Neutrophilic Dermatoses: An Update

Alavi, Afsáneh; Sajic, Dusan; Cerci, Felipe B.; Ghazarian, Danny; Rosenbach, Misha; Jorizzo, Joseph L.

doi:10.1007/s40257-014-0092-6

Cited by 64 publications

(68 citation statements)

References 67 publications

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“…Nevertheless, these disorders are linked by the presence of perivascular and diffuse neutrophilic infiltrates with no identifiable infectious agents. 8 Additionally, neutrophilic diseases commonly occur in association with an underlying systemic condition such as malignancy, neutropenia, rheumatologic diseases, infections, auto-inflammatory syndromes, and immunodeficiency. Moreover, neutrophilic diseases are frequently triggered by medications and pathergy, the latter being a typical sign of PG that is certainly involved in post-surgical and peristomal PG.…”

Section: Pathophysiologymentioning

confidence: 99%

“…Moreover, neutrophilic diseases are frequently triggered by medications and pathergy, the latter being a typical sign of PG that is certainly involved in post-surgical and peristomal PG. [8][9][10] The similarities between PG and neutrophilic diseases suggest that underlying common inflammatory pathways probably converge to their pathophysiology, leading to abnormalities in polymorphonuclear neutrophils (PMN) recruitment or homeostasis. 8 In PG and other neutrophilic diseases, elevated skin and/or circulating levels of the pro-inflammatory cytokines (IL1β, IL6, TNF-α, IFN-ϒ, G-CSF) [10][11][12] and, particularly, of the potent PMN attracting chemokines, namely IL8/CXCL8 and CXCL1,2,3, along with skin infiltration by T cells, particularly at the edge of the PG ulcer, suggests an active recruitment of PMN to the skin.…”

Section: Pathophysiologymentioning

confidence: 99%

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Pyoderma gangrenosum: challenges and solutions

Gameiro¹,

Pereira²,

Cardoso³

et al. 2015

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Pyoderma gangrenosum (PG) is a rare disease, but commonly related to important morbidity. PG was first assumed to be infectious, but is now considered an inflammatory neutrophilic disease, often associated with autoimmunity, and with chronic inflammatory and neoplastic diseases. Currently, many aspects of the underlying pathophysiology are not well understood, and etiology still remains unknown. PG presents as painful, single or multiple lesions, with several clinical variants, in different locations, with a non specific histology, which makes the diagnosis challenging and often delayed. In the classic ulcerative variant, characterized by ulcers with inflammatory undermined borders, a broad differential diagnosis of malignancy, infection, and vasculitis needs to be considered, making PG a diagnosis of exclusion. Moreover, there are no definitively accepted diagnostic criteria. Treatment is also challenging since, due to its rarity, clinical trials are difficult to perform, and consequently, there is no "gold standard" therapy. Patients frequently require aggressive immunosuppression, often in multidrug regimens that are not standardized. We reviewed the clinical challenges of PG in order to find helpful clues to improve diagnostic accuracy and the treatment options, namely topical care, systemic drugs, and the new emerging therapies that may reduce morbidity.

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Section: Pathophysiologymentioning

confidence: 99%

Section: Pathophysiologymentioning

confidence: 99%

Pyoderma gangrenosum: challenges and solutions

Gameiro¹,

Pereira²,

Cardoso³

et al. 2015

OTT

View full text Add to dashboard Cite

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“…IL-1 levels released by monocytyes, an action induced by lipopolysaccharides, can also be high in BD patients [51]. Similarly, activated neutrophils are frequently observed in pathological specimens, and BD is generally classified among neutrophilic dermatoses [53]. Systemic vasculitis and occlusive perivasculitis and thrombosis are observed [53].…”

Section: Abnormally Increased Inflammatory Responsementioning

confidence: 99%

“…Similarly, activated neutrophils are frequently observed in pathological specimens, and BD is generally classified among neutrophilic dermatoses [53]. Systemic vasculitis and occlusive perivasculitis and thrombosis are observed [53]. The hallmark histopathologic pattern of neutrophilic vasculitides is denoted as leukocytoclastic vasculitis, which is characterized by angiocentric segmental inflammation, endothelial cell swelling, and fibrinoid necrosis of blood vessel walls (postcapillary venules).…”

Section: Abnormally Increased Inflammatory Responsementioning

confidence: 99%

Vascular Manifestations of Behçet’s Disease

Demïrtürk¹,

Tünel²,

Alemdaroğlu³

2017

Behcet's Disease

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Behçet's disease (BD), a very morphologically diverse systemic disease, may involve the vascular system. The venous system is the most frequently attacked vessel system.The arterial system, when involved, increases the severity and morbidity of Behçet's disease. Cardiac involvement, although rare, can be very subtle and in itself increases the mortality. Vasculitis is the hallmark pathology resulting in occlusion, aneurysms, or both.Vascular involvement may be very challenging in all phases of treatment beginning from diagnosis till recovery and remission.

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“…Non-dermatologic manifestations have been described, including ocular, pulmonary, splenic, cardiac, and joint involvement [1]. Pathergy, or exacerbation of a wound following trauma, is a key feature in PG, and is familiar to some clinicians more for its association with Behçet's disease [2][3][4].…”

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confidence: 99%

Pyoderma gangrenosum near a cystostomy catheter

2017

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A 78-year-old man with chronic lymphocytic leukemia and neurogenic bladder with an indwelling suprapubic cystostomy presented with a small, painful lump surrounding his catheter site. The throbbing, lancinating pain started after a routine outpatient catheter exchange at his urologist's office. He reported that the pustule spread in a centrifugal fashion, and degenerated into an ulcer. He was prescribed cephalexin for presumed cellulitis by his primary care doctor.In the ensuing 72 h, the lesion continued to expand and was accompanied with increased pain despite taking antibiotics. He reported subjective fevers and night sweats, and subsequently presented to an outside hospital, where he was hospitalized for 5 days to receive intravenous antibiotics for presumed failure of oral therapy. He received vancomycin and piperacillin-tazobactam empirically. His blood cultures were negative for bacteria and fungi, and because of a mild improvement in symptoms, he was discharged home with amoxicillin-clavulanic acid to complete a 10-day course. His wound did not improve. He re-presented 5 days after discharge with worsening, uncontrolled pain and a continuously sprawling ulcer.Vital signs upon presentation showed a temperature of 38.4°C, heart rate of 121 beats/min, blood pressure of 144/81 mmHg, and respiration rate of 18 breaths/min. Abdominal examination revealed normal bowel sounds throughout, no tenderness to palpation away from the ulcer site, non-tender splenomegaly, and no rebound tenderness or guarding. Skin examination on the lower abdomen demonstrated a 30 9 10 cm ulcer with gunmetal gray, undermined borders (Fig. 1). He had exquisite tenderness to light touch over the ulcer. Laboratory studies showed a complete blood count consistent with a known pancytopenia from chemotherapy received 4 months previously, noting that his white blood cell count of 3270/lL (ANC 2808/lL), hemoglobin 8.8 g/dL, and platelet count of 100,000/lL were relatively unchanged. A peripheral blood smear was consistent with chemotherapy-induced pancytopenia. Urinalysis, liver function, and coagulation studies were within normal range. Urine and serum antigen studies for Histoplasma capsulatum and Blastomyces dermatitidis were negative. Repeat blood cultures were negative for fungal elements and bacteria. Abdominal computed tomography scan showed superficial abdominal wall stranding, but no signs of abscess. A biopsy of the ulcer edge showed a diffuse dermal infiltrate composed of mature neutrophils. Gram stains and special stains for microorganisms (Periodic Acid Schiff, Gomori Methenamine Silver, and Acid-Fast Bacilli) were negative for any bacteria and subsequent tissue culture yielded no growth. The biopsy site also was exquisitely painful and started to spread. His painful lesion progressed despite treatment with vancomycin and meropenem for 5 days and his pain was inadequately controlled despite multiple analgesics.Given his failure to improve with broad spectrum antibiotics, the onset of his illness was revisited. He stressed that the...

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Neutrophilic Dermatoses: An Update

Cited by 64 publications

References 67 publications

Pyoderma gangrenosum: challenges and solutions

Pyoderma gangrenosum: challenges and solutions

Vascular Manifestations of Behçet’s Disease

Pyoderma gangrenosum near a cystostomy catheter

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