8.2 Erythrodermas

Synonyms

Red man syndrome. Symptoms. Various diagnoses.

Epidemiology

See specific diagnoses. See also chapter Red Face.

Definition

Inflammatory and scaly or exfoliative skin condition withredness and scaling of >90% of the skin, which either is primarily involved or spreads secondarily from a non-erythrodermic dermatosis.

Aetiology & Pathogenesis

Depends on underlying diagnosis (see classification).

Signs & Symptoms

Almost complete (>90%) redness and different degree of scaling of the skin, with or without itch. Sparing of the skin in the axila and abdominal folds or on the trunk (nappes claires). Swelling of lymph nodes as a consequence of pruritus scratching, erosions and superinfections. Due to loss of albumin and electrolytes via the exfoliative scaling edema develops. Sometimes temperature dysregulation and/or fever. Cachexia in some cases.

Localisation

Total skin, according to definition.

Classification

Primary Erythrodermas

  • Sézary syndrome.
  • Erythrodermic mycosis fungoides.
  • Staphylococcal scalded skin syndrome (SSSS). Erythroderma due to secondary loss of function of the skin (deficiency of vitamins, electrolytes; malabsorption due to alcohol excess with exsiccation) Senile-(melano-) erythroderma (with cachexia).
  • Congenital ichthyosiform erythroderma.
  • Severe generalised sunburn.
  • Ofuji Disease.

 

Secondary erythrodermas

Laboratory & other workups

Depending on diagnosis (mostly full blood count, electrolytes, liver enzymes, lymphocyte sub-populations, histamine, IgE).

Dermatopathology

Depending on diagnosis.

Course

Can be acute, sub-acute and chronic.

Complications

Loss of fluid (exsiccation), of proteins and electrolytes (scaling), cardiac insufficiency; thermal loss.

Diagnosis

Redness and scaling of the total integument. Specific diagnosis of the underlying disorder is based on the dynamic and follow up of the dermatosis and the histologic features.

Differential diagnosis

See classification.

Prevention & Therapy

Depending on diagnosis. Because of emergency situations, there is often a necessity of an acute intervention.

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Review Articles

  • Thestrup-Pedersen, K., Halkier, S. L., Sogaard, H., & Zachariae, H. (1988). The red man syndrome. Exfoliative dermatitis of unknown etiology: a description and follow-up of 38 patients. J Am Acad Dermatol, 18(6), 1307-1312. 

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