ErythrodermiaL53.9

Hebra, 1868

The term "erythroderma" is a purely descriptive term for a uniform, universal reddening of the skin (affecting > 90% of the skin organ), as a common final stage of various inflammatory diseases.

Erythroderma is usually accompanied by pronounced scaling and intense itching (> 90% of patients), less frequently by oozing.

The pathogenesis of erythroderma is based on a severe inflammation of the skin with serious effects on the entire organism. It is not possible to draw conclusions about an underlying disease from the erythrodermic condition per se. The etiologic classification is only possible after appropriate examinations.

In principle, erythroderma should be regarded as a serious, usually life-threatening condition.

Patients with neonatal erythroderma should be regarded as special clinical cases, as this condition is potentially life-threatening in newborns and young infants due to heat loss, transepidermal water loss and the risk of transcutaneous infection.

Erythroderma is divided into primary (arising de novo) and secondary erythroderma (arising on the basis of a pre-existing dermatological disease).

• Primary erythroderma(s = very rare):
  • Acute primary erythroderma (developed de novo in response to current events):
    • Toxic erythroderma (erythrodermic dermatitis, e.g. due to burns, scalds, UV-damage, chemical burns)
    • Drug reaction, adverse
    • Pustulosis acute generalized exanthematous (AGEP)
    • toxic epidermal necrolysis (TEN)
    • Anticonvulsant hypersensitivity syndrome.
  • Chronic primary erythroderma (congenital- see also erythroderma, neonatal):
    • Rare congenital ichthyoses (see ichthyoses below).
    • Ichthyosis lamellosa
    • CHILD syndrome(s)
    • Erythrodermia congenitalis ichthyosiformis bullosa(s)
    • Ichthyosis congenita gravis (Harlequin fetus)(s)
    • Keratitis-ichthyosis-deafness syndrome (= KID)(s)
    • Severe dermatitis-multiple allergies-metabolic loss syndrome (=SAM syndrome)(s)
    • Refsum syndrome(s)
    • Sjögren-Larsson syndrome(s)
    • Immunodeficiencies, T-cellular, primary.
  • Chronic primary erythroderma (acquired, insidious onset):
    • Sézary syndrome
    • T- and B-cell lymphomas
    • Leukemias
    • Senile erythroderma (idiopathic erythroderma; senile erythroderma of man/red man syndrome)
• Secondary erythroderma (on the ground of a pre-existing disease!)(s = very rare):
  • Psoriasis vulgaris (60% of secondary erythroderma)
  • Atopic dermatitis
  • Seborrheic dermatitis
  • Impetigo herpetiformis(s)
  • Lichen planus exanthematicus(s)
  • Dermatitis, chronic actinic(s)
  • Pityriasis rubra pilaris(s)
  • Pemphigus foliaceus(s)
  • Pemphigus, paraneoplastic (s)
  • Pemphigoid, bullous
  • Dermatomyositis
  • Lupus erythematosus, systemic
  • IPEX syndrome/mutation in FOXP3
  • Comel-Netherton syndrome/mutation in SPINK5
  • OMENN syndrome/RAG1/2
  • SCID(s)
  • Scabies norvegica(s)
  • Hypereosinophilia syndrome(s)
  • Melanoerythroderma a.o. as paraneoplastic syndrome(s)
  • Papuloerythroderma (Ofuji)(s).

Epidemiological data on erythroderma are unreliable. Prevalences vary from 0.9/100,000 for Europe to 35/100,000 in India.

The diagnostic value of a histological examination must be considered in a differentiated manner. Often the histological result is uncharacteristic. In the case of congenital erythroderma, histological examination may be diagnostically groundbreaking. Lymphomas of the skin can be clearly diagnosed. In the case of secondary erythroderma, clues to the underlying disease may be found.

Histological examination: an important prerequisite for a meaningful histological examination is a long-term discontinuation of any therapy with glucocorticoids (external as well as internal).

Systematics with auxiliary word "SCALPID

• S = Sezary syndrome, Scabies crustosa, SCLE
• C = contact dermatitis
• A = atopic dermatitis
• L = lymphoma (cutaneous T-cell lymphoma), lymphocytic leukemia, lichen planus
• P = psoriasis, pityriasis rubra pilaris, pemphigus foliaceus, paraneoplasia
• I = Ichthyosis
• D = Drugs (drug reactions)

For the skin and the entire organism, erythroderma, regardless of the causative underlying disease, represents a severe burden, especially due to:

• Significantly increased skin perfusion with consecutive cardiovascular stress.
• Excessively increased heat radiation (constant freezing of the patient).
• Disturbance of the skin barrier with increased loss of fluidity.
• Increased desquamation with increased loss of albumin and proteins.
• Unspecific disturbance of the immunological defense with increased tendency to infections.

1. Borrás-Blasco J et al.(2001) Erythrodermia induced by omeprazole. Int J Clin Pharmacol Ther 39:219 w23.
2. Iliescu V et al.(1997) Erythrodermia Sézary with immunological deficiency and antibodies against human albumin. Acta Med Scand 197(1-2):141-144. Kiessling W et al.(1959) Melano-erythrodermia with cachexia. Arch Klin Exp Dermatol 208:579-591.
3. Mori S et al. (1988) Postoperative erythrodermia (POED), a type of graft-versus-host reaction (GVHR)? Pathol Res Pract 184:53-59.
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5. Oztas P et al. (2006) Imatinib-induced erythrodermia in a patient with chronic myeloid leukemia. Acta Derm Venereol 86:174-175.
6. Sequeira JH (1919) Case of Erythrodermia with Lymphatic Leukaemia. Proc R Soc Med.12(Dermatol Sect):54-56.
7. Wigley JE (1947) Exfoliative Erythrodermia with Marked Pigmentation. Proc R Soc Med 40:246-247.