Elsevier

Clinics in Dermatology

Volume 23, Issue 2, March–April 2005, Pages 206-217
Clinics in Dermatology

Life-threatening erythroderma: diagnosing and treating the “red man”

https://doi.org/10.1016/j.clindermatol.2004.06.018Get rights and content

Abstract

Exfoliative erythroderma, or diffuse erythema and scaling of the skin, may be the morphologic presentation of a variety of cutaneous and systemic diseases. Establishing the diagnosis of the underlying disease is often difficult and, not uncommonly, erythroderma is classified as idiopathic. Several cases are presented to demonstrate the diversity of presentation of this disease. Laboratory findings are typically unhelpful in establishing the etiology of erythroderma. Clinical data combined with multiple skin biopsies over time are necessary. Systemic complications of erythroderma include infection, fluid and electrolyte imbalances, thermoregulatory disturbance, high output cardiac failure, and acute respiratory distress syndrome. The initial approach to the management of erythroderma of any etiology includes attention to nutrition, fluid and electrolyte replacement, and the institution of gentle local skin care measures. Oatmeal baths and wet dressings to weeping or crusted sites should be followed by application of bland emollients and low-potency topical corticosteroids. Systemic dermatologic therapy may be required to maintain improvement achieved with local measures or to control erythroderma refractory to local measures. The prognosis of erythroderma is dependent on the underlying etiology.

Introduction

Exfoliative erythroderma, or diffuse erythema and scaling of the skin, may be the morphologic presentation of a variety of cutaneous and systemic diseases (Table 1). The dermatologist is faced with multiple challenges when caring for the patient with exfoliative erythroderma. Establishing the diagnosis of the underlying disease is often difficult and, not uncommonly, erythroderma is classified as idiopathic. Exfoliative erythroderma places the patient at high risk for secondary infection as well as cardiovascular and respiratory compromise. Additional risks are posed by systemic dermatologic therapy, which is often required to control the disease. The following case summaries highlight these clinical challenges.

Section snippets

Case 1

In March 2000, a 55-year-old man is referred for a second opinion to the university for a 1-year history of abrupt-onset erythroderma refractory to cyclosporine and acitretin. The patient complains of fissuring of the palms and soles and of being cold and pruritic but has been able to continue his work as a financial analyst. No new medications were initiated before the onset of erythroderma, and discontinuation of medications was unhelpful. The patient's past medical history is significant for

Epidemiologic and clinical features of erythroderma

The above cases are illustrative of the varied clinical presentations of erythroderma. There are multiple published epidemiologic studies of erythrodermic patients.3., 4., 25., 26., 32., 35., 42., 43., 44., 45., 46., 47., 48. A male predominance has been observed, with a male-to-female ratio ranging from 2:1 to 4:1. The average reported age of affected patients is 41 to 61 years, with most published series excluding children. The most commonly diagnosed etiologies are psoriasis, spongiotic

Laboratory investigations

Laboratory findings are most often not diagnostic for etiology of erythroderma. Common abnormalities found in erythrodermic patients include leukocytosis, anemia, elevated erythrocyte sedimentation rate, lymphocytosis, eosinophilia, and increased serum IgE.3., 25., 32., 43., 44., 46., 47., 53., 101. In a clinical study by Sigurdsson et al,53 13 of 102 patients had an erythrocyte sedimentation rate higher than 30 mm/h, 8 of whom had idiopathic erythroderma. Other findings include elevated

Histopathology

The histopathology of erythroderma varies depending on the underlying etiology (Table 3). Therefore, careful examination of the epidermis and dermis from a punch biopsy is recommended. In approximately one third of erythrodermic patients, however, the biopsies fail to reveal the diagnostic features of any specific disease. In addition, only 50% of any specific biopsy submitted on these patients are likely to reveal the underlying cause.107., 108. This is in part because the specific features of

Algorithm of approach to the erythrodermic patient

Erythrodermic patient presents to your office.

Obtain history of possible precipitating causes and pertinent past medical history and family history. Check medication history.

Full skin examination of patient for any telltale sign of underlying skin diseases. Check nails and mucosa. Check lymph nodes and rule out organomegaly. Take vital signs to establish patient stability.

Perform multiple skin biopsies for routine histology. Simultaneously initiate cool down topical therapies and supportive

Systemic complications

Systemic complications of erythroderma include infection, fluid and electrolyte imbalances, thermoregulatory disturbance, high output cardiac failure,109 acute respiratory distress syndrome,110 and gynecomastia. The inflamed, fissured, and excoriated skin is susceptible to bacterial colonization, and sepsis occurs occasionally. Staphylococcal sepsis is especially a risk for patients with CTCL and HIV-positive erythroderma.110., 111., 112. Fluid and electrolyte imbalances occur from loss of

Treatment

The initial approach to the management of erythroderma of any etiology includes attention to nutrition and fluid and electrolyte replacement and the institution of gentle local skin care measures. Oatmeal baths and wet dressings to weeping or crusted sites should be followed by application of bland emollients and low-potency topical corticosteroids. Higher potency topical corticosteroids are not recommended because of risk for systemic absorption secondary to the extensive body surface area and

Natural course of disease

The prognosis of erythroderma is dependent on the underlying etiology. With the exception of severe systemic hypersensitivity reactions, drug-induced erythroderma clears readily with discontinuation of the causative drug.44., 121. Erythroderma secondary to psoriasis and spongiotic dermatitis usually improves within several weeks to several months, although cases of chronic psoriatic atopic erythroderma are not uncommon. Erythrodermic psoriasis may recur in 15% of patients after initial clearing.

Conclusions

The erythrodermic patient requires immediate attention. Unfortunately, physical examination, routine laboratory findings, and even initial biopsies may be nondiagnostic, often making these patients challenging to diagnose and treat. Multiple biopsies and, often, repeat biopsies may be necessary to make a definitive diagnosis. Although death from cardiac failure, sepsis, capillary leakage syndrome, and even respiratory distress syndrome have been reported, early medical intervention and newer

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