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

Acessibilidade / Reportar erro

Brasil

Anais Brasileiros de Dermatologia

Español English

Brasil

Español English

sumário « anterior atual seguinte »

Sumário

What is your diagnosis? • An. Bras. Dermatol. 93 (3) • May-Jun 2018 • https://doi.org/10.1590/abd1806-4841.20187419 copy

Case for diagnosis. Erythroderma as manifestation of hypereosinophilic syndrome^* * Work conducted at Hospital das Clínicas da Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (FMB-Unesp), Botucatu (SP), Brazil.

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract:

Hypereosinophilic syndrome is defined as persistent eosinophilia (>1500/µL for more than six months) associated with organ involvement, excluding secondary causes. It is a rare, potentially lethal disease that should be considered in cutaneous conditions associated with hypereosinophilia. We report a case of erythroderma as a manifestation of hypereosinophilic syndrome. A 36-year-old male with no comorbidities presented progressive erythroderma, pruritus, peripheral neuropathy, and eosinophilia in the previous seven months. No mutations were found in FIP1L1/PDGFRA. Patient experienced rapid remission in response to oral prednisone and hydroxyurea. Cutaneous manifestations may be the only evidence of hypereosinophilic syndrome. Genotyping excludes myeloproliferative disease, thereby orienting treatment and prognosis.

Keywords:
Eosinophilia; Polyneuropathies; Dermatitis, Exfoliative

Original diagnostic criteria, Chusid et at.¹1 Simon HU, Rothenberg ME, Bochner BS, Weller PF, Wardlaw AJ, Wechsler ME, et al. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol. 2010;126:45-9.	Modification to diagnostic criteria for HES by Simon et at.¹1 Simon HU, Rothenberg ME, Bochner BS, Weller PF, Wardlaw AJ, Wechsler ME, et al. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol. 2010;126:45-9.	Comments
1. Peripheral eosinophilia > 1500/µL persisting for more than six consecutive months.	Persistent eosinophilia. Arbitrary value of 1500/µL is not mandatory	Presence of tissue infiltration with eosinophils leading to tissue damage is more important than setting a laboratory value
2. Rule out secondary causes of hypereosino- philia after extensive clinical workup	Peripheral eosinophilia should be confirmed, but its persistence for more than six months is no longer mandatory	In symptomatic patients with confirmed eosinophilia and organ involvement, proceed to treatment to prevent organ damage; any duration is significant, even less than six months
3. Symptomatic organ involvement secondary to hypereosinophilia	Signs and/or symptoms of organ involvement are not mandatory	At the time of diagnosis, it is very difficult to predict evolution in asymptomatic patients. Some may be asymptomatic at first evaluation and either remain asymptomatic or evolve due to tissue damage or organ involvement

Subtypes	Characteristics	Treatment	Prognosis
Myeloproliferative(HES-M)	Tyrosine kinase activation by FIP1L1 and PDGFRA gene fusion, eosinophilia due to clonal proliferation of myeloid precursors	Little response to systemic corticosteroids. More response to hydroxyurea, interferon, and chemotherapy - busulfan, chlorambucil, and vincristine.Imatinib is the drug of choice for patients with positive F/P mutation	Survival depends on severity of tissue injury, especially the degree of cardiac involvement. Risk of development of malign myeloproliferative disease
Idiopathic (HES-I)	T-cell polyclonal expansion unregulated production of Th2 cytokines (IL-5 and/or IL-3)	Good response to systemic corticosteroids alone or in combination with hydroxyurea, mepolizumab, interferon-a, or tofacitinib	Chronic, benign condition with indolent clinical course. Occasional development of lymphoma
Lymphocytic/Secondary (HES-L)	Polyclonal expansion of anomalous T-cells (CD3-CD4+) and unregulated production of Th2 cytokines (especially IL-5)	Adequate control with systemic corticosteroids alone or in combination with hydroxyurea, mepolizumab, or tocilizumab	Chronic recurrent.Occasional development of lymphoma

Sociedade Brasileira de Dermatologia Av. Rio Branco, 39 18. and., 20090-003 Rio de Janeiro RJ, Tel./Fax: +55 21 2253-6747 - Rio de Janeiro - RJ - Brazil
E-mail: revista@sbd.org.br

Acompanhe os números deste periódico no seu leitor de RSS

[1] Mailing address: Hélio Amante Miot. E-mail: heliomiot@fmb.unesp.br

Brasil

Brasil

Case for diagnosis. Erythroderma as manifestation of hypereosinophilic syndrome* * Work conducted at Hospital das Clínicas da Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (FMB-Unesp), Botucatu (SP), Brazil.

Abstract:

Case for diagnosis. Erythroderma as manifestation of hypereosinophilic syndrome^* * Work conducted at Hospital das Clínicas da Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (FMB-Unesp), Botucatu (SP), Brazil.