Erythroderma (Generalized Exfoliative Dermatitis) 

  • Author: Sanusi H Umar, MD, FAAD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 24, 2012
 

Background

Exfoliative dermatitis (ED) is a definitive term that refers to a scaling erythematous dermatitis involving 90% or more of the cutaneous surface. Exfoliative dermatitis is characterized by erythema and scaling involving the skin's surface and often obscures the primary lesions that are important clues to understanding the evolution of the disease. Clinicians are challenged to find the cause of exfoliative dermatitis by eliciting the history of illness prior to erythema and scaling, by probing with biopsies, and by performing blood studies. See the images below.

Exfoliative dermatitis diffuse skin involvement. Exfoliative dermatitis diffuse skin involvement. Exfoliative dermatitis close-up view showing erythExfoliative dermatitis close-up view showing erythema and scaling.

The term red man syndrome is reserved for idiopathic exfoliative dermatitis in which no primary cause can be found, despite serial examinations and tests. Idiopathic exfoliative dermatitis is characterized by marked palmoplantar keratoderma, dermatopathic lymphadenopathy, and a raised level of serum immunoglobulin E (IgE) and is more likely to persist than other types.

The term l'homme rouge refers to exfoliative dermatitis that is secondary to cutaneous T-cell lymphoma. The historic classification of exfoliative dermatitis into Wilson-Brocq (chronic process associated with exacerbation and remissions), Hebra or pityriasis rubra (relentlessly progressive disease), and Savill (self-limiting) types lacks any clinical significance.

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Pathophysiology

An increased skin blood perfusion occurs in exfoliative dermatitis that results in temperature dysregulation (resulting in heat loss and hypothermia) and possible high-output cardiac failure. The basal metabolic rate rises to compensate for the resultant heat loss. Fluid loss by transpiration is increased in proportion to the basal metabolic rate. The situation is similar to that observed in patients following burns (negative nitrogen balance characterized by edema, hypoalbuminemia, loss of muscle mass).

A marked loss of exfoliated scales occurs that may reach 20-30 g/d. This contributes to the hypoalbuminemia commonly observed in exfoliative dermatitis. Hypoalbuminemia results, in part, from decreased synthesis or increased metabolism of albumin. Edema is a frequent finding, probably resulting from fluid shift into the extracellular spaces. Immune responses may be altered, as evidenced by increased gamma-globulins, increased serum IgE in some cases, and CD4+ T-cell lymphocytopenia in the absence of HIV infection.

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Epidemiology

Mortality/Morbidity

Mortality varies according to the disease's cause. In a study of 91 of 102 patients with exfoliative dermatitis by Sigurdsson et al,[1] a mortality rate of 43% was observed. Only 18% of the deaths were directly related to exfoliative dermatitis. In 74% of the deaths, causes unrelated to exfoliative dermatitis were implicated.

Race

No racial predilection is reported for exfoliative dermatitis.

Sex

Male-to-female ratio is 2-4:1.

Age

Exfoliative dermatitis onset usually occurs in persons older than 40 years, except when the condition results from atopic dermatitis, seborrheic dermatitis, staphylococcal scalded skin syndrome, or a hereditary ichthyosis. Age of onset primarily is related to etiology.[2, 3]

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Contributor Information and Disclosures
Author

Sanusi H Umar, MD, FAAD  Attending Physician, Department of Dermatology, Charles R Drew/MLK Medical Center

Sanusi H Umar, MD, FAAD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

A Paul Kelly, MD  Chief, Clinical Professor, Department of Internal Medicine, Division of Dermatology, King/Drew Medical Center, Charles Drew University of Medicine and Science

A Paul Kelly, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society for Dermatologic Surgery, National Medical Association, and Pacific Dermatologic Association

Disclosure: Nothing to disclose.

Specialty Editor Board

James W Patterson, MD  Professor of Pathology and Dermatology, Director of Dermatopathology, University of Virginia Medical Center

James W Patterson, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Dermatopathology, Royal Society of Medicine, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD  Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology

Disclosure: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. 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. Jul 1996;35(1):53-7. [Medline].

  2. Sarkar R, Garg VK. Erythroderma in children. Indian J Dermatol Venereol Leprol. Jul-Aug 2010;76(4):341-7. [Medline].

  3. Fraitag S, Bodemer C. Neonatal erythroderma. Curr Opin Pediatr. Aug 2010;22(4):438-44. [Medline].

  4. Yuan XY, Guo JY, Dang YP, Qiao L, Liu W. Erythroderma: A clinical-etiological study of 82 cases. Eur J Dermatol. May-Jun 2010;20(3):373-7. [Medline].

  5. Byer RL, Bachur RG. Clinical deterioration among patients with fever and erythroderma. Pediatrics. Dec 2006;118(6):2450-60. [Medline].

  6. Griffiths TW, Stevens SR, Cooper KD. Acute erythroderma as an exclusion criterion for idiopathic CD4+ T lymphocytopenia. Arch Dermatol. Dec 1994;130(12):1530-3. [Medline].

  7. Scrivener Y, Cribier B, Le Coz C, Boehm N, Jelen G, Heid E, et al. [Erythroderma with immunoglobulin deposits along the basal membrane. Pemphigoid erythroderma?]. Ann Dermatol Venereol. Jan 1998;125(1):13-7. [Medline].

  8. Ram-Wolff C, Martin-Garcia N, Bensussan A, Bagot M, Ortonne N. Histopathologic diagnosis of lymphomatous versus inflammatory erythroderma: a morphologic and phenotypic study on 47 skin biopsies. Am J Dermatopathol. Dec 2010;32(8):755-63. [Medline].

  9. Zackheim HS, Kashani-Sabet M, Hwang ST. Low-dose methotrexate to treat erythrodermic cutaneous T-cell lymphoma: results in twenty-nine patients. J Am Acad Dermatol. Apr 1996;34(4):626-31. [Medline].

  10. Sigurdsson V, Toonstra J, van Vloten WA. Idiopathic erythroderma: a follow-up study of 28 patients. Dermatology. 1997;194(2):98-101. [Medline].

  11. Bruno TF, Grewal P. Erythroderma: a dermatologic emergency. CJEM. May 2009;11(3):244-6. [Medline].

  12. Burton JL, Holden WE. Lichenification and prurigo. In: Champion RH, ed. Textbook of Dermatology. 6th ed. London, England: Blackwell Science; 1998:673-8.

  13. Cohen LM, Skopicki DK, Harrist TJ. Non-infectious vesiculobullous and vesiculopostular diseases. In: Elenitsas R, ed. Lever's Histopathology of Skin. 8th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 1997:216.

  14. Freedberg IM. Exfoliative dermatitis. In: Freedburg IM, Fitzpatrick TB, Goldsmith LA, et al, eds. Fitzpatrick's Dermatology in General Medicine. 5th ed. New York, NY: McGraw-Hill; 1999:534-7.

  15. Nakamura M, Tokura Y. Tocilizumab-induced erythroderma. Eur J Dermatol. May-Jun 2009;19(3):273-4. [Medline].

  16. Prakash BV, Sirisha NL, Satyanarayana VV, Sridevi L, Ramachandra BV. Aetiopathological and clinical study of erythroderma. J Indian Med Assoc. Feb 2009;107(2):100, 102-3. [Medline].

  17. Sehgal VN, Srivastava G. Exfoliative dermatitis. A prospective study of 80 patients. Dermatologica. 1986;173(6):278-84. [Medline].

  18. Torres-Camacho P, Tirado-Sánchez A, Ponce-Olivera RM. A study of erythroderma: clues from eosinophilia and elevated lactate dehydrogenase levels. Indian J Dermatol Venereol Leprol. Sep-Oct 2008;74(5):499-500. [Medline].

  19. Wong KS, Wong SN, Tham SN, Giam YC. Generalised exfoliative dermatitis--a clinical study of 108 patients. Ann Acad Med Singapore. Oct 1988;17(4):520-3. [Medline].

 
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Exfoliative dermatitis diffuse skin involvement.
Exfoliative dermatitis close-up view showing erythema and scaling.
Table. Drugs Implicated in the Causation of Exfoliative Dermatitis
ACE inhibitorsAllopurinolAminoglutethimideAmiodaroneAmitriptyline
AmoxicillinAmpicillinArsenicAspirinAtropine
AuranofinAurothioglucoseBarbituratesBenactyzineBeta-blockers
Beta caroteneBumetanideBupropionButabarbitalButalbital
CaptoprilCarbamazepineCarbidopaChloroquineChlorpromazine
ChlorpropamideCimetidineCiprofloxacinClofazimineClofibrate
Co-trimoxazoleCromolynCytarabineDapsoneDemeclocycline
DesipramineDiazepamDiclofenacDiflunisalDiltiazem
DoxorubicinDoxycyclineEnalaprilEtodolacFenoprofen
FluconazoleFluphenazineFlurbiprofenFurosemideGemfibrozil
GoldGriseofulvinHydroxychloroquineImipramineIndomethacin
IsoniazidIsosorbideKetoconazoleKetoprofenKetorolac
LithiumMeclofenamateMefenamic AcidMeprobamateMethylphenidate
MinocyclineNalidixic AcidNaproxenNifedipineNitrofurantoin
NitroglycerinNizatidineNorfloxacinOmeprazolePenicillamine
PenicillinPentobarbitalPerphenazinePhenobarbitalPhenothiazines
PhenylbutazonePhenytoinPiroxicamPrimidoneProchlorperazine
PropranololPyrazolonesQuinaprilQuinidineQuinine
RetinoidsRifampinStreptomycinSulfadoxineSulfamethoxazole
SulfasalazineSulfisoxazoleSulfonamidesSulfonylureasSulindac
TetracyclineTobramycinTrazodoneTrifluoperazineTrimethoprim
VancomycinVerapamil
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