Initially lymphocytic Sweet's syndrome in male patients with myelodysplasia: a distinguished clinicopathological entity? Case report and systematic review of the literature.
BACKGROUND
Sweet's syndrome (SS) is an acute febrile neutrophilic dermatosis. It can occur as an idiopathic, drug-induced or malignancy-associated entity. SS is also seen in patients with myelodysplastic syndrome (MDS) where it may present atypically, both clinically and histologically. In a few rare cases of MDS, lymphocytic infiltrates are the presenting feature of SS.


METHODS
MEDLINE and Scopus were the data sources for our review.


RESULTS
A clinicopathological subsetemerged of 12 male SS patients with MDS and a mean age of 67.3 years in which the initial SS lesions were lymphocytic infiltrates. However, from 0.5 to 8 years later, sequential biopsies revealed neutrophilic dermal infiltration typical of SS.


CONCLUSIONS
Initially lymphocytic infiltrates in this subset could be attributed either to an early timing of the biopsy concerning the age of the lesion or to the dysgranulopoiesis syndrome. A possible relationship between the dysfunction of the receptor of the granulocyte-macrophage colony stimulating factor, the gene of which is located on the pseudoautosomal X-Y region, may exist in MDS patients with initially lymphocytic SS. This could explain the male gender of this subset and might establish initially lymphocytic SS as a distinguished clinicopathological entity for predicting the occurrence and even the prognosis of MDS.
Nikolaos
N
Kakaletsis
Georgia
G
Kaiafa
Christos
C
Savopoulos
Fotios
F
Iliadis
Vasilios
V
Perifanis
Vasilios
V
Tzalokostas
Alexandra
A
Grekou
Angeliki
A
Giannouli
Apostolos I
AI
Hatzitolios
D000368:Aged; D000754:Anemia, Refractory, with Excess of Blasts / Q000150:complications / Q000473:pathology; D001706:Biopsy; D041321:Chromosomes, Human, X / Q000235:genetics; D041322:Chromosomes, Human, Y / Q000235:genetics; D018450:Disease Progression; D006801:Humans; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D018448:Models, Immunological; D009190:Myelodysplastic Syndromes / Q000150:complications* / Q000473:pathology; D020556:Neutrophil Infiltration; D016187:Receptors, Granulocyte-Macrophage Colony-Stimulating Factor / Q000235:genetics / Q000502:physiology; D012737:Sex Factors; D012867:Skin / Q000473:pathology; D016463:Sweet Syndrome / Q000209:etiology* / Q000235:genetics / Q000276:immunology / Q000473:pathology
D016187:Receptors, Granulocyte-Macrophage Colony-Stimulating Factor