Sweet's syndrome: diagnostic criteria revisited

Abstract

Summary The diagnosis of Sweet's syndrome (SS) is based on a set of criteria that requires the presence of two major and at least two minor criteria. In some cases, however, the diagnosis is not as straightforward due to the absence of certain criteria. The objective of the present study was to review the clinical, histopathological, and laboratory features of the current diagnostic criteria for SS, and to evaluate their validity in the cases reported in the literature as well as in 40 patients treated at our institution. Our comprehensive review of the current criteria for SS reveals that the two major criteria have been consistently present in all cases – including ours – since the first description of SS in 1964. With regard to the minor criteria, on the other hand, there has been marked variability between different studies, and many cases failed to fulfill the requirement of showing two minor criteria. In order to simplify the diagnosis, avoid misdiagnosis, and allow for prompt treatment, we propose two sets of revised diagnostic criteria for SS. The first set comprises constant clinical and histopathological features that must be present and are by themselves sufficient for the diagnosis of SS to be established. The second set includes variable features whose absence does not warrant ruling out SS.

Upper Respiratory Infection Followed by Concurrent Sweet’s Syndrome and Erythema Nodosum

“…It appears mostly in women between age 30-60 years [3][4][5] . There are major and minor criteria for diagnosis of SS ( Figure 1) 6,7 . For the treatment of patients with Sweet's syndrome systemic corticosteroids have been considered the primary option, but iodide or colchicine can also result in rapid resolution symptoms and lesions.…”

Ways to understand neutrophilic dermatoses

“…Neutrophilic dermatoses are a heterogeneous group of diseases characterized by numerous neutrophilic granulocytes in a sterile cutaneous inflammation. The best known ones are probably Sweet's syndrome (acute febrile neutrophilic dermatosis), whose diagnostic criteria are critically evaluated in this issue of JDDG , pyoderma gangrenosum and pustular skin diseases such as acute, generalized exanthematous pustulosis (AGEP) . Several other (rare) diseases are also included.…”

“…follow this path using the example of Sweet's Syndrome. The authors succeed in adapting established major and minor criteria and making them more stringent and simpler .…”

Wege zum Verständnis neutrophiler Dermatosen

“…Neutrophile Dermatosen sind eine heterogene Gruppe von Erkrankungen, welche sich durch zahlreiche neutrophile Granulozyten in einer sterilen kutanen Entzündung auszeichnen. Am bekanntesten sind wahrscheinlich das Sweet Syndrom (akute febrile neutrophile Dermatose), dessen diagnostische Kriterien in dieser Ausgabe des JDDG kritisch evaluiert werden , das Pyoderma gangraenosum und pustulöse Hautkrankheiten wie die akute generalisierte exanthematische Pustulose (AGEP) . Etliche andere (seltene) Krankheiten gehören ebenfalls dazu.…”

“…am Beispiel des Swees‐Syndroms. Es gelingt den Autoren, etablierte Major‐ und Minor‐Kriterien anzupassen und stringenter sowie einfacher zu gestalten .…”