II. Definitions

  1. Erythroderma (Exfoliative Dermatitis)
    1. Serious to life-threatening dermatosis with generalized skin erythema and Scaling >80% surface area

III. Epidemiology

  1. Age over 40-50 years old most common
  2. More common in males

IV. Pathophysiology

  1. Generalized cutaneous vasodilation with inflammatory cell leakage into Dermis
  2. Generalized Edema and inflammation ensues
  3. Scaling follows inflammation after 5 days
  4. Rapid cell turnover results in frequent loss of cell contents including Proteins

V. Symptoms

  1. Diffuse Pruritus
  2. Constitutional symptoms (generalized weakness, malaise, chills)

VI. Signs

  1. Ill or toxic appearance
  2. Diffuse, generalized bright erythematous skin (typically including palms and soles)
  3. Diffuse Scaling of skin (within 5 days of onset)
  4. Onycholysis
  5. Alopecia

VII. Causes

  1. Preexisting dermatosis in 50% of cases (typically more gradual onset)
    1. Psoriasis
      1. Most common Erythroderma cause in adults (esp. with Medication Withdrawal)
      2. Central face is typically spared
      3. Pre-existing psoriatic Plaques may be obscured by diffuse exfoliation
        1. Plaques may also be replaced by sterile subcorneal Pustules
      4. Observe for nail changes (Onycholysis, Nail Pitting, subungual hyperkeratosis)
    2. Atopic Dermatitis (Eczema)
      1. Widespread erythematous dermatitis with intense Pruritus
      2. Lichenification with white scale in regions of chronic scratching
    3. Seborrheic Dermatitis
    4. Pityriasis rubra pilaris
      1. Salmon or orange-red colored skin lesions with variable Scaling and islands of sparing
      2. Follicular keratotic Papules on elbows and knees, as well as dorsal fingers
    5. Ichthyosis
    6. Lichen Planus
  2. Drug Reaction (rapid onset)
    1. See Life-Threatening Drug-Induced Rashes
    2. See Fixed Drug Eruption
    3. See DRESS Syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms)
    4. Typical onset as as Morbilliform or scarlatiniform rash
    5. Fever, Lymphadenopathy and hepatitis may be present
    6. Most common causes
      1. Antibiotics
      2. Seizure medications
      3. Cardiac medications
  3. Infection
    1. HIV Infection
    2. Toxic Shock Syndrome
    3. Norwegian crusted Scabies
    4. Staphylococcal Scalded Skin Syndrome
      1. Seen in infants and young children within 24 to 48 hours of Staphylococcus aureus infection
      2. Painful diffuse erythema, followed by flaccid subcorneal bullae that slough with raw, red exposed skin
      3. Spares mucous membranes
  4. Leukemia or Lymphoma
    1. Cutaneous T-Cell Lymphoma (esp. stage 4, Mycosis Fungoides)
  5. Other Causes
    1. Stevens-Johnson Syndrome (Erythema Multiforme Major)
    2. Toxic Epidermal Necrolysis

VIII. Management

  1. Consult regional burn unit
  2. Hospitalize all patients with suspected Erythroderma
  3. Treat underlying cause if identified (e.g. Toxic Shock Syndrome)
  4. Manage complications (similar to Burn Injury)
    1. Dehydration
    2. Electrolyte replacement
    3. Secondary infections
  5. Skin care - layered approach
    1. Layer 1: Low to moderate potency Corticosteroid (e.g. triamcinolone)
    2. Layer 2: Moist wrap or clothes
    3. Layer 3: Dry layer

IX. Complications

  1. Dehydration
  2. Metabolic abnormalities
  3. High output Heart Failure
  4. Secondary Skin Infections
  5. Hypoalbuminemia
  6. Peripheral Edema
  7. Anemia
  8. Temperature instability (Hypothermia, hyperthermia)

X. References

  1. Jhun, Grock and DeClerck in Herbert (2017) EM:Rap 17(3):18-9
  2. Fitzpatrick (1992) Color Atlas of Dermatology, p. 442-7
  3. Moon (2022) Am Fam Physician 105(1): 75-6 [PubMed]

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Ontology: Exfoliative dermatitis (C0011606)

Definition (NCI_CTCAE) A disorder characterized by generalized inflammatory erythema and exfoliation. The inflammatory process involves > 90% of the body surface area.
Definition (NCI) A generalized inflammatory cutaneous disorder characterized by erythema and desquamation.(NICHD)
Definition (MSH) The widespread involvement of the skin by a scaly, erythematous dermatitis occurring either as a secondary or reactive process to an underlying cutaneous disorder (e.g., atopic dermatitis, psoriasis, etc.), or as a primary or idiopathic disease. It is often associated with the loss of hair and nails, hyperkeratosis of the palms and soles, and pruritus. (From Dorland, 27th ed)
Definition (CSP) widespread involvement of the skin by a scaly, erythematous dermatitis occurring either as a secondary or reactive process to an underlying cutaneous disorder (e.g., atopic dermatitis, psoriasis, etc.), or as a primary or idiopathic disease; often associated with the loss of hair and nails, hyperkeratosis of the palms and soles, and pruritus.
Concepts Disease or Syndrome (T047)
MSH D003873
ICD10 L26 , L53.9
SnomedCT 200888009, 45491002, 55012006, 123702007, 399992009, 396349005, 396350005, 200948000, 400005007
English Dermatitis Exfoliativa, Erythroderma, Erythrodermas, Dermatitis, Exfoliative, Dermatitides, Exfoliative, Exfoliative Dermatitides, DERMATITIS EXFOLIATIVE, ERYTHRODERMA, Exfoliative dermatitis, exfoliative dermatitis, erythroderma (diagnosis), erythroderma, Dermatitis exfoliative NOS, Exfoliative dermatitis NOS, Dermatitis exfoliative generalised, Erythroderma NOS, Dermatitis, Exfoliative [Disease/Finding], Pityriasis rubra, erythrodermas, exfoliative erythroderma, dermatitis exfoliative, Hebra's pityriasis, Wilson-Brocq's disease, Erythematous eczema, Exfoliative Dermatitis, Dermatitis exfoliative, Dermatitis exfoliative generalized, Generalized erythroderma, Generalized exfoliative dermatitis, Pityriasis rubra of Hebra, Pityriasis rubra (Hebra), Erythrodermic eczema, Generalised exfoliative dermatitis, Erythroderma (disorder), Erythrodermic eczema (disorder), Generalised erythroderma, Generalized erythroderma (disorder), Generalized exfoliative dermatitis (disorder), Pityriasis rubra (Hebra) (disorder), Hebra; pityriasis, dermatitis; exfoliative, dermatosis; exfoliativa, exfoliativa; dermatosis, exfoliative; dermatitis, pityriasis; Hebra, Pityriasis Hebra, pityriasis rubra, Erythrodermatitis
French ERYTHRODERMIE, Dermite exfoliative généralisée, Dermatite exfoliatrice généralisée de Wilson-Brocq, Dermite exfoliatrice généralisée, Dermite exfoliatrice SAI, Dermite exfoliative SAI, Erythrodermie, Dermite exfoliative, DERMITE EXFOLIATRICE, Érythrodermie, Dermatite exfoliatrice, Dermatite exfoliative
Portuguese DERMATITE ESFOLIATIVA, ERITRODERMIA, Doença de Wilson Brocq, Eritrodermite, Dermatite exfoliativa NE, Dermatite esfoliativa generalizada, Dermatite exfoliativa generalizada, Dermatite exfoliativa, Dermatite Esfoliativa, Eritrodermia
Spanish DERMATITIS EXFOLIATIVA, ERITRODERMIA, Dermatitis exfoliativa NEOM, Enfermedad de Wilson-Brocq, Eritroderma, pitiriasis roja de Hebra, eccema eritematoso, dermatitis eccematosa exfoliativa, dermatitis exfoliativa generalizada, eccema exfoliativo, eczema eritematoso, dermatitis exfoliativa generalilzada (trastorno), dermatitis exfoliativa generalilzada, eccema eritrodérmico (trastorno), eccema eritrodérmico, eczema eritrodérmico, eritrodermia (trastorno), eritrodermia generalizada (trastorno), eritrodermia generalizada, eritrodermia, pitiriasis roja (de Hebra) (trastorno), pitiriasis roja (de Hebra), Dermatitis exfoliativa generalizada, Dermatitis exfoliativa, Dermatitis Exfoliativa, Eritrodermia
Italian Dermatite esfoliativa generalizzata, Malattia di Wilson-Brocq, Dermatite esfoliativa NAS, Eritroderma, Dermatite esfoliativa
Dutch exfoliatieve dermatitis NAO, erythroderma, ziekte van Wilson-Brocq, dermatitis exfoliatief gegeneraliseerd, Hebra; pityriasis, dermatitis; exfoliatief, dermatose; exfoliatief, exfoliatief; dermatitis, exfoliatief; dermatose, pityriasis; Hebra, Dermatitis exfoliativa, exfoliatieve dermatitis, gegeneraliseerde exfoliatieve dermatitis, Dermatitis, exfoliatieve, Erythrodermia, Erytrodermie, Exfoliatieve dermatitis
German exfoliative Dermatitis, generalisierte exfoliative Dermatitis, Dermatitis exfoliativ generalisiert, Dermatitis exfoliativa NNB, Erythrodermia, exfoliative Dermatitis NNB, Wilson-Brocq-Krankheit, DERMATITIS EXFOLIATIVA, ERYTHRODERMIE, Exfoliative Dermatitis, Dermatitis exfoliativa generalisiert, Dermatitis exfoliativa, Dermatitis, exfoliative, Erythrodermie
Japanese 全身性剥脱性皮膚炎, 剥脱性皮膚炎NOS, ウィルソン・ブロック病, ウィルソンブロックビョウ, コウヒショウ, ハクダツセイヒフエンNOS, ハクダツセイヒフエン, ゼンシンセイハクダツセイヒフエン, 皮膚炎-剥脱性, 紅皮症, 剥脱性皮膚炎, 剥脱性紅皮症, 紅皮症-剥脱性
Swedish Dermatit, exfoliativ
Czech dermatitida exfoliativní, erytrodermie, Generalizovaná exfoliativní dermatitida, Erytrodermie, Exfoliativní dermatitida, Exfoliativní dermatitida NOS, Wilson-Brocqova choroba
Finnish Kesivä ihottuma
Korean 탈락피부염
Polish Zapalenie skóry złuszczające, Erytrodermia
Hungarian Dermatitis exfoliativa, Erythroderma, Wilson-Brocq betegség, Dermatitis exfoliativ generalizált, Dermatitis exfoliativ k.m.n., Exfoliativ generalizált dermatitis, Exfoliativ dermatitis k.m.n., Exfoliativ dermatitis, Generalizált exfoliativ dermatitis, kiterjedt exfoliativ dermatitis
Norwegian Eksfoliativ dermatitt, Erythroderma