Pyoderma gangrenosum: Intractable leg ulcers in Sjogren's syndrome

Tsai, Yi-Hsuan; Huang, Chiung-Tang; Chai, Chee‐Yin; Wu, Chen-Ching

doi:10.1016/j.kjms.2013.11.005

Cited by 6 publications

(5 citation statements)

References 5 publications

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“… 27 SLE and pyoderma gangrenosum is an uncommon association. Seventeen cases of pyoderma gangrenosum associated with SLE, 9 , 28 – 41 3 cases associated with Sjögren syndrome, 42 – 44 and 1 case associated with dermatomyositis 45 have been reported in the literature. As in our case (case 1, Table 1 and Figure 2 ), in patients with SLE-associated pyoderma gangrenosum, the occurrence of pyoderma gangrenosum and the response to treatment were correlated with the SLE activity.…”

Section: Results Discussion and Literature Reviewmentioning

confidence: 99%

Neutrophilic Skin Lesions in Autoimmune Connective Tissue Diseases

et al. 2014

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The pathophysiology of neutrophilic dermatoses (NDs) and autoimmune connective tissue diseases (AICTDs) is incompletely understood. The association between NDs and AICTDs is rare; recently, however, a distinctive subset of cutaneous lupus erythematosus (LE, the prototypical AICTD) with neutrophilic histological features has been proposed to be included in the spectrum of lupus. The aim of our study was to test the validity of such a classification. We conducted a monocentric retrospective study of 7028 AICTDs patients. Among these 7028 patients, a skin biopsy was performed in 932 cases with mainly neutrophilic infiltrate on histology in 9 cases. Combining our 9 cases and an exhaustive literature review, pyoderma gangrenosum, Sweet syndrome (n = 49), Sweet-like ND (n = 13), neutrophilic urticarial dermatosis (n = 6), palisaded neutrophilic granulomatous dermatitis (n = 12), and histiocytoid neutrophilic dermatitis (n = 2) were likely to occur both in AICTDs and autoinflammatory diseases. Other NDs were specifically encountered in AICTDs: bullous LE (n = 71), amicrobial pustulosis of the folds (n = 28), autoimmunity-related ND (n = 24), ND resembling erythema gyratum repens (n = 1), and neutrophilic annular erythema (n = 1). The improvement of AICTDS neutrophilic lesions under neutrophil targeting therapy suggests possible common physiopathological pathways between NDs and AICTDs.

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Section: Results Discussion and Literature Reviewmentioning

confidence: 99%

Neutrophilic Skin Lesions in Autoimmune Connective Tissue Diseases

et al. 2014

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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%

“…Although the first findings are mostly sicca symptoms, the heterogenous nature of SjS as a multisystemic disease may affect gastrointestinal, pulmonary, neurologic, articular, and cutaneous organs (1,2). Rare examples of this multisystemic involvement include case reports in the literature declaring first clinical presentations with cerebellar degeneration, pulmonary amyloidosis, membranous nephropathy, or pyoderma gangrenosum (PG) (3)(4)(5)(6)(7)(8)(9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%

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A Rare Manifestation Of Primary Sjogren’s Syndrome With Pyoderma Gangrenosum : Case Report

Nalbant

2023

Med Sci Discov

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Objective: Sjögren’s Syndrome (SjS) is a systemic autoimmune disease typically presenting with sicca symptoms. However, a limited number of case reports in the literature have described rare initial presentations with pyoderma gangrenosum (PG). This report aims to underscore this infrequent association between PG and SjS by presenting another illustrative case. Case: This case report elucidates the diagnosis and treatment of primary Sjögren’s syndrome (pSS), focusing on the ulcerated lesion that manifested on the leg of a 54-year-old female patient with no underlying health conditions. The findings are discussed in the context of the existing literature. Discussion: SjS is a multisystemic autoimmune disease characterized by lymphocyte infiltration and damage to tissues, especially exocrine glands. Although sicca symptoms are typically the initial clinical findings, PG can rarely manifest as the primary symptom, presenting as an ulcerative skin lesion predominantly located on the lower extremities. There is currently no standard treatment recommendation in the existing guidelines for this rare condition. Conclusion: The co-occurrence of PG and SjS is uncommon, yet it may serve as a warning for associated systemic diseases, such as SjS. Treatment may involve a combination of corticosteroids and disease-modifying antirheumatic drugs (DMARDs).

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“…1,2 Additionally, the association of PG and SjS has been reported in the literature rarely. [5][6][7] Furthermore, PG may be the first clinical sign in patients with rheumatoid arthritis and SjS who having resistant leg ulcers on their lower extremities. 8,9 Although the etiopathogenesis of both diseases is quite different and poorly understood, a common unknown triggering factor may lead to dysregulation in the autoimmun system involving the activation of two distinct inflammatory pathways in the same patient.…”

Section: Discussionmentioning

confidence: 99%

Ulcerative Type Pyoderma Gangrenosum in Primary Sjogren's Syndrome

Gündoğdu¹,

Çiçek²,

Koca³

2018

Turkiye Klinikleri J Case Rep

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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 finding in SjS. In this case report, a rare association of PG and SjS has been revealed based on a review of the literature. CASE REPORTA 63-year-old female patient with SjS was admitted to rheumatology department because of an ulcer in her left leg (Figure 1). The patient had applied to the rheumatology polyclinic with dry mouth, dry eyes, diffuse joint pain four years ago. She patient's laboratory tests had revealed an increase in acute-phase response with the positivity for antinuclear antibody-indirect immunofluorescence (ANA-IFA) test (1/320 titre, speckled pattern) and anti-Sjogren's syndrome antibody (anti-SSA) (3+, ELISA) test. Schirmer's test had been bilaterally detected less than 5 mm. The minor salivary gland Turkiye Klinikleri J Case

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Pyoderma gangrenosum: Intractable leg ulcers in Sjogren's syndrome

Cited by 6 publications

References 5 publications

Neutrophilic Skin Lesions in Autoimmune Connective Tissue Diseases

Neutrophilic Skin Lesions in Autoimmune Connective Tissue Diseases

A Rare Manifestation Of Primary Sjogren’s Syndrome With Pyoderma Gangrenosum : Case Report

Ulcerative Type Pyoderma Gangrenosum in Primary Sjogren's Syndrome

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