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.
Highlights
Erythroderma is characterized by erythema and scaling affecting more than 80% of the body surface area
Several skin disorders may culminate with erythroderma: exacerbation of previous dermatoses, drug eruption, and cutaneous lymphomas
Among the 129 patients evaluated with the detailed protocol, 49/129 (38%) had a history of previous skin disease: all patients with erythrodermic atopic dermatitis (AD) (13/13) had a history of previous AD, and 14/18 (77.8%) patients with erythrodermic psoriasis had a history of psoriasis vulgaris
Summary
Erythroderma is characterized by erythema and scaling affecting more than 80% of the body surface area. Several skin disorders may culminate with erythroderma: exacerbation of previous dermatoses (e.g., psoriasis, eczema, atopic dermatitis, pityriasis rubra pilaris, pemphigus foliaceous), drug eruption, and cutaneous lymphomas (mycosis fungoides, Sézary syndrome, adult T-cell leukemia/lymphoma). The challenge in these patients is represented by the identification of the etiological agents or conditions, which is relevant in clinical management and prognosis. Demographic data Age Sex Ethnicity Clinical data Time of onset of erythroderma to the first evaluation Previous cutaneous diseases Exposition to new medications or possible allergens Comorbidities Weight loss Pruritus Non-scarring diffuse alopecia Palmo-plantar keratoderma Ungueal alterations Ectropion Lower limb edema Mucosal lesions Achromic vitiligo-like lesions Spared areas Lymph node enlargement Hepatosplenomegaly Temperature Heart rate Respiratory rate Blood pressure Laboratory tests Complete blood count (leukocytosis if ≥ 11,000/mm[3]; lymphocytosis if ≥ 3,400/mm[3]; eosinophilia if ≥ 500/mm3) Immunophenotyping of lymphocytes by flow cytometry[8] Sézary cell count in peripheral blood smear[68] T-cell receptor (TCR) gene rearrangement analysis by PCR in the blood[9] Lactate dehydrogenase Protein electrophoresis Total IgE HIV serology HTLV 1/2 serology[69] Three simultaneous skin biopsies to histology One skin biopsy to TCR gene rearrangement analysis Computed tomography (CT) of thorax, abdomen, and pelvis Lymph-node excisional biopsy if ≥ 1.5 cm, or if abnormal characteristics are found (firm, irregular, clustered, or fixed)
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
