Initially lymphocytic Sweet's syndrome in male patients with myelodysplasia: a distinguished clinicopathological entity? Case report and systematic review of the literature

Acta Haematol. 2014;132(2):220-5. doi: 10.1159/000357933.


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.

Conclusion: 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.

Publication types

  • Case Reports
  • Review
  • Systematic Review

MeSH terms

  • Aged
  • Anemia, Refractory, with Excess of Blasts / complications
  • Anemia, Refractory, with Excess of Blasts / pathology
  • Biopsy
  • Chromosomes, Human, X / genetics
  • Chromosomes, Human, Y / genetics
  • Disease Progression
  • Humans
  • Lymphocytes / pathology
  • Male
  • Middle Aged
  • Models, Immunological
  • Myelodysplastic Syndromes / complications*
  • Myelodysplastic Syndromes / pathology
  • Neutrophil Infiltration
  • Receptors, Granulocyte-Macrophage Colony-Stimulating Factor / genetics
  • Receptors, Granulocyte-Macrophage Colony-Stimulating Factor / physiology
  • Sex Factors
  • Skin / pathology
  • Sweet Syndrome / etiology*
  • Sweet Syndrome / genetics
  • Sweet Syndrome / immunology
  • Sweet Syndrome / pathology


  • Receptors, Granulocyte-Macrophage Colony-Stimulating Factor