PF18. Pyoderma gangrenosum asociata cu hemopatii maligne
Inclusa in grupul dermatozelor de tip vasculitic, pyoderma gangrenosum/PG cu diferitele ei forme clinice de manifestare se poate asocia cu afectiuni diverse: infectioase, cronice inflamatorii, autoimune, maligne. Prezentam cazurile a 2 pacienti la care PG s-a asociat cu: sindrom hemofagocitar coexistent cu histocitoza maligna intr-un caz si cu limfom T celular in alt caz. Primul pacient, de 45 de ani a prezentat leziuni cutanate ulcerative severe, predominant pe membrele inferioare. Dupa 2 luni de evolutie in care nu s-au putut evidentia alte afectiuni in afara unei hepatite cronice neinfectioase, bolnavul a manifestat o alterare rapida a starii generale, febra, hepatosplenomegalie, pancitopenie. Punctia medulara a pus in evidenta aspect de hemofagocitoza in cadrul unei histocitoze maligne. Examenul histopatologic cu tanat a relevat doar aspectul de PG. Evolutia cazului a fost fatala in 3 saptamani de la manifestarea panocitopeniei. Al doilea pacient, de 51 ani a manifestat cutanat leziuni pustuloase si ulcerative diseminate. Controlul hematologic periferic si medular nu a evidentiat anomalii in timp ce biopsia cutanata a pus in evidenta aspectul sugestiv pentru un limfom cutanat cu infiltrat limfoid epidermotrop. Leziunile cutanate au raspuns complet doar la polichimioterapie. Consideram importanta investigarea minutioasa a cazurilor de PG, cu o atentie deosebita in directia unui posibil sindrom hemofagocitar, asociere descrisa intr-un singur caz pana in prezent, sau a unui limfom incipient care nu poate fi uneori depistat nici prin punctie medulara.
PF18. Pyoderma Gangrenosum Associated With Malignant Haemopathies
Part of the vasculitic dermatoses, PG with its different clinical forms (ulcerous, pustulous, bullous and vegetant) can be associated with infectious, inflammatory, autoimune, malignant disorders. The paper presents two cases of PG associated with: haemofagocytic syndrome correlated with malignant histiocytosis, with one male patient and with T-cell cutaneous lymphoma in the other case. The first patient, aged 45, exhibited extensive cutaneous ulcerations mainly on the legs. After a two month evolution without any clinical or laboratory evidence of another disease except for a chronic hepatitis, the patient manifested a rapid deterioration, fever, hepatosplenomegaly, pancytopenia. Bone marrow showed malignant histiocytosis- based haemofagocytosis. Skin biopsy specimen revealed PG alterations. Three weeks after the pancytopenia onset the patient died. The second patient, 51 years old, exhibited disseminated pustulous and ulcerative cutaneous lesions. the peripheral and bone marrow blood count did not show abnormal values while skin biopsy presented an epidermotropic lymphoid infiltrate pointing to a cutaneous lymphoma. the lesion responded completely only to polychemotherapy. We consider that a detailed investigation of PG cases is important, special attention being given to a possible haemocytophagic syndrome, once published until now, or an incipient T-cell lymphoma, sometimes not highlighted even by medullary puncture.