Neurological Manifestations of Medical Disorders
John W. Scadding, Nicholas A. Losseff in Clinical Neurology, 2011
The CSF shows a lymphocytic pleocytosis, with a raised opening pressure in 20 per cent of patients. The protein level may be elevated and oligoclonal bands may be present in a minority. However, in some 30 per cent, the CSF may be normal. The opening pressure is increased if there is venous sinus thrombosis. The pathergy test (the formation of a sterile pustule at the site of a needle puncture) has been suggested as a diagnostic observation, but has variable sensitivity. MRI in the acute stage may show lesions that appear iso- or hypointense on T1 images and hyperintense on T2 and FLAIR, due to venous thrombosis with reversible oedema. Lesions, which can be single or multiple, are seen most commonly at the mesodiencephalic junction, in the cerebellar peduncle, basal ganglia and brainstem, but can also occur in the optic nerves and hemispheres. In chronic disease, brainstem atrophy with gliosis can develop.
Dermatologic Disorders Causing Vulvar Disease
William J. Ledger, Steven S. Witkin in Vulvovaginal Infections, 2017
Another uncommon problem is Behçet’s disease. Its infrequency makes it difficult to diagnose. It is an endemic disease in the native population of the Mediterranean areas, the Middle East, and the Far East and will be encountered in these patient populations. It is a multisystemic inflammatory disease classified among the vasculitides and not limited to the genital tract.34 Oral ulcers are present in 98% of cases. They heal within 10 days, usually without scarring. Vulvar lesions can scar. Posterior uveitis does occur as well as a wide spectrum of vasculitic lesions. There are no confirmatory tests to establish the diagnosis. Instead, it has to be based upon clinical findings. The established criteria are noted in Table 14.1. This diagnosis should be considered as a possibility in a patient whose family roots are in the geographical areas mentioned earlier. In the genital area, recurrent ulcers are noted, often with subsequent scarring. These patients will have no laboratory evidence of genital herpes, and vulvar biopsy will show no evidence of another vulvar disease. The pathergy test, cited in Table 14.1, assesses cutaneous hypersensitivity. A positive test consists of a sterile pustule that develops 24–48 hours later at the site of a needle prick to the skin.35 In addition to the general physical examination to check for a variety of skin lesions noted in Table 14.1, an ophthalmologist should be consulted to check for such eye pathology as anterior or posterior uveitis, cells in the vitreous, or retinal vasculitis.
Posterior uveitis
Gwyn Samuel Williams, Mark Westcott, Carlos Pavesio, Bushra Thajudeen in Practical Uveitis, 2017
While Behçet's disease is a clinical diagnosis there are some tests that can be done to provide further evidence, though in reality they are less useful than textbooks suggest. The oft-cited HLA-B51 test in actual fact is not useful at all and there is no rationale for ordering this except perhaps in some sort of research setting. A patient with active Behçet's disease will often have raised inflammatory markers (ESR and CRP) and also a neutrophilia, but these tests are not diagnostic. You can ask the patient if they report a pustule at the site of phlebotomy. However, the classic pathergy test so beloved by textbooks where a sterile needle is used to jab the skin with pustule formation a day afterwards indicating positivity, is unhelpful and should not be done. An MRI of the brain, as mentioned above, should be performed if there are neurological features. If there is evidence of vascular thrombosis then referral to the physicians who can then investigate for other differential diagnoses is best advised. In fact due to the many different specialties which may need to give input to a Behçet patient special combined clinics are set up in some areas to help co-ordinate what would otherwise be a multitude of clinic appointments and a whole rain forest of letters between various doctors. The first step is to ascertain whether your region has such a clinic and if not whether referral to such a clinic in a nearby region would be in the patient's interest.
Evaluation of different classification criteria in children with Behcet disease: results from a single referral center
Published in Expert Review of Clinical Immunology, 2020
Rabia Miray Kisla Ekinci, Ebru Esen, Ahmet Hakan Erol, Selcuk Sızmaz, Dilek Karagoz, Derya Ufuk Altintas, Sibel Balcı
Oral aphthous ulcers were the first symptom in 11 (68.8%) patients, three (18.7%) patients presented uveitis, and the remaining two (12.5%) patients presented skin symptoms as the first symptom of BD. Pathergy was positive in only five (31.2%) patients. Overall, the most frequent symptoms were oral aphthous (93.8%), joint involvement (75%), genital ulcers (50%), erythema nodosum (50%), and uveitis (37.5%) among pediatric BD patients. Of six patients with BD related uveitis, three had panuveitis, two patients had anterior chamber involvement and only one patient had isolated posterior retina involvement. One patient suffered from deep venous thrombosis as vascular involvement and another one had pseudotumor cerebri as a neurological manifestation. Table 2 summarizes the clinical manifestations of pediatric BD patients included in the study.
Atypical Pyoderma gangrenosum: a case of delayed recognition
Published in Journal of Community Hospital Internal Medicine Perspectives, 2021
Anum Qureshi, Kia Persaud, Sajida Zulfiqar, Ranadheer Dande, Chahat Puri, Shankar Awasthi
Pyoderma gangrenosum (PG) is a rare inflammatory skin condition with an annual incidence of 3–10 cases in one million people. The pathogenesis is unclear but involves aberrant neutrophil activity [1] as well as a genetic component [2]. Underlying systemic disease is present in 50–70% of cases [3], and includes hematological disorders, inflammatory bowel disease (IBD) and rheumatoid arthritis. It can manifest initially as a papule, pustule, or vesicle that progresses to a painful ulcer [4]. PG is often misdiagnosed as a skin and soft tissue infection and inappropriately treated with antibiotics with no benefit. This may prompt intervention with surgical debridement, which frequently leads to a vicious cycle of worsening necrosis given the strong association of PG with severe pathergy phenomenon. Significant morbidity can be prevented by early diagnosis and treatment [5]. PG of the breast is rare, with only 43 reported cases, 70% of which developed after breast surgery [6]. The most frequently involved areas are the lower extremities including the pretibial area. The trunk, head and neck, and peristomal skin are less frequently affected.
Long-Term Efficacy of Pegylated Interferon Alpha-2b in Behçet’s Uveitis: A Small Case Series
Published in Ocular Immunology and Inflammation, 2019
Hande Celiker, Haluk Kazokoglu, Haner Direskeneli
A 43-year-old male patient with BD presented with complaints of sudden onset of diminution of vision in the right eye (RE) for two days, associated with pain. His VA was 0,3 in RE, 0,0 in the left eye (LE) at presentation. Intraocular pressure (IOP) was 17 mmHg RE and 16 mmHg LE. The slit-lamp examination revealed marked (3+) cells in the anterior chamber (AC) of RE and was normal for LE. Fundus examination revealed no remarkable findings. According to a systemic evaluation, oral aphtae, arthritis, and pseudofolliculitis were positive, and genital ulcerations and erythema nodosum were negative. Laboratory workup revealed that HLA B51 was negative. Pathergy test was positive. The patient was given azathioprine 2–3 mg/kg per day, prednisolone 16 mg per day, colchicine 2x2 per day, topical cyclopentolate hydrochloride 1% twice per day, and topical prednisolone acetate 1% per hour every day for RE. During the follow-up period, he had several severe panuveitic attacks, and, in March 2014, peg-IFN-α-2b was started as 30 µg weekly dosage under the guide of the flowchart (Figure 1). During the peg-IFN-α-2b treatment, uveitic findings rapidly regressed. At the first month examination, liver transaminases were detected at higher levels (AST: 46 IU (range 10–37) ALT: 134 IU (range 10–40)); therefore, peg-IFN-α-2b dose was decreased to 20 µg weekly. One month later, liver transaminases assessment revealed normal levels (AST: 24 IU, ALT: 48 IU). Peg-IFN-α-2b was used for 24 months then switched to standard IFN-α-2a (3 MIU/three times a week). He was at quiescent phase during the peg-IFN-α-2b therapy. Because the number of injections was high, the patient was not satisfied with classical IFN-α treatment. We plan to switch to peg-IFN-α-2b again.
Related Knowledge Centers
- Behçet'S Disease
- Pyoderma Gangrenosum
- Koebner Phenomenon
- Autoimmune Disease
- Psoriasis
- Lupus
- Hla-B51
- Skin Condition