Erythroderma: a prospective study of 309 patients followed for 12 years in a tertiary center

Abstract

Erythroderma is characterized by erythema and scaling affecting more than 80% of the body surface area. It is potentially life-threatening, and diagnosis of the underlying disease is a challenge. Despite laboratory improvements, many cases remain idiopathic. We aimed to analyze clinical and laboratory findings of 309 erythrodermic patients to find clues to the etiologic diagnosis. We performed a prospective study at the University of São Paulo Medical School, from 2007 to 2018, with patients with acquired erythroderma. Clinical, laboratory, histology, and molecular biology data were collected. The median age at diagnosis was 57 years, with a male-to-female ratio of 2.2. Eczema was the most frequent etiology (20.7%), followed by psoriasis (16.8%), Sézary syndrome (12.3%), drug eruption (12.3%), atopic dermatitis (8.7%), and mycosis fungoides (5.5%). Other diagnoses (6.8%) included pemphigus foliaceous, paraneoplastic erythroderma, adult T-cell leukemia/lymphoma, dermatomyositis, pityriasis rubra pilaris, lichen planus, bullous pemphigoid, and leprosy. In 52 patients (16.8%), it was not possible to elucidate erythroderma etiology. Atopic dermatitis developed erythroderma at an earlier age (median 25 years; P = 0.0001). Acute onset was associated with drug reactions and atopic dermatitis (median time from erythroderma to diagnosis of 1 and 1.5 months, respectively; P = 0.0001). Higher immunoglobulin E levels were observed in atopic dermatitis (median 24,600 U/L; P = 0.0001). Histopathology was helpful and was consistent with the final diagnosis in 72.4%. Monoclonal T-cell proliferation in the skin was observed in mycosis fungoides (33.3%) and Sézary syndrome (90.9%). At the last assessment, 211 patients (69.3%) were alive with disease, 65 (21.7%) were alive without disease, and 27 (9.1%) died with active disease. Erythroderma is a challenging syndrome with a difficult diagnostic approach. Younger age and higher immunoglobulin E levels are associated with atopic dermatitis; acute onset is observed in drug eruptions and atopic dermatitis. Histopathology and molecular biology tests are essential tools in the investigation of erythroderma.

Erythroderma: A clinicopathological study of 47 cases from 2018 to 2020

“…In our study, the majority of patients were diagnosed with psoriasis. [6][7][8][9][10] Pemphigus foliaceus was not found in our study. The relative frequency of pemphigus foliaceus has been recently evaluated in a Tunisian retrospective study and was 6.25%.…”

“…The most common causes after psoriasis were drug eruption and MF. In our study, the majority of patients were diagnosed with psoriasis 6‐10 . Pemphigus foliaceus was not found in our study.…”

“…Other studies also report the elevated frequency of pruritus, affecting generally more than 90% of the patients 13,15 . While fever was as common as pruritus in our study, it was a rare finding in other studies 9 …”

“…13,15 While fever was as common as pruritus in our study, it was a rare finding in other studies. 9 Fever was generally more common in patients with drug reactions. This has been attributed to drug hypersensitivity in previous 16 In the literature, palmoplantar keratoderma and nail alterations are more commonly described in psoriasis.…”

“…In our study, palmoplantar keratoderma and nail changes were less common than previous studies. 2,9,13,17 Leukocytosis and eosinophilia are common in erythrodermic patients. In our study, while leukocyte was seen in 55% of patients, eosinophilia was seen in 23% of patients.…”