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erythroderma (exfoliative dermatitis)

Intense widespread (> 80-90%) reddening of the skin from dilated blood vessels, often associated with exfoliation. Etiology: 1) primary cutaneous disorders a) psoriasis, pustular psoriasis b) dermatitis 1] atopic dermatitis 2] stasis dermatitis 3] contact dermatitis 4] seborrheic dermatitis c) pityriasis rubra pilaris (chronic) [3] d) lichen planus 2) drug reactions a) type 1] hypersensitivity 2] acute generalized exanthamotous pustolosis 3] toxic epidermal necrolysis b) drugs 1] carbamazepine [6] 2] penicillins 3] barbiturates 4] gold salts 5] phenylbutazone 6] quinine 7] salicylates 8] sulfonamides - dapsone 9] systemic glucocorticoids (prednisone) - psoriasis can flare to erythroderma after systemic glucocorticoids [3] 3) infections/infestations a) Staphylococcal scalded skin syndrome b) toxic shock syndrome c) Tinea corporis d) scabies e) viral infection 4) autoimmune disease a) pemphigus foliaceus b) pemphigus vulgaris c) bullous pemphigoid d) linear IgA disease e) cutaneous lupus erythematosus f) dermatomyositis g) graph vs host disease (chronic) [3] h) psoriasis can flare to erythroderma after systemic glucocorticoids [3] 5) malignancy a) lymphoma - cutaneous T-cell lymphoma - mycosis fungoides (Sezary syndrome) b) paraneoplastic syndrome - squamous cell carcinoma of the lung (case report) [10] - prostate cancer [12] 6) other a) sarcoidosis b) graft vs host disease c) necrolytic migratory erythema d) idiopathic (25-47%) Pathology: - skin biopsy may show slight acanthosis & parakeratosis with a granulocytic infiltrate (case report) [10] Clinical manifestations: 1) widespread erythema from dilated blood vessels (> 80-90% of body surface) 2) erosions from severe pruritus 3) may be associated scales, pustules, exfoliation 4) may be papular, spare skin folds & face 4) alopecia, nail dystrophy, or thickening of the palms & soles are manifestations of a long-standing cause of erythroderma 3) potential systemic manifestations a) fever/chills b) hypothermia c) reactive lymphadenopathy (50%) d) peripheral edema e) high-output cardiac failure * images [8,9,10,12] Laboratory: - complete blood count a) eosinophilia (atopic dermatitis, hypersensitivity) b) atypical lymphocytosis (hypersensitivity) - peripheral blood smear: - Sezary cell in cutaneous T-cell lymphoma - serum chemistries a) abnormal liver function tests (hypersensitivity) b) renal function tests (hypersensitivity) - serum IgE elelvations favors atopic dermatitis - lymph node biopsy for suspected lymphoma - skin biopsy all patients - routine H&E staining - identifies cause in 50% of patients - confirms psoriasis - repeat biopsy may be needed [3] Complications: - infection - fluid & electrolyte imbalances - thermoregulatory disturbance - high output cardiac failure - acute respiratory distress syndrome [4] Management: - dermatologic urgency - treatment depends on etiology - discontinue offending medications - treat infection, malignancy - general measures - nutritional support - fluid & electrolyte replacement - gentle local skin care - oatmeal baths - wet dressings to weeping or crusted sites - followed with application of bland emollient & low-potency topical corticosteroid [4] - antihistamine for pruritus [3] - immunosuppression with systemic glucocorticoids & cyclosporine may suppress pruritus until tapered

Related

congenital nonbullous ichthyosiform erythroderma exfoliation (desquamation)

Specific

congenital ichthyosiform erythroderma

General

sign/symptom dermatitis

References

  1. Stedman's Medical Dictionary, 26th ed. Williams & Wilkins
  2. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 291
  3. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018.
  4. Rothe MJ, Bernstein ML, Grant-Kels JM. Life-threatening erythroderma: diagnosing and treating the "red man". Clin Dermatol. 2005 Mar-Apr;23(2):206-17. PMID: 15802214
  5. Bruno TF, Grewal P. Erythroderma: a dermatologic emergency. CJEM. 2009 May;11(3):244-6. PMID: 19523275
  6. Khaled A, Sellami A, Fazaa B et al Acquired erythroderma in adults: a clinical and prognostic study. J Eur Acad Dermatol Venereol. 2010 Jul;24(7):781-8 PMID: 20028449
  7. Okoduwa C, Lambert WC, Schwartz RA Erythroderma: review of a potentially life-threatening dermatosis. Indian J Dermatol. 2009;54(1):1-6 PMID: 20049259
  8. DermNet NZ: Erythroderma (images) http://www.dermnetnz.org/reactions/erythroderma.html
  9. Umar SH, Elston DM (images) Medscape: Erythroderma (Generalized Exfoliative Dermatitis) http://emedicine.medscape.com/article/1106906-overview
  10. Ampollini L, Rusca M. Erythroderma and a Pulmonary Nodule. N Engl J Med 2018; 379:e41 PMID: 30575466 https://www.nejm.org/doi/full/10.1056/NEJMicm1802572
  11. Mistry N, Gupta A, Alavi A, Sibbald RG. A review of the diagnosis and management of erythroderma (generalized red skin). Adv Skin Wound Care. 2015 May;28(5):228-36; quiz 237-8. Review. PMID: 2588266
  12. Li S, Yu X, Wang T Papuloerythroderma of Ofuji JAMA Dermatol. 2020;156(12):1365 PMID: 33052383 https://jamanetwork.com/journals/jamadermatology/fullarticle/10.1001/jamadermatol.2020.2973
  13. Inamadar AC, Ragunatha S. The rash that becomes an erythroderma. Clin Dermatol. 2019;37:88-98. PMID: 30981298