Clonality in the setting of Sweet's syndrome and pyoderma gangrenosum is not limited to underlying myeloproliferative disease.
Academic Article
Overview
abstract
BACKGROUND: The neutrophilic dermatoses encompass, among others, Sweet's syndrome (SS) and pyoderma gangrenosum (PG), which are associated with underlying systemic diseases including myeloid dyscrasias. METHODS: On skin biopsies from 16 patients with biopsy-proven SS and/or PG, we performed an X-inactivation assay to detect clonal restriction of neutrophils. There were two patient categories based on known diseases at the time of diagnosis: patients with myeloproliferative disease and patients without myeloproliferative disease. RESULTS: Among seven patients with acute myelogenous leukemia and two with myelodysplastic syndrome, clonal restriction was found in five; three were homozygous, precluding analysis. Among the seven control patients, infiltrates were clonally restricted in five; one was polyclonal and the other was homozygous for the allele, precluding analysis. Of the five patients with clonally restricted infiltrates, one was subsequently diagnosed with myelodysplasia, one had unexplained neutropenia and an additional patient developed breast cancer. Overall, the incidence of clonality in both groups was the same, averaging 81%. CONCLUSION: These findings suggest that clonality in neutrophilic dermatoses, while characteristic of underlying myeloid dyscrasia, is not observed exclusively in the setting of myeloproliferative diseases. The significance of clonal neutrophilic infiltrates unassociated with myeloproliferative disease is unclear, but it may have some implications regarding the pathogenesis of sterile neutrophilic infiltrates. Clonality is well described in the setting of lymphomatoid hypersensitivity, reflecting an overzealous response to antigenic stimuli. One could speculate a similar mechanism operational in cases of apparently reactive SS/PG associated with monoclonality; a localized form of cutaneous neutrophilic dyscrasia is also possible.
