2018
DOI: 10.5336/caserep.2018-60781
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Ulcerative Type Pyoderma Gangrenosum in Primary Sjogren's Syndrome

Abstract: yoderma gangrenosum (PG) is a rare neutrophilic dermatosis which presents as an inflammatory and ulcerative disorder of the skin. Although the etiology of PG is unknown, the impaired neutrophil chemotaxis plays an important role in the pathophysiology of PG. 1,2 Sjogren's syndrome (SjS) is a systemic, inflammatory disease characterized by lymphocytic infiltration of the exocrine organs, 3 Dryness of the skin, cutaneous vasculitis and Raynaud's phenomenon can occur in SjS. However, PG is not a common skin findi… Show more

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Cited by 1 publication
(3 citation statements)
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“…The co-occurrence of primary Sjögren's syndrome with pyoderma gangrenosum is an infrequent phenomenon documented in the literature. Among the seven previously reported cases (6)(7)(8)(9)(10)(11)(12), five were women, and the majority of cases occurred between the fourth and sixth decades of life. Except for a 25-year-old female patient (11), all cases were diagnosed with pSS before the occurrence of PG.…”
Section: Discussionmentioning
confidence: 97%
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“…The co-occurrence of primary Sjögren's syndrome with pyoderma gangrenosum is an infrequent phenomenon documented in the literature. Among the seven previously reported cases (6)(7)(8)(9)(10)(11)(12), five were women, and the majority of cases occurred between the fourth and sixth decades of life. Except for a 25-year-old female patient (11), all cases were diagnosed with pSS before the occurrence of PG.…”
Section: Discussionmentioning
confidence: 97%
“…Although no standard treatment is defined in the guidelines on this issue, most patients in the literature responded well to methylprednisolone (5 to 60 mg/day according to the severity of clinical symptoms) and azathioprine (20 mg/kg/day) and/or hydroxychloroquine (200 mg/day) combination (6,7,9,10,12). A patient with rheumatoid arthritis and secondary SjS was treated with rituximab and methotrexate (8), while cyclosporine and methylprednisolone combination was the chosen therapy for another patient (11).…”
Section: Discussionmentioning
confidence: 99%
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