The MDS Beacon™

Researchers from several French universities have observed that patients with recurring Sweet syndrome are highly likely to be diagnosed with myelodysplastic syndromes (MDS).

Though the exact association of Sweet syndrome with bone-marrow-related diseases is unknown, researchers concluded the changing composition of skin biopsy samples was able to predict the onset of MDS, thus heightening the correlation between the two conditions.

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a skin condition characterized by series of small bumps that appear predominantly on the face, neck, back, and upper limbs. These characteristic bumps can grow in size and spread in small clusters, becoming tender lesions.

Though the exact cause is unknown, patients usually have an initially high lymphocyte count (number of specific white blood cells in the immune system), which progresses to higher neutrophil counts (another type of white blood cell) in later stages.

Researchers established this association after analyzing the biopsy results of nine patients. Patients were all male, of median age 65, and had Sweet syndrome. For each patient, bumps healed without treatment and later returned. After relapsing several times, patients gradually developed lower levels of red blood cells and lymphocytes. While lymphocyte levels decreased, neutrophils increased in the body. All patients were eventually diagnosed with MDS a median of 3.5 years after their first skin symptoms.

The follow-up, which ranged from three to ten years, showed that MDS could be controlled by blood transfusions or erythropoietin therapy, which is the addition of a substance naturally produced by the kidneys that stimulates the bone marrow to make red blood cells. Treatments also prevented progression to acute myeloid leukemia in all nine patients. Eight of the nine patients died after being diagnosed with MDS from various causes, a median of 5.5 years after developing Sweet syndrome. These cases demonstrated that a poor prognosis for Sweet syndrome may be associated with MDS.

Twenty percent of Sweet syndrome cases are associated with MDS or other blood development conditions. Patients who have both conditions often show signs of premature death of living tissue as well as immature bone marrow cells.

Though researchers have not yet determined why the skin disease appears years before MDS is identified, the presence of Sweet syndrome may help in the early diagnosis and treatment of MDS.

More information can be found in the Archives of Dermatology.