BEHCET overig neurologie vasculitis trombose artritis renaal GE ulceratie longafwijkingen op X-thorax Van: Heer, Koen de Verzonden: woensdag 8 april 2015 15:16 Aan: Heer, Koen de Onderwerp: aften O/ striae over de armen voeding, hormonaal niet uitgesloten maar onwaarschijnlijk Conclusie: 1. Complexe aftose, ook vaginaal, 3-4x/jaar, IBD door MDL uitgesloten vanwege ook peri-anale fistel (coloscopie, CRP, kliniek), ANA-negatief, d.d. hypoferritinemie, coeliakie, Behcet, zeldzaam (MAGIC, etc.) Normaal B12, geen HIV. 2. IJzerdeficientie bij forse menstruaties, wil geen OAC, mogelijk uterus myomatosus. 3. raciale neutropenie B/ ijzer hervatten p.m. steroidcreme in Orabase coeliakie-serologie Behcet uitsluiten: i.c.c. oogarts (soms tranende ogen), pathergietest p.m. cyclische neutropenie uitsluiten painful oral lesions localized, shallow, round to oval ulcers with a grayish base Recurrent aphthous stomatitis (RAS) most common cause of mouth ulcers 10 to 14 days, heal without scarring >5 mm, that can last for six weeks is less common two to four outbreaks per year, while others may have almost continuous eruptions become less frequent in adulthood herpetiform ulcers: multiple small clusters of pinpoint lesions that coalesce sodium lauryl sulfate, a toothpaste detergent, may prolong healing time RF/ familial tendency, trauma, hormonal factors, food or drug hypersensitivity, immunodeficiency (eg, HIV disease), and emotional stress AO/ HIV, neutropenie, coeliakie, IBD, med (MTX), B12 Treatment — applied to the ulcer two to four times daily until the ulcer is healed symptomatic triamcinolone acetonide in Orabase fluocinonide gel covered by Orabase topical analgesics chemical cautery (silver nitrate, sulfuric acid with sulfonated phenolics) after numbing with topical lidocaine: mogelijk more rapid improvement in pain, does not speed overall healing. Rinse with water for several minutes after the procedure. Intralesional or oral glucocorticoids are indicated for recalcitrant lesions or severe disease. Colchicine, dapsone, pentoxifylline, interferon alpha, and levamisole also may have therapeutic value in severe cases of RAS [54-57]. Thalidomide? Complex aphthosis — recurrent large oral ulcers in conjunction with genital lesions in the absence of other criteria for Behcet's disease d.d. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage), FAPA (fever, aphthosis, pharyngitis, and adenitis) syndrome, and cyclic neutropenia, infectious cases (Yersinia enterocolitis, tuberculous enterocolitis, and typhoid fever) Behcet's disease — neutrophilic inflammatory disorder that presents with recurrent oral and genital ulcerations initially manifest oral aphthae that are grossly and histologically similar to common oral ulcers more extensive and often multiple healing typically spontaneous within one to three weeks 75% genital lesions D/ recurrent oral ulcers (three times per year) required plus 2/4 recurrent genital ulcers eye lesions skin lesions positive pathergy test [24 to 48 hours after oblique insertion of a 20 to 25 gauge needle]) Th/ colchicine, topical anesthetics, topical or intralesional corticosteroids, or systemic glucocorticoids painful oral lesions localized, shallow, round to oval ulcers with a grayish base Recurrent aphthous stomatitis (RAS) most common cause of mouth ulcers 10 to 14 days, heal without scarring >5 mm, that can last for six weeks is less common two to four outbreaks per year, while others may have almost continuous eruptions become less frequent in adulthood herpetiform ulcers: multiple small clusters of pinpoint lesions that coalesce sodium lauryl sulfate, a toothpaste detergent, may prolong healing time RF/ familial tendency, trauma, hormonal factors, food or drug hypersensitivity, immunodeficiency (eg, HIV disease), and emotional stress AO/ HIV, neutropenie, coeliakie, IBD, med (MTX), B12 Treatment — applied to the ulcer two to four times daily until the ulcer is healed symptomatic triamcinolone acetonide in Orabase fluocinonide gel covered by Orabase topical analgesics chemical cautery (silver nitrate, sulfuric acid with sulfonated phenolics) after numbing with topical lidocaine: mogelijk more rapid improvement in pain, does not speed overall healing. Rinse with water for several minutes after the procedure. Intralesional or oral glucocorticoids are indicated for recalcitrant lesions or severe disease. Colchicine, dapsone, pentoxifylline, interferon alpha, and levamisole also may have therapeutic value in severe cases of RAS [54-57]. Thalidomide? Complex aphthosis — recurrent large oral ulcers in conjunction with genital lesions in the absence of other criteria for Behcet's disease d.d. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage), FAPA (fever, aphthosis, pharyngitis, and adenitis) syndrome, and cyclic neutropenia, infectious cases (Yersinia enterocolitis, tuberculous enterocolitis, and typhoid fever) Behcet's disease — neutrophilic inflammatory disorder that presents with recurrent oral and genital ulcerations initially manifest oral aphthae that are grossly and histologically similar to common oral ulcers more extensive and often multiple healing typically spontaneous within one to three weeks 75% genital lesions D/ recurrent oral ulcers (three times per year) required plus 2/4 recurrent genital ulcers eye lesions skin lesions positive pathergy test [24 to 48 hours after oblique insertion of a 20 to 25 gauge needle]) Th/ colchicine, topical anesthetics, topical or intralesional corticosteroids, or systemic glucocorticoids Weer zeer veel aften gehad, ook vaginaal. Afgelopen jaar wel 3-4x. Geen familiaire belasting. Vorig jaar veel stress, nu niet per se. Geen trauma. Geen medicatie. Veel jeuk huid. Soms tranende ogen, egen andere klachten van ogen. Geen keelpijn. Geen diarree. Herkent foto's van pathergie niet. O/ striae over de armen Ook fistel peri-anaal waarvoor binnenkort coloscopie DD/ voeding, hormonaal niet uitgesloten HIV/B12-deficientie uitgesloten; nu wel weer laag ferritine, echter afgelopen jaar niet en nog steeds aften B/ coloscopie afwachten p.m. steroidcreme in Orabase Recurrent aphthous stomatitis (RAS) is the most common cause of mouth ulcers [43]. familial tendency, trauma, hormonal factors, food or drug hypersensitivity, immunodeficiency (eg, HIV disease), and emotional stress [46,47]. RAS may be seen in patients with celiac disease and inflammatory bowel disease. Other causes of aphthae include use of antimetabolites such as methotrexate and neutropenia of any cause. Vitamin and mineral deficiencies have also been implicated in the pathogenesis of recurrent oral aphthae, particularly deficiency of vitamin B12. However, in a randomized trial involving 120 patients with RAS, multivitamin supplementation did not reduce the number or duration of RAS episodes [48]. Sodium lauryl sulfate, a toothpaste detergent, may prolong the ulcer healing time [49]. Some people have only two to four outbreaks per year, while others may have almost continuous eruptions. Aphthae occur more commonly in childhood and adolescence and become less frequent in adulthood. Treatment — The most common treatment for aphthous ulcers includes symptomatic relief with agents such as triamcinolone acetonide in Orabase, fluocinonide gel covered by Orabase, and topical analgesics. These are applied to the ulcer two to four times daily until the ulcer is healed. Early initiation of treatment may result in more rapid healing [50]. Chemical cautery can also be used for aphthous ulcers [51]. Randomized trials with silver nitrate [52], or with sulfuric acid with sulfonated phenolics [53], suggest that cautery can lead to more rapid improvement in pain, although it does not appear to speed overall healing. Lesions can be numbed with topical lidocaine prior to chemical cautery, and patients should rinse with water for several minutes after the procedure. Intralesional or oral glucocorticoids are indicated for recalcitrant lesions or severe disease. Colchicine, dapsone, pentoxifylline, interferon alpha, and levamisole also may have therapeutic value in severe cases of RAS [54-57]. Thalidomide has been studied in patients with severe RAS [41,42,58-60]. In a randomized trial of HIV-infected patients with RAS, treatment with thalidomide, 200 mg/day for four weeks, resulted in healing in 16 of 29 patients in the thalidomide group (55 percent) compared with 2 of 28 patients receiving placebo (7 percent) [41]. A retrospective study of 92 non-HIV infected patients with RAS (including 16 with Behcet's disease) reported efficacy of lower doses; at a mean initial dose of 50 mg/day, thalidomide induced complete remission in 85 percent of subjects within 14 days [60]. Recurrence is common following cessation of therapy [58]; low maintenance doses of thalidomide may reduce recurrences [59,60]. Thalidomide is teratogenic (pregnancy class X). In the United States, thalidomide can only be prescribed through a special distribution program designed to minimize the chance of fetal exposure to the drug (S.T.E.P.S. program; access at www.thalomid.com/steps_program.aspx). Behcet's disease — Behcet's disease is a neutrophilic inflammatory disorder that presents with recurrent oral and genital ulcerations. ●Most, but not all, patients initially manifest oral aphthae that are grossly and histologically similar to common oral ulcers; however, they tend to be more extensive and often multiple (picture 14). Healing of the oral ulcerations is typically spontaneous within one to three weeks; although with recurrent lesions, many patients will have ulcers present almost constantly. ●Genital lesions occur in about 75 percent of patients with Behcet's disease. They are similar in appearance to the oral aphthae. Recurrent oral ulcers (three times per year) are required for the diagnosis of Behcet's disease, in addition to two other clinical findings (recurrent genital ulcers, eye lesions, skin lesions, or a positive pathergy test [24 to 48 hours after oblique insertion of a 20 to 25 gauge needle]) (table 1) [61]. (See "Clinical manifestations and diagnosis of Behçet’s disease".) The mucocutaneous manifestations of Behcet's disease can be treated with oral colchicine, topical anesthetics, topical or intralesional corticosteroids, or systemic glucocorticoids [62]. Thalidomide and dapsone have also been shown to be effective. (See "Treatment of Behçet’s disease".) Complex aphthosis — Complex aphthosis is the term used to describe the occurrence of recurrent large oral ulcers in conjunction with genital lesions in the absence of other criteria for Behcet's disease. Other syndromes associated with both oral and genital lesions are the MAGIC syndrome (mouth and genital ulcers with inflamed cartilage), FAPA (fever, aphthosis, pharyngitis, and adenitis) syndrome, and cyclic neutropenia (see "Cyclic neutropenia"). Infectious cases of oral and vulvar aphthae include Yersinia enterocolitis, tuberculous enterocolitis, and typhoid fever. AUTOIMMUNE DISEASES Systemic lupus erythematosus — Mucus membrane involvement