Life-threatening erythroderma: diagnosing and treating the “red man”
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.
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Obtain history of possible precipitating causes and pertinent past medical history and family history. Check medication history.
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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.
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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
References (121)
- et al.
The red man syndrome: exfoliative dermatitis of unknown etiology: a description and follow-up study of 38 patients
J. Am. Acad. Dermatol.
(1988) - et al.
Subacute cutaneous lupus erythematosus presenting as erythroderma
J. Am. Acad. Dermatol.
(1988) - et al.
Dermatomyositis presenting as erythroderma
J. Am. Acad. Dermatol.
(1992) - et al.
Paraneoplastic dermatomyositis presenting as erythroderma
J. Am. Acad. Dermatol.
(1998) - et al.
Erythrodermic dermatomyositis
J. Am. Acad. Dermatol.
(1993) - et al.
Adult-onset polymyositis-dermatomyositis: description of 25 patients with emphasis on treatment
Semin. Arthritis Rheum.
(1990) - et al.
Erythroderma after autologous bone marrow transplantation modified by administration of cyclosporine and interferon gamma for breast cancer
J. Am. Acad. Dermatol.
(1996) - et al.
Postoperative erythroderma after cardiac operations: the possible role of depressed cell-mediated immunity
J. Thorac. Cardiovasc. Surg.
(1992) - et al.
Acquired ichthyosiform erythroderma and sarcoidosis
J. Am. Acad. Dermatol.
(1996) - et al.
Erythroderma with spongiotic dermatitis: association with common variable hypogammaglobinemia
Am. J. Med.
(1985)
Omenn's syndrome and related combined immunodeficiency syndromes: diagnostic considerations in infants presenting with persistent erythroderma and failure to thrive
J. Am. Acad. Dermatol.
Severely hyperkeratotic erythroderma associated with Hodgkin's disease: does a high serum level of granulocyte-colony stimulating factor contribute to formation of skin lesions?
J. Am. Acad. Dermatol.
Adult T-cell leukemia with regression of erythroderma and simultaneous emergence of leukemia
J. Am. Acad. Dermatol.
Erythroderma: a clinical and follow-up study of 102 patients, with special emphasis on survival
J. Am. Acad. Dermatol.
Erythroderma secondary to esophageal carcinoma
J. Am. Acad. Dermatol.
Erythroderma: a follow-up of fifty cases
J. Am. Acad. Dermatol.
Erythrodermic psoriasis: precipitating factors, course, and prognosis in 50 patients
J. Am. Acad. Dermatol.
Erythroderma: a clinical and follow-up study of 102 patients, with special emphasis on survival
J. Am. Acad. Dermatol.
Severe exfoliative dermatitis associated with hand ischemia during cisplatin therapy
Mayo Clin. Proc.
Severe erythroderma as a complication of continuous epoprostenol therapy
Chest
Generalized exfoliative dermatitis caused by erythropoietin
Med. Clin. (Barc)
Cutaneous reactions to recombinant cytokine therapy
J. Am. Acad. Dermatol.
Erythroderma to pseudoephedrine in a patient with contact allergy to phenylephrine
Allergol. Immunopathol. (Madr)
A new cutaneous side effect of isotretinoin
J. Am. Acad. Dermatol.
Dermatologic side effects of thalidomide in patients with multiple myeloma
J. Am. Acad. Dermatol.
Chronic erythroderma induce by β-blocker (timolol maleate) eyedrops
J. Am. Acad. Dermatol.
Zidovudine-related erythroderma and successful desensitization: a case report
J. Allergy Clin. Immunol.
Sézary syndrome: diagnosis, prognosis, and critical review of treatment options
J. Am. Acad. Dermatol.
Bullous pemphigoid presenting as exfoliative erythroderma
J. Am. Acad. Dermatol.
Cutaneous plasmacytic pseudolymphoma with erythroderma
Int. J. Dermatol.
Generalized Hailey-Hailey disease
Br. J. Dermatol.
Exfoliative dermatitis: a clinicopathologic study of 135 cases
Arch. Dermatol.
Acute cutaneous toxoplasmosis presenting as erythroderma
Int. J. Dermatol.
Disseminated histoplasmosis presenting as exfoliative erythroderma
Arch. Dermatol.
Histoplasmosis presenting as erythroderma in a patient with the acquired immunodeficiency syndrome
Int. J. Dermatol.
Erythroderma as presentation form of visceral leishmaniasis in a patient with human immunodeficiency virus infection
Rev. Clin. Exp.
Erythroderma: an unusual presentation of pulmonary tuberculosis
Br. J. Dermatol.
Sarcoidosis in a child, presenting as an erythroderma with keratotoic spines and palmar pits
Br. J. Dermatol.
Throtoxicosis presenting as generalized pruritic exfoliative dermatitis and fever
Dermatology
Exfoliative erythroderma resulting from inadequate intake of branched-chain amino acids in infants with maple syrup urine disease
Arch. Dermatol.
Malignant histiocytosis presenting as erythroderma
Int. J. Dermatol.
Erythrodermia induced by hypercalcitoninemia
Ann. Dermatol. Venereol.
Erythroderma: review of 82 cases
South Med. J.
Erythroderma: a clinico-etiologic study of 90 cases
Int. J. Dermatol.
Erythroderma, an unusual manifestation of B cell lymphoma
Br. J. Dermatol.
Primary cutaneous anaplastic large-cell lymphoma with a prolonged erythrodermic prodrome
Br. J. Dermatol.
Aleukemic leukemia cutis: an unusual presentation of adult myelomonocytic leukemia
Dermatologica
101 cases exfoliative dermatitis
Arch. Dermatol.
Myelodysplasia presenting as erythroderma
Postgrad. Med. J.
Chronic eosinophilic leukaemia presenting with erythroderma, mild eosinophilia and hyper-IgE: clinical, immunological and cytogenetic features and therapeutic approach
Acta Haematol.
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