Cutaneous leukaemic infiltrations in a patient with previously undiagnosed myelodysplastic syndrome

Abstract

We report the rare case of a patient with leukaemia cutis first presenting only on the hand and fingers and then subsequently spreading over the trunk and face. The lesions heralded the transformation of a previously undiagnosed myelodysplastic syndrome type RAEB (refractory anaemia with blast excess) into frank myeloid leukaemia. The haematological disease was first detected by the dermatohistopathologist. This case underlines the need to look meticulously for skin changes and perform early skin biopsies in haematological patients, as the skin can reveal the first clinical signs of an otherwise not evident bone marrow disorder. Leukaemia cutis as the initial clinical presentation of a transforming myelodysplastic syndrome type RAEB into acute myeloid leukaemia has been reported only very rarely.

Stasis dermatitis-like leukaemic infiltration in a patient with myelodysplastic syndrome

“…The temporal association between leukaemia cutis and acute leukaemia is highly variable; in established acute leukaemia, it tends to develop late. In contrast, when leukaemia cutis develops in association with chronic myeloid leukaemia (CML) or other myeloproliferative disorders, it is usually a sign of impending blastic transformation 5–7 . In the case of MDS, unlike de novo AML, leukaemia cutis frequently develops before or concomitantly with identifiable leukaemic transformation in blood or in bone marrow, 5–7 as in our case.…”

“…In contrast, when leukaemia cutis develops in association with chronic myeloid leukaemia (CML) or other myeloproliferative disorders, it is usually a sign of impending blastic transformation 5–7 . In the case of MDS, unlike de novo AML, leukaemia cutis frequently develops before or concomitantly with identifiable leukaemic transformation in blood or in bone marrow, 5–7 as in our case. In the series reported by Longacre and Smoller, the interval between skin‐biopsy diagnosis and the appearance of leukaemia varied from 3 weeks to 20 months (mean 6 months) 5 …”

Rosazea-artige kutane Infiltration des Gesichtes (Facies leontina) bei chronisch-lymphatischer Leukämie

“…Erniedrigt waren: Hämoglobin 12,9 mg/dl, Hämatokrit 39,5 % (40 ± 50 %), Erythrozyten 4,3 10 6 /ml (4,5 ± 5,9 10 6 /ml, Thrombozyten 116 000/ml (150 000 ± 350 000/ml), neutrophile Plaques, Makulae, Ulzerationen oder palpable Purpura [3]. In einzelnen Fällen sind kutane Infiltrate das primäre Symptom, das zur Diagnosestellung führt [4]. Diffuse erythematöse, knoti− ge, rosacea− oder facies leontina−artige Infiltrate im Gesicht sind bisher nur in 2 Fällen beschrieben worden, wobei die Patienten im einen Fall eine zugrundeliegende T−Zell−Leukämie [5] und im anderen Fall eine akute myeloische Leukämie [6] Fraglich ist, ob in unserem Fall nicht ursprünglich eine Rosazea bestand, die ± ähnlich wie in der Literatur für Herpesviren be− schrieben [7] ± als Triggerfaktor für die Einwanderung der B−Lymphozyten in die Haut fungierte.…”