Abstract
Cutaneous manifestations in hematologic malignancies, especially in leukemia, are not common and may be very variable. Here we report a very unusual case of a patient (female, 70 years old) who was admitted to the hospital in 2016 because of skin lesions on the face, the trunk of the body and the extremities. She had a history of breast cancer in the year 2004 (pT1b, pN0, cM0, L0, V0, R0) which had been resected and treated with adjuvant radiation and chemotherapy (cyclophosphamide, methotrexate, 5-fluorouracile) as well as psoriasis treated with methotrexate and cyclosporine. Because of mild cytopenia a bone marrow aspirate/biopsy was performed showing myelodysplastic syndrome (MDS) with multilineage dysplasia. Cytogenetic review revealed a complex aberrant karyotype denoting adverse outcome. Simultaneously, a skin biopsy could confirm leukemic skin infiltration. Consequently, a therapy with azacitidine was started. After the first cycle the patient developed severe pancytopenia with a percentage of 13% peripheral blasts (previously 0–2%) as well as fever without evidence for infection which was interpreted as progressive disease. Therefore, the therapeutic regimen was changed to a biomodulatory therapy consisting of low-dose azacitidine 75 mg/day (given sc d1-7 of 28), pioglitazone 45 mg/day per os, and all-trans-retinoic acid (ATRA) 45 mg/m2/day per os. After cycle 1 of this combined biomodulatory therapy the patient showed hematologic recovery; besides a mild anemia (hemoglobin 11.1 g/dl) she developed a normal blood count. Moreover, the cutaneous leukemic infiltrates which had been unaffected by the azacitidine ameliorated tremendously after 2 cycles resulting in a complete remission of the skin lesions after cycle 6. In conclusion, we report a very unusual case with cutaneous infiltrates being the first clinical manifestation of hematologic disease, preceding the development of acute myeloid leukemia. While azacitidine alone was ineffective, a combined biomodulatory approach resulted in a complete remission of the cutaneous manifestation.
Highlights
Clinical PresentationIn May 2016 a 70-year-old female patient was admitted to the hospital suffering from multiple skin lesions which primarily appeared on the chest, sacrum and the face 3 months ago
Since the laboratory work-up still revealed mild cytopenia a bone marrow biopsy was performed in July 2016 confirming myelodysplastic syndrome with multilineage dysplasia and ringsideroblasts (MDS-RS-MLD): hypercellular marrow with dysplasia in all lineages and 36% ringsideroblasts
leukemic skin (LC) occur in ∼3% of patients with acute myeloid leukemia (AML) (Agis et al, 2002), and less frequently in MDS (Patel et al, 2012); with most frequent association in AML with acute myelomonocytic and monocytic differentiation (Kaddu et al, 1999; Pont et al, 2001)
Summary
In May 2016 a 70-year-old female patient was admitted to the hospital suffering from multiple skin lesions which primarily appeared on the chest, sacrum and the face 3 months ago. Topic as well systemic therapy with (high dose) corticosteroids had been ineffective in the treatment. A skin biopsy had already been performed showing no evidence for malignancy or autoimmune disease. A broad laboratory work-up showed no pathologies besides mild leukopenia (leukocytes 3.8/nl), mild anemia (11 g/dl), and a positive ANA titer (1:160). Re-biopsy of the skin on the right upper arm demonstrated interface dermatitis consistent with the diagnosis of erythema exsudativum, drug rash or dermatomyositis. Chest x-ray and ultrasound of the abdomen showed no pathological findings. A therapy with topic agents as well as systemic corticosteroids (prednisolone 1 mg/kg) was (re-)started leading to minimal improvements
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