Dermal necrosis and chromosome Iq abnormality in a man with a familial myeloproliferative disorder

A 33 year old man, with pre-existing psoriasis and a family history of multiple occurrence of acute myeloid leukemia and other myeloproliferative disorders, developed steroid-responsive ulcerating skin lesions, pancytopenia, marrow hypoplasia, hyperglobulinemia and polyarthritis. An abnormal karyotype (47,XY + i(1q] was detected in the bone marrow, and comparison with a case previously reported by Lee et al. Suggested that this abnormality may be significant. His sister, who developed chronic leucocytoclastic vasculitis, had pre-existing psoriasis, variable mild leucopenia and marrow dysplasia. Review of available records of other affected family members documented the occurrence of steroid responsive pancytopenia, knee swelling and terminal lipoid pneumonia in a first cousin. Four other relatives died with acute myeloblastic leukemia and another died with myelofibrosis. Two healthy first degree relatives were subjected to laboratory investigations with essentially negative findings.