Erythroderma

Management Strategy

The precise diagnosis of erythroderma is essential for proper management and better therapeutic outcome. Erythroderma has a gradual and insidious onset except for drug-induced cases. Psoriasis is the most frequent cause of ED.

ED is a dermatological emergency necessitating immediate therapeutic intervention. The diagnosis of underlying cause of erythroderma and early recognition of consequences of erythroderma is crucial in reducing associated morbidity and mortality. The consequences of the erythroderma differ depending upon the etiology: vesiculobullous diseases like pemphigus foliaceus can end in acute skin failure and inflammatory disease (psoriasis, AD) and other disorders of keratinization (PRP) will culminate in ED with a chronic course leading to chronic skin failure. The consequences of ED are due to failure of primary functions of the skin such as loss of an effective mechanical barrier, loss of thermoregulation from increased fluid loss via evaporation or exudation, and loss of protein due to the increased proliferative and metabolic activity with the risk of high-output cardiac failure. All these effects are greatest at the extremes of age.

The diagnostic clues can be uncovered by a detailed history, including comprehensive drug history comprising all over-the-counter and herbal remedies and thorough clinical examination. Table 78.2 lists the cutaneous examination clues for the diagnosis of diseases causing erythroderma. Drugs implicated in erythroderma described in the literature are anticonvulsants, antibiotics, non-steroidal antiinflammatory drugs, and even herbal medicines.

The clinicopathologic correlation is difficult because non-specific features of erythroderma may mask the specific features of an underlying dermatosis. Direct immunofluorescence studies may be of diagnostic utility in cases of erythroderma secondary to pemphigus foliaceus, bullous pemphigoid, graft-versus-host disease, and connective tissue disorders. Histopathology should be carried out as soon as possible and should be repeated in different areas and different stages of erythroderma. Immune-histochemistry techniques allow a better evaluation of the skin infiltrates, especially in cutaneous T-cell lymphoma (CTCL). Table 78.3 lists the investigations to be done and clinical parameters to be evaluated.

Initial management of all types of erythroderma is similar, even without an etiologic diagnosis. The main goal of management of erythroderma is restoring the functions of skin, and careful monitoring and prevention of infectious, metabolic, and systemic complications.

The first step is discontinuation of any unnecessary medications and appropriate workup. Bed rest and sedation should be advised when necessary. A sedative antihistamine such as hydroxyzine hydrochloride 25 mg twice daily is useful. Regulating environmental temperature is crucial since patients with this condition lose homeostatic body functions that prevent cooling or overheating. Ideally, room temperature must be kept at 30–32°C in order to slow catabolism and reduce the loss of calories through the skin . Skin barrier function can be improved with colloid baths, wet compresses, along with emollient creams and low-potency topical corticosteroids . Therapy must also include nutrition , preferably enteral nutrition, as it has also a protective effect on the intestinal mucosa and decreases bacterial colonization. Usually the number of calories required is 1500–2000 kcal/day. Fluid balance is a main focus in the management of ED. Once the percentage of the involved skin is established, Ringer lactate (RL) infusion of 1–2 mL/kg/% of involved skin must be started during the first 24 h.

A marked loss of exfoliated scales occurs that may reach 20–30 g/day. This contributes to the hypoalbuminemia commonly observed in ED. Albumin is recommended only if albumin serum level is <2.5 mg/dL. Nutritional supplementation is indicated if there is clinical evidence of pedal edema, or if biochemical values support for low serum protein, low serum proteins and reversal of albumin/globulin ratio are evident.

Wet wrap therapy (WWT) is an extremely effective treatment for acute erythrodermic eczema.

Bleach bath and antibiotics are advised if there is evidence of secondary infection. Routine use of antibiotics is contraindicated but there are specific indications for the use of antibiotics in ED, such as an increased number of bacteria cultured from the skin with selection of a single strain, sudden drop in temperature, deterioration in the patient’s condition, and symptoms of infections pertaining to a particular system.

The use of more specific pharmaceutical intervention requires careful consideration as directed by the etiology of the ED. Immunosuppressive drugs may be considered to be contraindicated if malignancy is suspected (particularly cutaneous lymphoma or paraneoplastic ED). When a firm diagnosis cannot be made, treatment may have to be directed at the most likely cause, based on the clinical and histologic features.