Erythroderma

These patients will be at high risk from a range of potentially life threatening complications that relate to the high degree of skin inflammation.

The condition constitutes a true dermatological emergency and requires prompt resuscitation and referral to a dermatologist.

Pathology

Causes:

Erythroderma can arise from a variety of causes but does so most often as an extension of a pre-existing skin disorder.

The causes of a generalized erythrodermic skin reaction include:

1.         Pre-existing dermatitis:

            Generalized:

●          Seborrheic

●          Atopic

●          Contact

2.         Pre-existing Psoriasis:

            ●          Generalized, and may include a generalized pustular psoriasis.

3.         Pre-existing Blistering diseases:

            ●          Pemphigus and bullous pemphigoid

4.         Drug reactions, (Toxic epidermal necrolysis):

Many drugs have been implicated, including:

●          Allopurinol

●          Antiepileptics:

♥          Phenytoin, carbamazepine, phenobarbitone, lamotrigine.

●          Antimicrobials:

♥          Beta-lactams particularly cefoxitin, sulphonamides, nitrofurantoin,             griseofulvin

●          Less commonly:

♥          Angiotensin converting enzyme inhibitors, hydroxychloroquine,     chlorpromazine, diltiazem, lithium

5.         Idiopathic:

            ●          The cause is not found in up to 30% of patients.

6.         Haematological:

            ●          Lymphoma related:

                        ♥          Cutaneous T-cell lymphoma (Sezary syndrome)

            ●          Leukaemia

7.         Pityriasis rubra pilaris

8.         Infective:

            ●          Crusted (Norwegian) scabies.

            ●          HIV

Complications:

These can include:

●          Dehydration from fluid loss

●          Electrolyte loss

●          Protein loss

●          Secondary bacterial infection

●          Hypothermia

●          High output cardiac failure in elderly patients.

Clinical assessment

Features of the erythroderma:

Features of erythrodermic skin reactions include:

• If a drug is the cause, erythroderma is usually preceded by a morbilliform (measles-like) eruption.
• Generalized skin redness (erythema) and swelling (oedema) involving 90% or       more of the skin surface
• Serous ooze, resulting in clothes and dressings sticking to the skin and an unpleasant smell

●          Scaling 2-6 days after the onset of erythema, as fine flakes or large sheets

●          Varying degrees of itching, sometimes intolerable

●          Thick scaling may develop on scalp with varying degrees of hair loss including     complete baldness

●          Thickening of palms of hands and soles of feet (keratoderma)

●          Eyelid swelling may result in ectropion (exposure of the inside surface of the         lower eyelid)

●          Nails become ridged and thickened or may shed

●          Longstanding erythroderma may result in pigmentary changes (brown and / or       white skin patches)

●          Secondary infection may occur with pustules and crusting

●          Lymphadenopathy

Important points of history:

●          Past medical history, in particular of any conditions known to predispose to an      erythrodermic reaction.

●          Drug history is very important

Important points of examination:

●          Vital signs

●          Circulatory status

●          Hydration status

●          Look for any secondary bacterial infection

●          Look for signs of possible haematological malignancy

Investigations

Blood tests:

1.         FBE

2.         U&Es/ glucose

3.         Calcium/ phosphate

Skin biopsy:

In cases of diagnostic uncertainty, as kin biopsy me be required to establish the underlying cause.

Management

Principles of management include:

1.         Fluid resuscitation

2.         Correction of electrolytes disturbances

3.         Consider albumin replacement

4.         Protect from hypothermia

5.         Treat any secondary bacterial infection

6.         Barrier nursing, (positive pressure room).

7.         Cease any suspected or possible offending drugs, or all non essential drugs.

8.         Antihistamines:

            ●          For pruritis

9.         Skin care:

●          Maintain skin moisture with wet wraps, other types of wet dressings,          emollients and mild topical steroids. The advice of a dermatologist should be sought.

10.       Steroids/ immunosuppressive agents:

●          These may be helpful in cases of dermatoses, or drug induced reactions,     but should only be initiated in the advice of a dermatologist.

11.       Treat the underlying cause where possible.

Disposition:

●          Urgent referral to Dermatology Unit

●          Possible referral to Burns Unit

●          Infectious Diseases Unit, if an infective cause is suspected.

●          Oncologist for malignant related reactions.

Prognosis:

Prognosis of erythroderma depends on the underlying disease process.

If the cause can be removed or corrected then prognosis is generally very good.

If erythroderma is the result of a generalized spread of a primary skin disorder such as psoriasis or dermatitis, it usually clears with appropriate treatment of the skin disease but may recur at any time.

The course of idiopathic erythroderma is unpredictable. It may persist for prolonged periods with episodes of acute exacerbation.

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References

FOAMed

Publications

• Erythroderma. DermNet