Association of pyoderma gangrenosum and sterile osteomyelitis in a patient having myelodysplastic syndrome with der(1;7)(q10;q10)

Abstract

Neutrophilic dermatoses such as Sweet's disease and pyoderma gangrenosum (PG) are occasionally associated with myelodysplastic syndrome (MDS). We present here a 67-yr-old male having PG and sterile osteomyelitis in association with underlying MDS (refractory anemia) and Crohn's disease. To establish the diagnosis of MDS, sternal bone marrow puncture was performed, which showed chromosomal abnormality containing der(1;7)(q10;q10). After the puncture, he suffered from gradually progressive skin ulceration, flare, and bone pain. Magnetic resonance imaging (MRI) of the sternum showed severe inflammation in the sternum and the overlying subcutaneous tissue. All of the cultures obtained from the wound were negative for both bacteria and fungus. Biopsy was performed from the antero-sternal skin lesion, which showed epidermal ulceration with prominent infiltration of neutrophils. He was thus diagnosed as having PG and sterile osteomyelitis, and was treated with prednisolone, which completely resolved the symptoms. We consider that the bone marrow aspiration in the present patient provoked PG and sterile osteomyelitis. As was previously reported by others, certain chromosomal abnormalities in MDS may be related with the development of neutrophilic dermatoses.

Pyoderma gangrenosum in a patient with myelodysplastic syndrome followed by possible extracutaneous manifestations in the gallbladder, liver, bone and lung

“…Pyoderma gangrenosum with systemic involvement may occur in patients with hematological malignancies; these include hepatic aseptic abscesses, 1 osteomyelitis 1,2 and pulmonary involvement 2–5 . Although acute cholecystitis associated with PG has not been reported to date, a number of cases of acute acalculous cholecystitis associated with PG‐like symptoms have appeared in the published work 6–8 .…”

Superficial granulomatous pyoderma associated with chronic osteomyelitis

“…Although the full significance of the immunohistochemical profile is unclear, we know that CD99 has been demonstrated in subpopulations of fibroblasts. 9 We hypothesize that these findings in LGFMS are compatible with a CD34-⁄CD99 + subpopulation of fibroblasts.…”

“…According to other authors, bone marrow aspiration can provoke PG and sterile osteomyelitis. 9 We feel that the osseous lesion is unlikely to be a direct extension of the cutaneous superficial form of PG. On the other hand, it may be presumed that SGP and osteomyelitis in the same patient are associated with the same underlying process.…”

Pyoderma gangrenosum: an expert commentary

“…These extracutaneous lesions are also sterile neutrophilic abscesses, which are often misdiagnosed as infections. Extracutaneous involvement has been noted in bone; termed multifocal sterile recurrent osteomyelitis [19,20], cornea [21], lung [22], liver and spleen [23], heart [24], muscles [25] and the CNS [26]. All of these manifestations are rare, but pulmonary involvement is most common.…”