Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Feb 16, 2015; 3(2): 196-198
Published online Feb 16, 2015. doi: 10.12998/wjcc.v3.i2.196
Sweet syndrome and differentiation syndrome in a patient with acute promyelocytic leukemia
Guillermo Solano-López, Mar Llamas-Velasco, Maria José Concha-Garzón, Esteban Daudén
Guillermo Solano-López, Mar Llamas-Velasco, Maria José Concha-Garzón, Esteban Daudén, Department of Dermatology, Hospital Universitario de la Princesa, 28006 Madrid, Spain
Author contributions: Solano-López G contributed to manuscript writing and patients data collection; Llamas-Velasco M contributed to main idea, patients data collection; Concha-Garzón MJ contributed to patients data collection; Daudén E contributed to supervision.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Guillermo Solano-López, MD, Department of Dermatology, Hospital Universitario de la Princesa, C/Diego de León 62, 28006 Madrid, Spain. guitje1@hotmail.com
Telephone: +34-91-5202433 Fax: +34-91-5202435
Received: June 9, 2014
Peer-review started: June 10, 2014
First decision: July 18, 2014
Revised: October 27, 2014
Accepted: November 17, 2014
Article in press: November 19, 2014
Published online: February 16, 2015
Processing time: 241 Days and 11.8 Hours
Abstract

The differentiation syndrome is an inflammatory reaction with increased capillary permeability that occurs in up to 25% of patients with acute promyelocytic leukemia treated with all-trans retinoic acid. A 50-year-old man with acute promyelocytic leukemia underwent chemotherapy with idarubicin and all-trans retinoic acid. On day +21 the patient developed pruritic prepatelar papules as well as several 10 mm subcutaneous nodules in both thighs accompanied by persistent fever. On the day +25 the patient presented with bilateral pulmonary crackles, infiltrates in the right lower lobe and severe hypotension which required dopamine infusion. Biopsy of one of the thighs nodules was performed. A Sweet syndrome associated to a differentiation syndrome was suspected. All-trans retinoic acid therapy was discontinued and dexamethasone was administered. In 48 h the patient showed remission of the fever and the infiltrates and the skin lesions acquired a residual aspect. It is debatable whether these two syndromes are distinct entities with common mechanisms or whether they are poles of the same spectrum. Dermatologists and hematologists must be aware of these two syndromes and its pathophysiologic association.

Keywords: Differentiation syndrome; Sweet syndrome; Acute promyelocytic leukemia; All-trans retinoic acid

Core tip: It is debatable whether the differentiation syndrome and the sweet syndrome are distinct syndromes with common mechanisms or whether they are poles of the same spectrum. We believe that there may be more cases of differentiation presenting with skin sweet syndrome lesions, which are underdiagnosed, overshadowed by the critical state of these patients. Dermatologists and hematologists must be aware of these two syndromes and its pathophysiologic association. It is very likely that these two specialties are staring the same phenomenon from two different points of view.