Case for diagnosis. Erythroderma as manifestation of hypereosinophilic syndrome

Merlotto, Maira Renata; Cantadori, Lucas Oliveira; Sakabe, Delmo; Miot, Hélio Amante

doi:10.1590/abd1806-4841.20187419

Cited by 6 publications

(8 citation statements)

References 11 publications

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“…We cite, for example, pruritic erythematous papules, urticaria, angioedema, dermographism, mouth and genital ulcers, erythema annulare centrifugum, acral bullae, and erythroderma. Histopathological examination of the skin lesion is generally nonspecific, with inconstant eosinophilic infiltration [2].…”

Section: Discussionmentioning

confidence: 99%

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A Lymphocytic Variant of Hypereosinophilic Syndrome Presenting With Isolated Cutaneous Manifestations

Idrissi¹,

Hali²,

Fatoiki³

et al. 2022

Cureus

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Hypereosinophilic syndrome (HES) is a rare disease defined by a persistent increase in eosinophilic cells associated with organ damage without any underlying cause. Three variants have been identified: myeloproliferative, lymphocytic, and idiopathic syndrome. The symptomatology is variable because it depends on the involvement of different organs, including the circulatory system, skin, lungs, digestive tract, peripheral and central nervous system, and eyes. Although cutaneous involvement may frequently reveal an underlying HES. We report a case of a 52-year-old man with a 12-year history of skin lesions with intense pruritus. On examination, the patient presented with erythroderma, extensive infiltrated plaques, excoriated itchy papules, palmoplantar pustules, ear infiltration, periorbital edema, and cutaneous xerosis.Histopathology showed lichenoid dermatitis without epidermotropism. Inflammatory infiltrates in the dermis were principally composed of eosinophilic cells and lymphocytes. Serum immunoglobulin E and peripheral blood immunophenotyping showed atypical T lymphocyte proliferation CD4+CD3-, and clonal TCR gene rearrangement was in favor of lymphocytic HES. The patient was treated with prednisone (1 mg/kg/day) and pegylated interferon alpha with improvement.This case shows that HES should be suspected in patients with dermatological lesions and hypereosinophilia, without obvious cause. Elimination of secondary causes of eosinophilia, evaluation of deep organ involvement, and cytogenetic studies to assess prognosis are paramount. Pegylated interferon alpha 2a may be an effective treatment option for steroid-resistant or refractory patients with lymphocytic HES.

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Section: Discussionmentioning

confidence: 99%

“…HES is classified as myeloproliferative, lymphocytic, and idiopathic [2]. The lymphocytic variant of HES (L-HES) is a distinct subtype, characterized by aberrant clonal T-cell populations that produce eosinophilpromoting cytokines [3].…”

Section: Introductionmentioning

confidence: 99%

A Lymphocytic Variant of Hypereosinophilic Syndrome Presenting With Isolated Cutaneous Manifestations

Idrissi¹,

Hali²,

Fatoiki³

et al. 2022

Cureus

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“…Hypereosinophilic syndrome should also be considered with the presentation of erythroderma, as it has been reported in several cases. [41][42][43] Other cutaneous signs of internal organ dysfunction may be evident on physical examination. Splinter hemorrhages of the nails and Janeway lesions secondary to HES-related thromboembolic disease have also been described.…”

Section: Clinical Presentation Of Hesmentioning

confidence: 99%

An Approach to Hypereosinophilic Syndrome Presenting With Cutaneous Features

Fourzali¹,

Yosipovitch²

2022

Dermatitis

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Hypereosinophilic syndrome (HES) is a heterogeneous group of disorders characterized by persistent peripheral hypereosinophilia and eosinophilia-mediated tissue damage. Hypereosinophilic syndrome can have life-threatening effects on multiple organ systems but may initially, or in some cases solely, present with skin lesions. The clinical presentation of HES in the skin represents a diagnostic challenge for the dermatologist, because cutaneous manifestations are highly variable and may be mistaken for several dermatologic conditions. Once peripheral and tissue eosinophilia is diagnosed, the differential diagnosis is quite broad, spanning hematoproliferative disorders, infectious diseases, drug reactions, and many others. Workup and management may also present a challenge, because the prospect for organ system involvement in those with apparent skin-limited disease is unclear. This article provides a dermatology-centered approach to HES and provides a reference for the differential diagnosis, workup, and management of this complex disorder.

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“…Dermatologists should consider pruritic, erythematous papules, urticaria, angioedema, dermographism, oral and genital ulcers, centrifugal annular erythema, acral bullae, and erythroderma. Histopathologic examination of the skin lesion is usually nonspecific, with viable eosinophilic infiltration (12). For the initial diagnosis of our patient, we ruled out the above-mentioned disorders.…”

mentioning

confidence: 99%

“…The diagnosis of HES is often delayed due to its pleomorphic dermatological manifestations and insidious evolution. It is thus crucial that in all cases of erythroderma, HES should be considered as a differential diagnosis (12).…”

mentioning

confidence: 99%