Erythroderma; exfoliative dermatitis; L53.92

Hebra, 1868

Erythroderma is a descriptive term for a universal redness (dermatitis) of the skin (affecting > 90% of the skin organ), as a common terminal pathway of various diseases. Usually accompanied by marked scaling, intense pruritus (> 90% of patients), rarely weeping. Erythroderma is a severe inflammation of the skin with serious effects on the whole organism. An inference to an underlying disease cannot be derived from the erythrodermic condition per se.

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, danger of transcutaneous infection.

Erythrodermias are divided into primary (arising de novo) and secondary erythrodermias (arising on the ground of a preexisting dermatological disease).

• Primary erythroderma (s = very rare):
  • Acute primary erythroderma (arising de novo):
    • Toxic erythroderma (erythrodermic dermatitis, e.g. due to burns, scalds, UV damage, chemical burns).
    • Drug reaction, undesirable
    • Pustulosis acute generalized exanthematous (AGEP)
    • toxic epidermal necrolysis (TEN)
    • Anticonvulsant hypersensitivity syndrome.
  • Chronic primary erythroderma (congenital; see also erythroderma, neonatal):
    • Rare congenital ichthyoses (see below ichthyoses)
    • Ichthyosis lamellosa
    • CHILD syndrome (s)
    • Erythrodermia congenitalis ichthyosiformis bullosa(s)
    • Ichthyosis congenita gravis (Harlequin fetus) (s)
    • Keratitis-ichthyosis-deafness syndrome (= KID) (s)
    • Netherton syndrome (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):
    • Sézary syndrome
    • T- and B-cell lymphomas
    • Leukemias
    • senile erythroderma (idiopathic erythroderma).
• Secondary erythroderma (on the ground of a pre-existing disease!) (s = very rare):
  • Psoriasis vulgaris (60% of secondary erythroderma)
  • Atopic eczema
  • Seborrheic eczema
  • Impetigo herpetiformis (s)
  • Lichen planus exanthematicus (s)
  • Chronic actinic dermatitis (s)
  • Pityriasis rubra pilaris (s)
  • Pemphigus foliaceus (s)
  • Pemphigus, paraneoplastic
  • Pemphigoid, bullous
  • Dermatomyositis
  • Lupus erythematosus, systemic
  • Omenn's syndrome (s)
  • Scabies norvegica (s)
  • Hypereosinophilia syndrome (s)
  • Melanoerythroderma as a paraneoplastic syndrome(s), among others
  • Papuloerythroderma (Ofuji) (s).

The epidemiological data on erythroderma are unreliable. They vary between 0.9/100,000 for Europe and 35/100,000 in India.

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

Important: an important prerequisite for a 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=contatkdermatitis
• 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)

• Significantly increased skin circulation with consecutive cardiovascular stress.
• Excessively increased heat radiation (constant freezing of the patient).
• Disruption of the skin barrier with increased loss of fluid.
• Increased desquamation with increased loss of albumin and proteins.
• Non-specific disturbance of the immunological defence 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.
4. O'DONOVAN WJ (1950)Exfoliative erythrodermia with lymphadenopathy. Proc R Soc Med. 43:563-564.
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 (10947) Exfoliative erythrodermia with marked pigmentation. Proc R Soc Med 40:246-247.