENL) Lucio phenomenon Mechanism Delayed hypersensitivity possibly immune-complex deposition Cutaneous vasculitis probably due to immune-complex deposition Diseases Borderline(BT,BL) Lepromatous (BL,LL)   Cutaneous lesions Erythema, edema and scaling of previous lesions Appearance of –several tender, evanescent erythematous nodules on face, flexures, legs, which sometimes becomes pustular Large, sharply marginated , ulcerative lesions that appear in crops over the lower limbs and may recur episodically   Neuritis Common and severe Common and severe Not common 39

41. Lepra Reactions   Type 1(reversal) Type 2(ENL) Lucio phenomenon Systemic Manifestations Not common Fever, malaise, Arthralgia, tender lymphadenopathy, orchitis , acute uveitis, glomerulonephritis Not common Treatment Mild: NSAIDS Mod: NSAIDS and or Prednisolone Severe: NSAIDS and Prednisolone (for up-to 18 months depending upon severity) Mild: NSAIDS Mod: NSAIDS, Prednisolone   Severe: NSAIDS,Prednisolone , High dose Aspirin Supportive Prednisolone 40

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43. Bacterial Toxins Mediated Illness 42 Staphylococcal scalded Skin Syndrome Toxic Shock Syndrome Cause d/t exfoliative toxin A and B TSS-1 toxin and super antigens of Staph. Aureus (MRSA) GAS organism Skin involvement Yes Yes Mucous involvement No Yes Multisystem Involvement No Yes Prognosis Good Poor Treatment Good skin care Antibiotics (Iv preferred) Anti-Shock therapy Surgical debridement Antibiotic therapy

44. 43 SSSS STSS

45. 44 Condition Mechanism Clinical Picture Treatment Remarks Kawasaki Disease Multifactorial Fever Conjunctivits Skin rash Oral mucositis Polymorphous rash Cervical lymphadenopathy Erythema, induration & or desquamation of extremities IV IG Aspirin Steroids Supportive measures 6mo -5yrs commonly affected Boys more affected Purpura Fulminans Congenital or Acquired deficiency of Protein C/ protein S/ anti thrombin III Ecchymoses Areas of Skin necrosis FFP Protein C concentrate Anticoagulants 3 variants Idiopathic Neonatal Acute Infective

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47. 46 Purpura fulminans

48. Acute Graft Vs Host Disease due to inflammatory immune cell infiltrate involving T cells, macrophages, monocytes and neutrophil granulocytes with associated tissue destruction and apoptosis After allogeneic hematopoietic cell transplant (HCT) 47

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50. Clinical Picture maculopapular rash Blanchable erythema Initially nape of neck, face and extremities later becomes generalized Pruritic and or painful persistent nausea and/or emesis abdominal cramps with diarrhea (bloody) rising serum bilirubin concentration 49

51. Why an Emergency? Multisystem involvement Marked thrombocytopenia ~50% chance of mortality in severe form 50

52. Management Based on the severity General Measures plus Grade 1: Topical steroids Topical 0.1% Tacrolimus Oral Roxulitinib Gardes ≥2 : Systemic steroids Methylprednisolone 2mg/kg/day in divided doses Oral Roxulitinib 10mg BD 51 Resistant cases

53. Bacterial Infections Disease Clinical Feature Investigations Why an Emergency? Cellulitis poorly defined   lesion with induration  erythema, edema, warmth, tenderness Basic lab profile Rapid progression Septicemia NSSTI diffuse erythema, edema crepitus, skin necrosis, Ecchymosis signs of systemic toxicity. Basic lab profile CPK raised Deep tissue culture Rapid progression Limb Amputation Sepsis DIC Death 52

54. 53 Cellulitis NSSTI

55. 54 Bacterial Infections Disease Clinical Feature Investigations Why an Emergency? Abscess Painful, tender, and fluctuant red swelling May have surrounding erythema and induration Basic lab profile Rapid progression Septicemia

56. Signs of skin and soft tissue infection? Signs of necrotizing infection? Yes Emergency surgical debridement + Tissue culture and sensitivity Empiric IV antibiotics Daptomycin OR linezolid OR Vancomycin plus Carbapenam OR Piperacillin/ tazobactam , OR Fluoroquinolone+Metronidazole OR Ceftriaxone + Metronidazole No Non-purulent infection Cellultis Purulent infections Abscess Severity determination PE, labs ± Imaging TREATMENT 55

57. Severity determination PE, labs ± Imaging Mild infections Locally confined Oral Antibiotics: Cephalexin Flucloxacillin Dicloxacillin Clindamycin Incision and Drainage only Topical Antibiotics to be considered Moderate infection systemic involvement IV antibiotics Penicillin Ceftriaxone Cefazoline Clindamycin Incision and Drainage with Culture and sensitivity Oral/IV antibiotics: TMP/SMX Doxycycline Flucloxacillin Contd … 56

58. Severe infection Failed initial treatment SIRS Immunocompromised Features of necrosis IV antibiotics: Vancomycin PLUS Piperacillin/ tazobactam OR Meropenam Surgical Debridement + Culture and sensitivity Incision and drainage with culture and sensitivity IV antibiotics: Vancomycin Daptomycin Linezolid Telavancanin Ceftaroline Specific antibiotic therapy as per culture and sensitivity reports To evaluate and treat risk factors for recurrent infections Contd.. 57

59. 58 Condition Mechanism Clinical Picture Treatment Herpes Zoster Ophthalmicus  reactivation of VZV in the ophthalmic division of the trigeminal nerve  Prodrome Pain Vesicles formation Conjunctivitis/Uveitis/ Keratitis Rest and Hydration Acyclovir 800ng IV or PO 5 times/d x 1-3 weeks Cyclopegics Steroids Herpes Zoster Oticus  reactivation of VZV in the geniculate ganglion, affecting the seventh (facial) and eighth (vestibulocochlear) cranial nerves  Prodrome Vesicle s in EAC Ipsilateral CN VII paralysis Otalgia SNHL Vertiogo ± Rest and Hydration Acyclovir 800ng IV or PO 5 times/d x 1-3 weeks Prednisolone 1mg/kg/d 1-3 wks Amitryptilline for neuralgia Viral Infection

60. 59 Ramsay Hunt Syndrome Herpes zoster ophthalmicus

61. References Textbook of Adult Emergency Medicine-5 th edition-Peter Cameron Critical Care in Dermatology 2 nd edition – Jaypee Publications Uptodate 1

62. Thank You 1