Clonality in the setting of Sweet’s syndrome and pyoderma gangrenosum is not limited to underlying myeloproliferative disease

Abstract

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.

Sweet's syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis

“…These findings demonstrated that clonality of the neutrophilic infiltrate in Sweet's syndrome skin lesions is not exclusively restricted to patients with an established myeloproliferative disease. The significance of the clonal neutrophilic infiltrate in Sweet's syndrome patients without an underlying myeloid dysplasia remains to be determined; however, the investigators speculated that it may have some implications regarding the pathogenesis of sterile neutrophilic infiltrates [429]. …”

Unusual Causes of Cutaneous Ulceration

“…However, it is postulated to be the result of neutrophil dysfunction, which may either consist of a defect in chemotaxis or be due to neutrophil hyper- reactivity. Also, clonality of T- cells has been detected in patients with PG in the absence of myeloproliferative disease, possibly indicating an excessive response to antigenic stimuli 19–20…”

Acute promyelocytic leukemia‐associated Sweet's syndrome mimicking an axillary abscess: A case report with review of literature

“…23 (5) SS is a form of indolent, localized, cutaneous neutrophilic dyscrasia showing clonal restriction. 24 Treatment of MASS is treatment of underlying malignancy. Systemic corticosteroids may be administered.…”