Erythroderma in adults
- Mark DP Davis, MD
Mark DP Davis, MD
- Professor of Dermatology
- Mayo Clinic College of Medicine
Erythroderma (literally, "red skin"), also sometimes called exfoliative dermatitis, is a severe and potentially life-threatening condition that presents with diffuse erythema and scaling involving all or most of the skin surface area (≥90 percent, in the most common definition). Erythroderma is the clinical presentation of a wide range of cutaneous and systemic diseases (including psoriasis and atopic dermatitis), drug hypersensitivity reactions, and more rarely Sézary syndrome, a leukemic subtype of cutaneous T-cell lymphoma. Although uncommon in pediatric patients, erythroderma may be the clinical presentation of a wide range of acquired and inherited diseases, including infections, inflammatory skin diseases, ichthyoses, and congenital immunodeficiencies.
This topic will discuss the clinical manifestations, diagnosis, and treatment of erythroderma in adults. Erythroderma in neonates and infants and Sézary syndrome are discussed separately. (See "Neonatal and infantile erythroderma" and "Clinical presentation, pathologic features, and diagnosis of Sézary syndrome".)
Erythroderma is a rare condition. The annual incidence has been estimated to be approximately 1 per 100,000 in the adult population . In a retrospective study, erythroderma accounted for 13 in 100,000 patients presenting with skin diseases in China . Erythroderma can occur at any age and in both sexes, but is more frequent in older adults (mean age 42 to 61 years) and in males [2-6]. Erythroderma is exceedingly rare in children; its prevalence is estimated to be approximately 0.1 percent in pediatric dermatology clinic populations [7,8].
Cutaneous and systemic conditions — A wide range of cutaneous or systemic diseases can evolve to or cause erythroderma (table 1).
●Exacerbation of a preexisting inflammatory dermatosis – The most common cause of erythroderma is the exacerbation of a preexisting inflammatory dermatosis, most often psoriasis or atopic dermatitis [3-6]. In patients with psoriasis, triggers of erythroderma include the abrupt discontinuation of systemic corticosteroids or other immunosuppressant therapy, systemic illnesses, phototherapy burns, medications (eg, lithium, antimalarials), or HIV infection .
- Sigurdsson V, Steegmans PH, van Vloten WA. The incidence of erythroderma: a survey among all dermatologists in The Netherlands. J Am Acad Dermatol 2001; 45:675.
- Li J, Zheng HY. Erythroderma: a clinical and prognostic study. Dermatology 2012; 225:154.
- Pal S, Haroon TS. Erythroderma: a clinico-etiologic study of 90 cases. Int J Dermatol 1998; 37:104.
- Akhyani M, Ghodsi ZS, Toosi S, Dabbaghian H. Erythroderma: a clinical study of 97 cases. BMC Dermatol 2005; 5:5.
- Rym BM, Mourad M, Bechir Z, et al. Erythroderma in adults: a report of 80 cases. Int J Dermatol 2005; 44:731.
- Sigurdsson V, Toonstra J, Hezemans-Boer M, van Vloten WA. Erythroderma. A clinical and follow-up study of 102 patients, with special emphasis on survival. J Am Acad Dermatol 1996; 35:53.
- Sarkar R, Garg VK. Erythroderma in children. Indian J Dermatol Venereol Leprol 2010; 76:341.
- Sarkar R, Basu S, Sharma RC. Neonatal and infantile erythrodermas. Arch Dermatol 2001; 137:822.
- Rothe MJ, Bernstein ML, Grant-Kels JM. Life-threatening erythroderma: diagnosing and treating the "red man". Clin Dermatol 2005; 23:206.
- Sheen YS, Chu CY, Wang SH, Tsai TF. Dapsone hypersensitivity syndrome in non-leprosy patients: a retrospective study of its incidence in a tertiary referral center in Taiwan. J Dermatolog Treat 2009; 20:340.
- Sigurdsson V, Toonstra J, van Vloten WA. Idiopathic erythroderma: a follow-up study of 28 patients. Dermatology 1997; 194:98.
- Thestrup-Pedersen K, Halkier-Sørensen L, Søgaard H, Zachariae H. The red man syndrome. Exfoliative dermatitis of unknown etiology: a description and follow-up of 38 patients. J Am Acad Dermatol 1988; 18:1307.
- Groves RW, Kapahi P, Barker JN, et al. Detection of circulating adhesion molecules in erythrodermic skin disease. J Am Acad Dermatol 1995; 32:32.
- Sigurdsson V, de Vries IJ, Toonstra J, et al. Expression of VCAM-1, ICAM-1, E-selectin, and P-selectin on endothelium in situ in patients with erythroderma, mycosis fungoides and atopic dermatitis. J Cutan Pathol 2000; 27:436.
- Sigurdsson V, Toonstra J, Bihari IC, et al. Interleukin 4 and interferon-gamma expression of the dermal infiltrate in patients with erythroderma and mycosis fungoides. An immuno-histochemical study. J Cutan Pathol 2000; 27:429.
- Kanthraj GR, Srinivas CR, Devi PU, et al. Quantitative estimation and recommendations for supplementation of protein lost through scaling in exfoliative dermatitis. Int J Dermatol 1999; 38:91.
- Green MS, Prystowsky JH, Cohen SR, et al. Infectious complications of erythrodermic psoriasis. J Am Acad Dermatol 1996; 34:911.
- Jaffe D, May LP, Sanchez M, Moy J. Staphylococcal sepsis in HIV antibody seropositive psoriasis patients. J Am Acad Dermatol 1991; 24:970.
- Bakri FG, Al-Hommos NA, Shehabi A, et al. Persistent bacteraemia due to methicillin-resistant Staphylococcus aureus with reduced susceptibility to vancomycin in a patient with erythrodermic psoriasis. Scand J Infect Dis 2007; 39:457.
- Talpur R, Bassett R, Duvic M. Prevalence and treatment of Staphylococcus aureus colonization in patients with mycosis fungoides and Sézary syndrome. Br J Dermatol 2008; 159:105.
- Garg G, Thami GP. Psoriasis Herpeticum due to Varicella Zoster Virus: A Kaposi's Varicelliform Eruption in Erythrodermic Psoriasis. Indian J Dermatol 2012; 57:213.
- Santmyire-Rosenberger BR, Nigra TP. Psoriasis herpeticum: three cases of Kaposi's varicelliform eruption in psoriasis. J Am Acad Dermatol 2005; 53:52.
- Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol 1980; 5:105.
- Klein A, Landthaler M, Karrer S. Pityriasis rubra pilaris: a review of diagnosis and treatment. Am J Clin Dermatol 2010; 11:157.
- Suzuki M, Oki T, Sugiyama T, et al. Muscarinic and alpha 1-adrenergic receptor binding characteristics of saw palmetto extract in rat lower urinary tract. Urology 2007; 69:1216.
- Bi MY, Curry JL, Christiano AM, et al. The spectrum of hair loss in patients with mycosis fungoides and Sézary syndrome. J Am Acad Dermatol 2011; 64:53.
- Ram-Wolff C, Martin-Garcia N, Bensussan A, et al. Histopathologic diagnosis of lymphomatous versus inflammatory erythroderma: a morphologic and phenotypic study on 47 skin biopsies. Am J Dermatopathol 2010; 32:755.
- Zip C, Murray S, Walsh NM. The specificity of histopathology in erythroderma. J Cutan Pathol 1993; 20:393.
- Vasconcellos C, Domingues PP, Aoki V, et al. Erythroderma: analysis of 247 cases. Rev Saude Publica 1995; 29:177.
- Çetinözman F, Jansen PM, Willemze R. Expression of programmed death-1 in skin biopsies of benign inflammatory vs. lymphomatous erythroderma. Br J Dermatol 2014; 171:499.
- Nagler AR, Samimi S, Schaffer A, et al. Peripheral blood findings in erythrodermic patients: importance for the differential diagnosis of Sézary syndrome. J Am Acad Dermatol 2012; 66:503.
- Rosenbach M, Hsu S, Korman NJ, et al. Treatment of erythrodermic psoriasis: from the medical board of the National Psoriasis Foundation. J Am Acad Dermatol 2010; 62:655.
- Viguier M, Pagès C, Aubin F, et al. Efficacy and safety of biologics in erythrodermic psoriasis: a multicentre, retrospective study. Br J Dermatol 2012; 167:417.
- Khaled A, Sellami A, Fazaa B, et al. Acquired erythroderma in adults: a clinical and prognostic study. J Eur Acad Dermatol Venereol 2010; 24:781.
- Egeberg A, Thyssen JP, Gislason GH, Skov L. Prognosis after Hospitalization for Erythroderma. Acta Derm Venereol 2016.
- Boyd AS, Menter A. Erythrodermic psoriasis. Precipitating factors, course, and prognosis in 50 patients. J Am Acad Dermatol 1989; 21:985.
- Kubica AW, Davis MD, Weaver AL, et al. Sézary syndrome: a study of 176 patients at Mayo Clinic. J Am Acad Dermatol 2012; 67:1189.
- Kim YH, Bishop K, Varghese A, Hoppe RT. Prognostic factors in erythrodermic mycosis fungoides and the Sézary syndrome. Arch Dermatol 1995; 131:1003.
- Cutaneous and systemic conditions
- CLINICAL MANIFESTATIONS
- Cutaneous signs and symptoms
- Extracutaneous findings
- Laboratory abnormalities
- CLINICAL COURSE
- Hemodynamic and metabolic disturbances
- DETERMINATION OF UNDERLYING CAUSE
- Physical examination
- Skin biopsy and histopathologic examination
- Laboratory and imaging tests
- Initial management
- Treatment of underlying conditions
- Management of patients with idiopathic erythroderma
- - Monitoring
- SUMMARY AND RECOMMENDATIONS