Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Erythroderma (Generalized Exfoliative Dermatitis)

  • Author: Sanusi H Umar, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Mar 03, 2016
 

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 eryth Exfoliative 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 (a chronic process associated with exacerbation and remissions), Hebra or pityriasis rubra (relentlessly progressive disease), and Savill (self-limiting) types lacks any clinical significance.

Next

Pathophysiology

An increased skin blood perfusion occurs in exfoliative dermatitis (ED) 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, eosinophil infiltration, and CD4+ T-cell lymphocytopenia in the absence of HIV infection. Oxidative stress is also associated with drug-induced erythroderma.[1]

Previous
Next

Epidemiology

Race

No racial predilection is reported for exfoliative dermatitis (ED).

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]

Previous
 
 
Contributor Information and Disclosures
Author

Sanusi H Umar, MD, FAAD Clinical Instructor of Medicine, Department of Medicine, Division of Dermatology, University of California, Los Angeles, David Geffen School of Medicine

Sanusi H Umar, MD, FAAD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, 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 Medical Association, American Society for Dermatologic Surgery, National Medical Association, Pacific Dermatologic Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman 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, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

References
  1. Verma P, Bhattacharya SN, Banerjee BD, Khanna N. Oxidative stress and leukocyte migration inhibition response in cutaneous adverse drug reactions. Indian J Dermatol Venereol Leprol. 2012 Sep-Oct. 78(5):664. [Medline].

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

  3. Fraitag S, Bodemer C. Neonatal erythroderma. Curr Opin Pediatr. 2010 Aug. 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. 2010 May-Jun. 20(3):373-7. [Medline].

  5. Jusufbegovic D, Char DH. Clinical variability of ocular involvement in mycosis fungoides. JAMA Ophthalmol. 2015 Mar. 133(3):341-3. [Medline].

  6. Mateo S, García-Martínez FJ, Sánchez-Aguilar D, Amarelo J, Toribio J. Psoriasiform exfoliative erythroderma induced by golimumab. Clin Exp Dermatol. 2014 Aug 22. 39(7):813-15. [Medline].

  7. Nishizawa A, Igawa K, Teraki H, Yokozeki H. Diffuse disseminated lichenoid-type cutaneous sarcoidosis mimicking erythroderma. Int J Dermatol. 2014 Aug. 53(8):e369-70. [Medline].

  8. Doukaki S, Aricò M, Bongiorno MR. Erythroderma related to the administration of 99mTc-sestamibi: the first report. J Nucl Cardiol. 2010 Jun. 17(3):520-2. [Medline].

  9. Rolfes N, Lümmen G. Hypertension and palmar plantar erythroderma. Management of adverse events of angiogenetic inhibitors in the treatment of renal cell carcinoma. [Article in German]. Urologe A. 2011 Nov. 50(11):1387-91. [Medline].

  10. Huang HY, Luo XQ, Chan LS, Cao ZH, Sun XF, Xu JH. Cutaneous adverse drug reactions in a hospital based Chinese population. Clin Exp Dermatol. 2011 Mar. 36(2):135-41. [Medline].

  11. Zhang B, Bolognia J, Marks P, Podoltsev N. Enhanced skin toxicity associated with the combination of clofarabine plus cytarabine for the treatment of acute leukemia. Cancer Chemother Pharmacol. 2014 Aug. 74(2):303-7. [Medline].

  12. Zhang JC, Sun YT. Efavirenz-induced exfoliative dermatitis. Scand J Infect Dis. 2013 Jan. 45(1):70-2. [Medline].

  13. Ram-Wolf C, Mahé E, Saiag P. Escitalopram photo-induced erythroderma. J Eur Acad Dermatol Venereol. 2008 Aug. 22(8):1015-7. [Medline].

  14. Mumoli N, Luschi R, Camaiti A, Cei M, Bagnoni G, Biondi A. Severe exfoliative dermatitis caused by esomeprazole. J Am Geriatr Soc. 2011 Dec. 59(12):2377-8. [Medline].

  15. Dua R, Sindhwani G, Rawat J. Exfoliative dermatitis to all four first line oral anti-tubercular drugs. Indian J Tuberc. 2010 Jan. 57(1):53-6. [Medline].

  16. Lee HY, Tay LK, Thirumoorthy T, Pang SM. Cutaneous adverse drug reactions in hospitalized patients. Singapore Med J. 2010 Oct. 51(10):767-74. [Medline].

  17. Reynaud F, Giraud P, Cisterne JM, Verdier D, Kouchakipour Z, Hermelin A, et al. Acute immune allergic interstitial nephritis after treatment with fluindione. Seven cases. [Article in French]. Nephrol Ther. 2009 Jul. 5(4):292-8. [Medline].

  18. Tamer E, Gur G, Polat M, Alli N. Flare-up of pustular psoriasis with fluoxetine: possibility of a serotoninergic influence?. J Dermatolog Treat. 2009. 20(3):1-3. [Medline].

  19. Ozuguz P, Kacar SD, Ozuguz U, Karaca S, Tokyol C. Erythroderma secondary to gliclazide: a case report. Cutan Ocul Toxicol. 2014 Dec. 33(4):342-4. [Medline].

  20. Hulmani M, Nandakishore B, Bhat MR, Sukumar D, Martis J, Kamath G, et al. Clinico-etiological study of 30 erythroderma cases from tertiary center in South India. Indian Dermatol Online J. 2014 Jan. 5(1):25-9. [Medline].

  21. Kumar S, Mahajan BB, Kaur S, Banipal RP, Singh A. Imatinib mesylate induced erythroderma: A rare case series. J Cancer Res Ther. 2015 Oct-Dec. 11(4):993-6. [Medline].

  22. Markvardsen LH, Jakobsen J. Exfoliative dermatitis as a side effect of intravenous immunoglobulin treatment. [Article in Danish]. Ugeskr Laeger. 2011 Oct. 173(43):2725-6. [Medline].

  23. Igawa K, Konishi M, Moriyama Y, Fukuyama K, Yokozeki H. Erythroderma as drug eruption induced by intravesical mitomycin C therapy. J Eur Acad Dermatol Venereol. 2015 Mar. 29(3):613-4. [Medline].

  24. Choi CU, Rha SW, Suh SY, Kim JW, Kim EJ, Park CG, et al. Extensive exfoliative dermatitis induced by non-ionic contrast medium Iodixanol (Visipaque) used during percutaneous coronary intervention. Int J Cardiol. 2008 Feb. 124(2):e25-7. [Medline].

  25. Vaish AK, Tripathi AK, Gupta LK, Jain N, Agarwal A, Verma SK. An unusual case of DRESS syndrome due to leflunomide. BMJ Case Rep. 2011 Sep. 2011:[Medline].

  26. Sadeghpour M, Bunick CG, Robinson DM, Galan A, Tigelaar RE, Imaeda S. Midodrine-induced acute generalized exanthematous pustulosis. Cutis. 2014 May. 93(5):E17-20. [Medline].

  27. Arai S, Mukai H. Erythroderma induced by morphine sulfate. J Dermatol. 2011 Mar. 38(3):288-9. [Medline].

  28. Bhandarkar AP, Kop, PB, Pai VV. Nevirapine induced exfoliative dermatitis in an HIV-infected patient. Indian J Pharmacol. 2011 Nov-Dec. 43(6):738-739. [Medline].

  29. Sánchez-Borges M, González-Aveledo L. Exfoliative erythrodermia induced by pantoprazole. Allergol Immunopathol (Madr). 2012 May-Jun. 40(3):194-5. [Medline].

  30. Bilaç C, Müezzinoğlu T, Ermertcan AT, Kayhan TC, Temeltaş G, Oztürkcan S, et al. Sorafenib-induced erythema multiforme in metastatic renal cell carcinoma. Cutan Ocul Toxicol. 2009. 28(2):90-2. [Medline].

  31. Smith EV, Shipley DR. Severe exfoliative dermatitis caused by strontium ranelate: two cases of a new drug reaction. Age Ageing. 2010 May. 39(3):401-3. [Medline].

  32. Eyler JT, Squires S, Fraga GR, Liu D, Kestenbaum T. Two cases of acute generalized exanthematous pustulosis related to oral terbinafine and an analysis of the clinical reaction pattern. Dermatol Online J. 2012 Nov. 18(11):5. [Medline].

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

  34. Rowe CJ, Robertson I, James D, McMeniman E. Warfarin-induced erythroderma. Australas J Dermatol. 2015 Feb. 56(1):e15-7. [Medline].

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

  36. Clark RA, Shackelton JB, Watanabe R, Calarese A, Yamanaka K, Campbell JJ, et al. High-scatter T cells: a reliable biomarker for malignant T cells in cutaneous T-cell lymphoma. Blood. 2011 Feb 10. 117(6):1966-76. [Medline].

  37. Kirsch IR, Watanabe R, O'Malley JT, Williamson DW, Scott LL, Elco CP, et al. TCR sequencing facilitates diagnosis and identifies mature T cells as the cell of origin in CTCL. Sci Transl Med. 2015 Oct. 7(308):308ra158. [Medline].

  38. Sbidian E, Battistella M, Rivet J, Flageul B, Molina JM, Joly P, et al. Remission of severe CD8(+) cytotoxic T cell skin infiltrative disease in human immunodeficiency virus-infected patients receiving highly active antiretroviral therapy. Clin Infect Dis. 2010 Sep. 51(6):741-8. [Medline].

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

  40. Bosseila M, Mahgoub D, El-Sayed A, Salama D, Abd El-Moneim M, Al-Helf F. Does fluorescence diagnosis have a role in follow up of response to therapy in mycosis fungoides?. Photodiagnosis Photodyn Ther. 2014 Dec. 11(4):595-602. [Medline].

  41. 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? [Article in French]. Ann Dermatol Venereol. 1998 Jan. 1. 25(1):13-7. [Medline].

  42. 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. 2010 Dec. 32(8):755-63. [Medline].

  43. Lee WK, Kim GW, Cho HH, Kim WJ, Mun JH, Song M, et al. Erythrodermic psoriasis treated with golimumab: a case report. Ann Dermatol. 2015 Aug. 27(4):446-9. [Medline].

  44. Wang J, Wang YM, Ahn HY. Biological products for the treatment of psoriasis: therapeutic targets, pharmacodynamics and disease-drug-drug interaction implications. AAPS J. 2014 Sep. 16(5):938-47. [Medline].

  45. Sanford M, McKeage K. Secukinumab: first global approval. Drugs. 2015 Feb. 75(3):329-38. [Medline].

  46. Alberti-Violetti S, Talpur R, Schlichte M, Sui D, Duvic M. Advanced-stage mycosis fungoides and Sézary syndrome: survival and response to treatment. Clin Lymphoma Myeloma Leuk. 2015 Jun. 15(6):e105-12. [Medline].

  47. Cather JC, Crowley JJ. Use of biologic agents in combination with other therapies for the treatment of psoriasis. Am J Clin Dermatol. 2014 Dec. 15(6):467-78. [Medline].

  48. Rosenbach M, Hsu S, Korman NJ, Lebwohl MG, Young M, Bebo BF Jr, et al. Treatment of erythrodermic psoriasis: from the medical board of the National Psoriasis Foundation. J Am Acad Dermatol. 2010 Apr. 62(4):655-62. [Medline].

  49. Armstrong AW, Bagel J, Van Voorhees AS, Robertson AD, Yamauchi PS. Combining biologic therapies with other systemic treatments in psoriasis: evidence-based, best-practice recommendations from the Medical Board of the National Psoriasis Foundation. JAMA Dermatol. 2015 Apr. 151(4):432-8. [Medline].

  50. Al Hothali GI. Review of the treatment of mycosis fungoides and Sézary syndrome: A stage-based approach. Int J Health Sci (Qassim). 2013 Jun. 7(2):220-39. [Medline].

  51. Rupoli S, Canafoglia L, Goteri G, Leoni P, Brandozzi G, Federici I, et al. Results of a prospective phase II trial with oral low dose bexarotene plus photochemotherapy (PUVA) in refractory and/or relapsed patients with mycosis fungoides. Eur J Dermatol. 2015 Dec 16. [Epub ahead of print]. [Medline].

  52. Sokolowska-Wojdylo M, Florek A, Zaucha JM, Chmielowska E, Giza A, Knopinska-Posluszny W, et al. Polish Lymphoma Research Group experience with bexarotene in the treatment of cutaneous T-cell lymphoma. Am J Ther. 2014 Apr 11. [Epub ahead of print]. [Medline].

  53. Chung CG, Poligone B. Cutaneous T cell lymphoma: an update on pathogenesis and systemic therapy. Curr Hematol Malig Rep. 2015 Dec. 10(4):468-76. [Medline].

  54. Galper SL, Smith BD, Wilson LD. Diagnosis and management of mycosis fungoides. Oncology (Williston Park). 2010 May. 24(6):491-501. [Medline].

  55. Wilcox RA. Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management. Am J Hematol. 2016 Jan. 91(1):151-65. [Medline].

  56. Humme D, Nast A, Erdmann R, Vandersee S, Beyer M. Systematic review of combination therapies for mycosis fungoides. Cancer Treat Rev. 2014 Sep. 40(8):927-33. [Medline].

  57. Hughes CF, Khot A, McCormack C, Lade S, Westerman DA, Twigger R, et al. Lack of durable disease control with chemotherapy for mycosis fungoides and Sézary syndrome: a comparative study of systemic therapy. Blood. 2015 Jan. 125(1):71-81. [Medline].

  58. Duvic M, Olsen EA, Breneman D, Pacheco TR, Parker S, Vonderheid EC, et al. Evaluation of the long-term tolerability and clinical benefit of vorinostat in patients with advanced cutaneous T-cell lymphoma. Clin Lymphoma Myeloma. 2009 Dec. 9(6):412-6. [Medline].

  59. Prince HM, Dickinson M, Khot A. Romidepsin for cutaneous T-cell lymphoma. Future Oncol. 9(12). 2013 Dec.:1819-27. [Medline].

  60. Foss F, Advani R, Duvic M, Hymes KB, Intragumtornchai T, Lekhakula A, et al. A Phase II trial of Belinostat (PXD101) in patients with relapsed or refractory peripheral or cutaneous T-cell lymphoma. Br J Haematol. 2015 Mar. 168(6):811-9. [Medline].

  61. Guttman-Yassky E, Dhingra N, Leung DY. New era of biologic therapeutics in atopic dermatitis. Expert Opin Biol Ther. 2013 Apr. 13(4):549-61. [Medline].

  62. Lancrajan C, Bumbacea R, Giurcaneanu C. Erythrodermic atopic dermatitis with late onset--case presentation. J Med Life. 2010 Jan-Mar. 3(1):80-3. [Medline].

  63. 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. 1996 Apr. 34(4):626-31. [Medline].

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

  65. Patel S, Patel T, Kerdel FA. The risk of malignancy or progression of existing malignancy in patients with psoriasis treated with biologics: case report and review of the literature. Int J Dermatol. 2015 Dec. [Epub ahead of print]. [Medline].

  66. Hsu L, Armstrong AW. Anti-drug antibodies in psoriasis: a critical evaluation of clinical significance and impact on treatment response. Expert Rev Clin Immunol. 2013 Oct. 9(10):949-58. [Medline].

  67. Zattra E, Belloni Fortina A, Peserico A, Alaibac M. Erythroderma in the era of biological therapies. Eur J Dermatol. 2012 Mar-Apr. 22(2):167-71. [Medline].

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

 
Previous
Next
 
Exfoliative dermatitis diffuse skin involvement.
Exfoliative dermatitis close-up view showing erythema and scaling.
Table. Drugs Implicated in the Causation of Exfoliative Dermatitis
99mTC-sestamibi[8] ACE inhibitors Allopurinol Aminoglutethimide Amiodarone
Amitriptyline Amoxicillin Ampicillin Angiogenetic inhibitors[9] Arsenic
Aspirin Atropine Auranofin Aurothioglucose Barbiturates
Benactyzine Beta-blockers Beta carotene Bumetanide Bupropion
Butabarbital Butalbital Captopril Carbamazepine Carbidopa
Cephalosporins[10] Chloroquine Chlorpromazine Chlorpropamide Cimetidine
Ciprofloxacin Cisplatin Clofarabine[11] Clofazimine Clofibrate
Co-trimoxazole Cromolyn Cytarabine Dapsone Demeclocycline
Desipramine Diazepam Diclofenac Diflunisal Diltiazem
Doxorubicin Doxycycline Efavirenz[12] Enalapril Escitalopram[13]
Esomeprazole[14] Ethambutol[15] Etodolac Fenofibrate[16] Fenoprofen
Fluconazole Fluindione[17] Fluoxetine[18] Fluphenazine Flurbiprofen
Furosemide Gemfibrozil Gliclazide[19] Glipizide[20] Gold
Griseofulvin Hydroxychloroquine Imatinib[21] Imipramine Indomethacin
Intravenous immunoglobulin[22] Intravesical mitomycin C[23] Iodixanol[24] Isoniazid Isosorbide
Ketoconazole Ketoprofen Ketorolac Leflunomide[25] Lithium
Meclofenamate Mefenamic Acid Meprobamate Methylphenidate
Midodrine[26] Minocycline Morphine sulfate[27] Nalidixic Acid Naproxen
Nevirapine[28] Nitrazepam[20] Nifedipine Nitrofurantoin Nitroglycerin
Nizatidine Norfloxacin Omeprazole Pantoprazole[29] Penicillamine
Penicillin Pentobarbital Perphenazine Phenobarbital Phenothiazines
Phenylbutazone Phenytoin Piroxicam Primidone Prochlorperazine
Propranolol Pyrazinamide[15] Pyrazolones Quinapril Quinidine
Quinine Retinoids Rifampin Sorafenib[30] Streptomycin
Strontium ranelate[31] Sulfadoxine Sulfamethoxazole Sulfasalazine Sulfisoxazole
Sulfonamides Sulfonylureas Sulindac Terbinafine[32] Tetracycline
Tobramycin Tocilizumab[33] Trazodone Trifluoperazine Trimethoprim
Vancomycin Verapamil Warfarin[34]
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.