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Erythroderma: a Manifestation of Cutaneous and Systemic Diseases

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Erythroderma: a Manifestation of Cutaneous and Systemic Diseases Erythroderma: A Manifestation of Cutaneous and Systemic Diseases Authors: Sindi Hoxha, *Monika Fida, Ritjona Malaj, Ermira Vasili University Hospital Center ‘Mother Teresa’ Tirana, Tirana, Albania *Correspondence to [email protected] Disclosure: The authors have declared no conflicts of interest. The institutional ethical board of UMT approved this study. Received: 17.10.19 Accepted: 12.02.20 Keywords: Emergency, erythroderma, psoriasis. Citation: EMJ Allergy Immunol. 2020;DOI/10.33590/emjallergyimmunol/19-00182 Abstract Introduction: Erythroderma, or generalised exfoliative dermatitis, is a rare inflammatory disorder characterised by generalised erythema, involving more than 90% body surface area accompanied by a variable degree of scaling. Objectives: This retrospective study aimed to determine characteristics of erythroderma as a marker of cutaneous and systemic disease and to identify the underlying causes of this condition. Materials and methods: Hospitalised patients diagnosed with erythroderma at the dermatology and venereology department of UHC ‘Mother Teresa’ from 2012–2017 were selected for this study. Epidemiological, clinical, laboratory, and histological data of these patients were electronically compiled and analysed using SPSS© software (IBM, Armonk, New York, USA). Results: 116 patients were diagnosed with erythroderma. Of these, 43.1% were female and 56.9% were male. The average age of onset was 55.1 years. A variable hospitalisation period was observed from 1–49 days. Previous episodes of erythroderma were indicated in 11.2% of cases, and 29.3% of cases appeared as emergencies. The most common cause of erythroderma was exacerbation of pre-existing dermatoses, namely psoriasis (53.4%), eczema (3.4%), and dermatitis (10.3%). Drug hypersensitivity reactions were evidenced in 4.3% of cases. In 16.4% of cases, the cause of erythroderma was undetermined. This study demonstrated a high percentage of secondary erythroderma to a pre-existing cutaneous pathology, psoriasis in 53.4% of cases, and a low percentage of primary erythroderma. Conclusions: Erythroderma can be a manifestation of systemic and cutaneous diseases, but mostly erythrodermic psoriasis. Often it presents as a life-threatening emergency. The condition masks the primary pathology lesions, making it difficult to establish the correct diagnosis of the underlying aetiology. However, biopsy provides a positive correlation between clinical findings and anatomopathological diagnosis. DERMATOLOGY • April 2020 EMJ INTRODUCTION RESULTS Erythroderma, or generalised exfoliative Epidemiology dermatitis, is a rare inflammatory disorder characterised by generalised erythema, involving During this 6-year study, 116 patients were more than 90% of the body surface area diagnosed with erythroderma and admitted to accompanied by a variable degree of scaling.1 the authors’ department. The age of the patients Erythroderma can be a primary condition, when varied from 2–86 years, with a mean onset age of the cause is unknown, or a secondary condition, 55.1 years and the highest incidence of caused by known diseases. The age of its onset erythroderma occurring in the 61–70 age is related to its underlying aetiology, which group (26.7%; n=31). There was a slight male in most cases are skin diseases or disorders, predominance with a male to female ratio of hypersensitivity towards drugs and medications, 1.32:1.00 (57.0% male versus 43.0% female). There systemic diseases associated with cutaneous was no change regarding the age of onset of erythema, and in rare instances, malignancies. erythroderma in males and females using the Erythroderma predominates in males and most independent t-test. Both groups exhibited a commonly manifests in older ages. It is a potentially highest frequency of erythroderma onset in the life-threatening disease as it alters cutaneous seventh life decade. Considering all cases, the total functions, therefore erythrodermic patients number of patients hospitalised during the period require hospitalisation, rigorous monitoring, and of July and August was higher compared to other follow-up. Determining the underlying cause of months (16.4% versus 14.7%, respectively). erythroderma is important in choosing the right treatment for these patients. Clinical Parameters Patients were hospitalised in the department AIM of dermatology for an average of 12.6 days (day range: 1–49 days) following onset of This retrospective study aimed to identify erythroderma. A shorter hospitalisation period the underlying cause of erythroderma and to was observed in patients with drug-induced determine for the first time its epidemiological, erythroderma and a longer hospitalisation period clinical, and laboratory features in Albanian in erythrodermic psoriasis patients. A history of patients with erythroderma. previous erythrodermic episodes was noted in 11.2% of cases (n=13), 10 of which were caused MATERIALS AND METHODS by psoriasis, with the other three being caused by other pathologies, namely chronic eczema, The authors conducted a retrospective analysis of subcorneal pustulosis, and chronic dermatitis. The a total of 116 patients diagnosed with erythroderma majority of admissions were planned admissions, at the dermatology and venereology department however 29.3% of all the cases presented of UHC “Mother Teresa” from 20122017. Diffuse as emergencies. erythema, (over 70–90% of body surface) Clinical Findings in Admission accompanied by various degrees of scaling and pruritus, were used as the diagnostic clinical In the first clinical examination, generalised criteria. Epidemiological characteristics, clinical erythema, scaling, and pruritus were identified. symptoms, performed examinations, skin biopsy, Scaling and erythema are constant identifying immunophenotyping (whenever indicated), and markers of the pathology and will be found follow-up information were collected. Emergency in 100.0% of cases. Pruritus was detected in all and relapse cases were also highlighted when patients, and 34.5% of cases (n=40) had fever this information was available. The collected data >38.5 °C during the onset of the erythrodermic were electronically analysed using SPSS v.19. episode. Changes in the nails, psoriatic arthritis, and palmoplantar keratoderma were findings that prevailed in patients with erythrodermic psoriasis in almost 27.6% of cases (n=32) (Figure 1). Creative Commons Attribution-Non Commercial 4.0 April 2020 • DERMATOLOGY Figure 1: One of the patients with erythroderma caused by psoriasis vulgaris. (This photo was taken and used in thestudy with the patient’s permission). Laboratory Findings histopathological features were observed in 19 cases (16.4%). The most common abnormalities found in the patients included increased erythrocyte Final diagnosis was based on evaluation of the sedimentation rate and C-reactive protein in 93.1% clinical, biochemical, and histological findings, of cases (n=108), leukocytosis in 43.9% of cases as well as the development of erythroderma (n=51), and decreased haemoglobin in 30.2% of in each individual patient. The most common cases (n=35). underlying cause was exacerbation of pre-existing dermatoses, including psoriasis Cutaneous biopsy was performed in 105 patients in 59.0% of the cases, subcorneal pustulosis (90.5%). In the remaining 11 cases, when the in 5.2%, pityriasis rubra pilaris 1.7%, chronic cause of erythroderma was clear at the time of dermatitis in 3.4%, and pityriasis rosea in 1.7%. the examination, biopsy was unnecessary. This Drug hypersensitivity was seen in 4.3% of cases group included six cases previously diagnosed and no cause could be identified in 16.4% of with psoriasis and five cases of generalised cases. Diagnosis of idiopathic erythroderma was erythroderma appearing after the use of a made in patients who manifested an extended new drug. condition with the typical clinical signs of Histopathological examination was decisive generalised erythema, severe pruritus, and for concluding the diagnosis in 86/105patients scaling. In these cases, the significance of who received biopsy. The results were as histopathological examination is emphasised. follows: psoriasis vulgaris (53.4%) in 62 cases, The most important differential diagnosis in psoriasiform dermatitis (6.9%) in eight cases, idiopathic erythroderma is cutaneous lymphoma, subcorneal pustulosis (5.2%) in six cases, drug especially in older adult males with an extended hypersensitivity (4.3%) in five cases, chronic and relapsing course of pruritic erythroderma. eczema (3.4%) in four cases, chronic dermatitis Close monitoring and repeated biopsy in cases (2.6%) and erythema multiforme (2.6%) in with no found cause of erythroderma may reveal three cases each, pityriasis rosea (1.7%) and an undiagnosed cutaneous T-cell lymphoma pytiriasis rubra pilaris (1.7%) in two cases each, (CTCL). In these cases, histopathology results and spongiotic dermatitis (0.9%) and ichthyosis were nonspecific, and as such were diagnosed as (0.9%) in one case each. Nonspecificidiopathic erythroderma. DERMATOLOGY • April 2020 EMJ The relationship between a drug and definition.2,4,5 Similar results were indicated in this drug-induced erythroderma was decided from a study. Drug-induced erythrodermic cases were carefully conducted anamnesis with the patient, associated with a shorter hospitalisation period monitoring the intake of a suspected drug in while the erythrodermic psoriasis cases marked the days prior to the onset of erythroderma the longest hospitalisation periods observed.
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