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for the BD classification recruited from the Department of Internal Medicine, Rabta Hospital in Tunis and compared with 125 native Tunisian healthy age and sex matching volunteers.According to our findings, the frequency of HLAB 51 was significantly higher in BD patients than in controls (p<0.001). Positive pathergy test (PPT) (p = 0.01) and retinal vasculitis (p = 0.045), were significantly more frequent in HLA B51(+) patients, while the frequency of arterial aneurysms (p = 0.009) and neurological
also had vesicles. In one patient, lesions resembled bullous impetigo. In all, the lesions involved the face, followed by the extremities in three and the trunk in two patients. Pathergy phenomena was present in one patient. Hepatomegaly and a leukemoid reaction were present in all patients. Bone marrow showed an M7 immunophenotype in three patients and normal cellularity in one. Follow-up ranged from 2 to 11 months, during which the patients were healthy.Recognition of the cutaneous eruptions
presentation is intestinal ulceration. There are distinct regional differences in disease expression of BS with fewer cases of intestinal disease in the Mediterranean basin and less severe eye disease and less frequent skin pathergy among patients reported from northern Europe or America. The clustering of symptoms, especially with the recently described increased frequency of the acne/arthritis cluster in familial cases, suggests that more than one pathological pathway is involved in what we call BS today (...) . Supportive evidence for this contention also comes from the observations that (a) the genetic component is very complex with perhaps different genetic modes of inheritance in the adult and in the pediatric patients; and (b) there are differing organ responses to one same drug. For example, the anti-TNF agents successfully control the oral ulcers while they have no effect on the pathergy reaction.
25 to 55. It can manifest in various subtypes. Pathophysiology Pathophysiology of pyoderma gangrenosum is poorly understood but may involve problems with neutrophil chemotaxis. IL-8 is overexpressed in lesions. Ulcerations of pyoderma gangrenosum occur after trauma or injury to the skin in about 30% of patients; this process is termed pathergy. General reference Pyoderma gangrenosum: An update on pathophysiology, diagnosis and treatment. Am J Clin Dermatol 1-18, 2017. doi: 10.1007/s40257-017-0251
Staged correction of an equinovarus deformity due to pyoderma gangrenosum using a Taylor spatial frame and tibiotalar calcaneal fusion with an intramedullary device Pyoderma gangrenosum is a rare autoinflammatory syndrome manifested by skin lesions eventually creating ulcers. Surgical management can lead to scarring and contracture at the site of the lesion due to the pathergy phenomenon. A 43-year-old woman presented with a 5-year history of severe equinovarus deformity due to chronic pyoderma
pathergy reaction with surgical cleaning of the skin before the procedure, the acne-arthritis association carrying similar features to acne-associated reactive arthritis, a higher rate of tonsillectomy, cold sores, late birth order, higher number of siblings, history of travel to countries with a high incidence of BS and earlier age at first sexual intercourse. Moreover, basic research on both viruses and bacteria suggests that micro-organisms may be playing a role, possibly through heat shock proteins
on clinical findings including oral and genital ulcers, skin lesions such as erythema nodosum, acne, or folliculitis, ocular inflammatory findings and a pathergy reaction. Inflammatory markers such ESR, and CRP may be elevated. A complete ophthalmic examination may include a slit lamp examination, to detect nerve loss, visual field examinations, fundoscopic examination to assess optic disc atrophy and retinal disease, fundoscopic angiography, and visual evoked potentials, which may demonstrate increased (...) or more after needle-prick). The pathergy test has a specificity of 95 percent to 100 percent, but the results are often negative in American and European patients (papulo-pustules, , , in post-adolescents not on corticosteroids) Despite the inclusive criteria set forth by the International Study Group, there are cases where not all the criteria can be met and therefore a diagnosis cannot readily be made. There is however a set of clinical findings that a physician can rely upon in making a tentative
Folgoration as an Example of Pathergy in a Patient Affected by Pyoderma Gangrenosum and Takayasu's Arteritis Pyoderma gangrenosum (PG) is a neutrophilic dermatosis of unknown aetiology. Clinical manifestations of PG are characterized by destructive, necrotizing, and noninfective ulceration of the skin. 20-30% of cases are initiated and aggravated by minor trauma or surgery, a phenomenon named pathergy. PG is related to several autoimmune diseases including ulcerative colitis, Crohn's disease (...) , rheumatoid arthritis, and monoclonal gammopathy. The association with Takayasu's arteritis (TA), a chronic inflammatory and stenotic disease of large and medium-sized arteries, is instead less common. We report a case of PG associated with TA that was induced by an accident with folgoration of the skin; in this case the folgoration can be considered as an exemple of Pathergy, that is, a characteristic feature of PG.
Eruptive postoperative squamous cell carcinomas exhibiting a pathergy-like reaction around surgical wound sites. The isomorphic response of Koebner and the pathergy skin reaction have not classically been associated with neoplastic processes such as cutaneous squamous cell carcinoma (SCC).We report 5 patients who developed postoperative eruptive SCCs within and around the healed or healing sites of excised nonmelanoma skin cancers. The original cancers were all excised with histologically tumor (...) was approximately 6 weeks and histologic examination of the nodules demonstrated well-differentiated SCC.This study is limited by its relatively small number of cases.Acutely eruptive SCC is a rare but significant postoperative complication of skin cancer surgery. Furthermore, this case series suggests that some individuals appear to be predisposed to this recurrent, pathergy-like reaction involving SCC.
, genital ulcers and uveitis. This was originally characterized by the Turkish physician Hulusi Behcet in 1937. While these symptoms are the most common of Behcet’s, other tissues may be affected as Behcet’s is a systemic vasculitic disorder. GI symptoms, neurological sequelae, the pathergy reaction, erythema nodosum, papulopustular acne, and arthritides are all manifestations of Behcet’s Syndrome. There are no pathognomonic laboratory tests or imaging for Behcet’s; the diagnosis is made clinically (...) thrombosis and pulmonary arterial aneurysm with hemoptysis can easily be mistaken as manifestations of pulmonary embolism; however, anticoagulation of pulmonary arterial aneurysm is potentially disastrous, creating a serious diagnostic dilemma. The final clinical expression of Behcet’s described by Dr. Yazici was the pathergy reaction whereby one makes multiple intradermal skin breaks with a 20 gauge needle. Importantly, this test should not break a blood vessel. A positive reaction consists
-a, IL-8, IL-12, IL-17 and IL-21) have been implicated in the pathogenesis of BD. Diagnostic methods International classification criteria, as defined by clinical presentation, are sensitive and specific. The presence of recurrent oral aphtae, at least 3 times over 12 months, is mandatory, in combination with two of the following: recurrent genital ulceration, eye lesions, skin lesions or pathergy. In neuro-BD, lumbar puncture is mandatory and MRI may reveal inflammatory lesions in the cerebral trunk
Pyoderma Gangrenosum and Full-Thickness Burns: is there a Problem of Differential Diagnosis? A case is presented of pyoderma gangrenosum, describing its characteristic features with particular reference to the phenomenon of pathergy, which considerably limits the surgical approach to the disease. After an account of the numerous skin ulcerations that have to be taken into consideration in order to make a differential diagnosis, it is noted that pyoderma gangrenosum can only on rare occasions
Pyoderma gangrenosum of the penis: a potentially dramatic skin disease. We report the case of a 17-year-old boy who presented with penile ulceration and a urethral fistula that had failed to heal after plastic reconstructions with skin grafts. The patient had a history of pathergy, because the initial lesion was an ulcer that deteriorated and led to the development of the fistula after surgical interventions for its repair. On the basis of the patient's history and normal laboratory evaluation
Short-term trial of etanercept in BehÃ§et's disease: a double blind, placebo controlled study. To determine the effect of the tumor necrosis factor-alpha blocker etanercept on the pathergy and monosodium urate (MSU) status and on the mucocutaneous and articular manifestations of patients with Behçet's disease (BD).Forty male patients with BD, all with positive pathergy and MSU tests and mucocutaneous disease and/or arthritis, were randomized (20 patients to each study arm) to receive either (...) etanercept 25 mg twice a week or placebo for 4 weeks. The pathergy and MSU responses and the frequencies of mucocutaneous and articular manifestations were compared between the 2 groups.There were no decreases in the pathergy and MSU responses in the etanercept group compared to the placebo group at any time. The mean numbers of oral ulcers, nodular lesions, and papulopustular lesions were less in the etanercept group compared to the placebo group at all weekly evaluations, except for the second week
Postoperative pyoderma gangrenosum: diagnostic value of 16s ribosomal RNA sequencing and review of the literature. Pyoderma gangrenosum (PG) is an uncommon condition, but can lead to the diagnosis of an underlying systemic disease. It can appear spontaneously or as a result of the pathergy phenomenon after trauma or surgery.We review three patients with postoperative PG (PPG) and the relevant literature. We also report an accurate method for microbial detection by 16S ribosomal (r)RNA
T helper type 1 immune reaction was reconfirmed in recent studies, especially during active phases, but T helper type 2 reaction may also play a role. Interleukin-12B heterozygosity is associated with Behçet's disease susceptibility and plays an important role in mediating T helper type 1 antistreptococcal immune response. Selenium binding protein may be a target antigen in Behçet's uveitis. Pathergy reaction is most frequently positive in the forearm; multiple needle pricks increase positive
Pyoderma gangrenosum: an update. Pyoderma gangrenosum (PG) can be differentiated into classic and atypical forms. The classic form is characterized by ulcers and the atypical form by deep erosions with bullous blue-gray margins. Pathergy, the development of cutaneous lesions at sites of trauma, is a common feature of both forms of PG. Approximately 50% of patients who have PG have underlying systemic diseases, most commonly inflammatory bowel disease, myeloproliferative disorders, and various
hundred and sixty-one patients were involved in this multicentre study. The symptoms of the disease were recorded retrospectively in the time order of the manifestations in each patient.Oral ulcers were the most common manifestation (100%), followed by genital ulcers (85.3%), papulopustular lesions (55.4%), erythema nodosum (44.2%), skin pathergy reaction (37.8%), and articular (33.4%) and ocular involvement (29.2%). Oral ulcers were the most common onset manifestation (88.7%). The mean +/- SD (...) duration between the onset symptom and the fulfilment of diagnostic criteria was calculated to be 4.3 +/- 5.7 years. The clinical severity score was significantly increased in the noncompliant treatment group compared with the compliant group with the passage of time (P < 0.001). The frequency of ocular involvement and genital ulcers was significantly higher in patients whose disease onset was at < 40 years. Genital ulcers, ocular involvement, papulopustular lesions, thrombophlebitis and skin pathergy
Pyoderma gangrenosum following isotretinoin therapy for acne nodulocystic. A 19-year-old man with nodulocystic acne on baseline was treated with isotretinoin therapy. After 1 month on the medication, he developed pyoderma gangrenosum on his pubis area, arms and legs, and pathergy on a puncture site. Possible underlying diseases were excluded. The patient was started on steroids (prednisone 1 mg/kg/d) and isotretinoin therapy was withdrawn. Later the prednisone was tapered and dapsone 100 mg