Dermatology
A 64 year old man, known to have psoriasis, presents to the Emergency Department with a flare up. Over 90% of the skin on his body is intensely red and his trunk is showing signs of exfoliation and excoriations where he has been scratching due to intense itching. His skin feels warm to the touch. His observations are recorded as:
- Heart rate: 89 beats/minute
- Blood pressure: 134/76 mmHg
- Respiratory rate: 18 breaths/minute
- Oxygen saturations: 96% on air
- Temperature: 37.9°C
What is the diagnosis?
Answer:
Erythroderma is the term used to describe intense and usually widespread reddening of the skin due to inflammatory skin disease. It often precedes or is associated with exfoliation (skin peeling off in scales or layers), when it may also be known as exfoliative dermatitis (ED). Idiopathic erythroderma is sometimes called the ‘red man syndrome’. The most common skin conditions to cause erythroderma are:- Drug eruption — with numerous diverse drugs implicated
- Dermatitis especially atopic dermatitis
- Psoriasis, especially after the withdrawal of systemic steroids or other treatment
- Pityriasis rubra pilaris
Erythroderma
Dermatology
Last Updated: 12th February 2021
Erythroderma is the term used to describe intense and usually widespread reddening of the skin due to inflammatory skin disease. It often precedes or is associated with exfoliation (skin peeling off in scales or layers), when it may also be known as exfoliative dermatitis (ED). Idiopathic erythroderma is sometimes called the ‘red man syndrome’.
Causes
Erythroderma is rare. It can arise at any age and in people of all races. It is about 3 times more common in males than in females. Most have a pre-existing skin disease or a systemic condition known to be associated with erythroderma. About 30% of cases of erythroderma are idiopathic.
The most common skin conditions to cause erythroderma are:
- Drug eruption — with numerous diverse drugs implicated
- Dermatitis especially atopic dermatitis
- Psoriasis, especially after the withdrawal of systemic steroids or other treatment
- Pityriasis rubra pilaris
Other skin diseases that less frequently cause erythroderma may include:
- Other forms of dermatitis: contact dermatitis (allergic or irritant), stasis dermatitis (venous eczema) and in babies, seborrhoeic dermatitis or staphylococcal scalded skin syndrome
- Blistering diseases including pemphigus and bullous pemphigoid
- Sezary syndrome (the erythrodermic form of cutaneous T-cell lymphoma)
- Several very rare congenital ichthyotic conditions.
Erythroderma may also be a symptom or sign of systemic disease. These may include:
- Haematological malignancies, such as lymphoma and leukaemia
- Internal malignancies, such as carcinoma of rectum, lung, fallopian tubes, colon, prostate (paraneoplastic erythroderma)
- Graft-versus-host disease
- HIV infection
Clinical features
Erythroderma is often preceded by a morbilliform (measles-like) eruption, dermatitis, or plaque psoriasis. Generalised erythema can develop quite rapidly in acute erythroderma, or more gradually over weeks to months in chronic erythroderma.
Signs and symptoms of erythroderma:
- By definition, generalised erythema and oedema or papulation affect 90% or more of the skin surface.
- The skin feels warm to the touch.
- Itch is usually troublesome and is sometimes intolerable. Rubbing and scratching leads to lichenification.
- Eyelid swelling may result in ectropion.
- Scaling begins 2-6 days after the onset of erythema, as fine flakes or large sheets.
- Thick scaling may develop on the scalp with varying degrees of hair loss including complete baldness.
- Palms and soles may develop yellowish, diffuse keratoderma.
- Nails become dull, ridged, and thickened or develop onycholysis and may shed (onychomadesis).
- Lymph nodes become swollen (generalised dermatopathic lymphadenopathy).
Systemic symptoms may be due to the erythroderma or to its cause:
- Lymphadenopathy, hepatosplenomegaly, abnormal liver dysfunction and fever may suggest a drug hypersensitivity syndrome or malignancy.
- Leg oedema may be due to inflamed skin, high output cardiac failure and/or hypoalbuminaemia.
Complications
Erythroderma often results in acute and chronic local and systemic complications. The patient is unwell with fever, temperature dysregulation and losing a great deal of fluid by transpiration through the skin.
- Heat loss leads to hypothermia.
- Fluid loss leads to electrolyte abnormalities and dehydration.
- Red skin leads to high-output heart failure.
- A secondary skin infection may occur (impetigo, cellulitis).
- General unwellness can lead to pneumonia.
- Hypoalbuminaemia from protein loss and increased metabolic rate causes oedema.
- Longstanding erythroderma may result in pigmentary changes (brown and/or white skin patches).
Investigations
- The blood count may show anaemia, white cell count abnormalities, and eosinophilia. Marked eosinophilia should raise suspicions for lymphoma.
- C-reactive protein may or may not be elevated.
- Proteins may reveal hypoalbuminaemia and abnormal liver function.
- Polyclonal gamma globulins are common, and raised immunoglobulin E (IgE) is typical of idiopathic erythroderma.
- Skin biopsies from several sites may be taken if the cause is unknown. They tend to show nonspecific inflammation on histopathology. Diagnostic features may also be present.
- Direct immunofluorescence is of benefit if an autoimmune blistering disease or connective tissue disease is considered.
Management
Erythroderma is potentially serious, even life-threatening, and the patient may require hospitalisation for monitoring and to restore fluid and electrolyte balance, circulatory status and body temperature.
The following general measures apply:
- Discontinue all unnecessary medications
- Monitor fluid balance and body temperature
- Maintain skin moisture with wet wraps, other types of wet dressings, emollients and mild topical steroids
- Prescribe antibiotics for bacterial infection
- Antihistamines may or may not be helpful for the itch
If a cause can be identified then specific treatment should be started, such as topical and systemic steroids for atopic dermatitis; acitretin or methotrexate for psoriasis.
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- Biochemistry
- Blood Gases
- Haematology
| Biochemistry | Normal Value |
|---|---|
| Sodium | 135 – 145 mmol/l |
| Potassium | 3.0 – 4.5 mmol/l |
| Urea | 2.5 – 7.5 mmol/l |
| Glucose | 3.5 – 5.0 mmol/l |
| Creatinine | 35 – 135 μmol/l |
| Alanine Aminotransferase (ALT) | 5 – 35 U/l |
| Gamma-glutamyl Transferase (GGT) | < 65 U/l |
| Alkaline Phosphatase (ALP) | 30 – 135 U/l |
| Aspartate Aminotransferase (AST) | < 40 U/l |
| Total Protein | 60 – 80 g/l |
| Albumin | 35 – 50 g/l |
| Globulin | 2.4 – 3.5 g/dl |
| Amylase | < 70 U/l |
| Total Bilirubin | 3 – 17 μmol/l |
| Calcium | 2.1 – 2.5 mmol/l |
| Chloride | 95 – 105 mmol/l |
| Phosphate | 0.8 – 1.4 mmol/l |
| Haematology | Normal Value |
|---|---|
| Haemoglobin | 11.5 – 16.6 g/dl |
| White Blood Cells | 4.0 – 11.0 x 109/l |
| Platelets | 150 – 450 x 109/l |
| MCV | 80 – 96 fl |
| MCHC | 32 – 36 g/dl |
| Neutrophils | 2.0 – 7.5 x 109/l |
| Lymphocytes | 1.5 – 4.0 x 109/l |
| Monocytes | 0.3 – 1.0 x 109/l |
| Eosinophils | 0.1 – 0.5 x 109/l |
| Basophils | < 0.2 x 109/l |
| Reticulocytes | < 2% |
| Haematocrit | 0.35 – 0.49 |
| Red Cell Distribution Width | 11 – 15% |
| Blood Gases | Normal Value |
|---|---|
| pH | 7.35 – 7.45 |
| pO2 | 11 – 14 kPa |
| pCO2 | 4.5 – 6.0 kPa |
| Base Excess | -2 – +2 mmol/l |
| Bicarbonate | 24 – 30 mmol/l |
| Lactate | < 2 mmol/l |
