Erythroderma (Generalized Exfoliative Dermatitis) Clinical Presentation

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

History

History is the most important aid in diagnosing exfoliative dermatitis (ED).[4]

  • Patients may have a history of the primary disease (eg, psoriasis, atopic dermatitis).
  • Elicit a comprehensive drug history, including over-the-counter drugs.
  • Disease usually evolves rapidly when it results from drug allergens, lymphoma, leukemia, or staphylococcal scalded skin syndrome.
  • Disease evolution is more gradual when it results from psoriasis, atopic dermatitis, or the spread of primary disease.
  • Pruritus is a prominent and frequent symptom.
  • Malaise, fever, and chills may occur.
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Physical

  • Patients often present with generalized erythema.
  • Scaling appears 2-6 days after the onset of erythema, usually starting from flexural areas.
  • Pruritus commonly results in excoriations.
  • When exfoliative dermatitis persists for weeks, hair may shed; nails may become ridged and thickened and also may shed.
  • Periorbital skin may be inflamed and edematous, resulting in ectropion (with consequent epiphora).
  • In chronic cases, pigmentary disturbances can occur (especially in darker-skinned races); patchy or widespread loss of pigment (resembling vitiligo) has been reported.
  • Diligent search for residual signs of underlying disease occasionally yields dividends. Residual signs may include the following:
    • Islands of sparing in pityriasis rubra pilaris
    • Few typical psoriatic plaques in psoriasis
    • Papules or oral lesions of lichen planus
    • Superficial blisters of pemphigus foliaceus
    • Erythematous papular lesions of an early drug eruption
  • Dermatopathic lymphadenopathy can occur in exfoliative dermatitis not caused by lymphoma or leukemia. A lymph node biopsy is advised when lymph nodes exhibit lymphomatous characteristics (eg, large size, rubbery consistency) and the cause of exfoliative dermatitis is undetermined.
  • The general picture is modified according to the nature of the underlying disease and the patient's general physical condition.
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Causes

Determining specific etiologies in exfoliative dermatitis often is not possible; however, it is necessary to attempt since etiology may impact disease course and management options. The list of conditions that can cause exfoliative dermatitis is extensive and continues to expand. Cutaneous diseases that cause exfoliative dermatitis and the systemic diseases associated with them include the following:

  • Atopic dermatitis - Acute and chronic leukemia
  • Contact dermatitis - Reticulum cell sarcoma
  • Dermatophytosis - Carcinoma of rectum
  • Hailey-Hailey disease - Carcinoma of fallopian tubes
  • Leiner disease -Graft versus host disease
  • Lichen planus - HIV infection
  • Lupus erythematosus - Lymphoma (including Hodgkin disease)
  • Mycosis fungoides -Multiple myeloma
  • Pemphigoid - Carcinoma of lung
  • Pemphigus foliaceus - Mycosis fungoides
  • Pityriasis rubra pilaris - Reiter syndrome
  • Psoriasis
  • Sarcoid
  • Seborrheic dermatitis
  • Stasis dermatitis

The most common causes of exfoliative dermatitis are best remembered by the mnemonic device ID-SCALP. The causes and their frequencies are as follows:

  • Idiopathic - 30%
  • Drug allergy - 28%
  • Seborrheic dermatitis - 2%
  • Contact dermatitis - 3%
  • Atopic dermatitis - 10%
  • Lymphoma and leukemia - 14%
  • Psoriasis - 8%

More than 60 drugs have been implicated in the causation of exfoliative dermatitis (see Table). In many cases of protracted exfoliative dermatitis classified as being of undetermined cause, careful follow-up care and reevaluation implicated atopic dermatitis in older patients, intake of drugs overlooked by the patient, and prelymphomatous eruption as causative factors.

Table. Drugs Implicated in the Causation of Exfoliative Dermatitis (Open Table in a new window)

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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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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