Published online Feb 16, 2015. doi: 10.12998/wjcc.v3.i2.196
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
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.
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.