Review Article

Mucocutaneous Involvement in Behçet’s Disease: How Systemic Treatment Has Changed in the Last Decades and Future Perspectives

Table 2

Overview of works derived from the medical literature reporting treatment indications of mucocutaneous lesions in Behçet’s disease.

DrugsDoseAuthors (year)Number of patientsStudyEffectiveness

Azathioprine2,5 mg/kg/day Yazici et al. (1990) [34]45Randomized controlled trialOral and genital ulcers and eye involvement

Colchicine Aktulga et al. (1980) [35] 35Double-blind trialErythema nodosum-like lesions
1-2 mg/day Yurdakul et al. (2001) [36]116Randomized controlled trialGenital ulcers
1 mg/dayDavatchi et al. (2009) [37]169Randomized controlled trialGenital and oral ulcers and pseudofolliculitis

Colchicine versus benzathine penicillin + colchicine Çalgüneri et al. (1996) [38]60/94Open studyOral ulcers and erythema nodosum and genital ulcers

Benzathine penicillin versus colchicine versus benzathine penicillin + colchicineBenzathine penicillin 1.2 million units by injection monthly/colchicine 1 mg day/benzathine penicillin + colchicineAl-Waiz et al. (2005) [39]20/21/25Open studyOral ulcers and erythema nodosum

AzithromycinMumcu et al. (2005) [42]8Case seriesFolliculitis and oral ulcers
1500 mg/week for 4 weeksMumcu et al. (2013) [43]10Case seriesFolliculitis and oral ulcers

MinocyclineKaneko et al. (1997) [44]

Thalidomide100–300 mg/dayHamuryudan et al. (1998) [45]98Randomized controlled trialOral and genital ulcers and folliculitis lesions
50 mg/day De Wazières et al. (1999) [46]17Open studyOral and genital ulcers
400 mg/day for 5 days and then 200 mg/day for 4 weeksGardner-Medwin et al. (1994) [47]23Open studyOral and genital ulcers
400 mg/day for 5 days and then 200 mg/day Saylan and Saltik  (1982) [48]22Open studyOral and genital ulcers
Hamza (1986) [49]Open study

Cyclosporine10 mg/kg/day versus colchicine (1 mg/day)Masuda et al. (1989) [50]96Randomized controlled trialOral aphthous, dermal lesion, and genital ulcers
5 mg/kg/day Assaad-Khalil (1991) [51]Controlled studyOrogenital ulcers and skin lesion
Avci et al. (1997) [52]

Dapsone100 mg/daySharquie et al. (2002) [55]Double-blind placebo-controlled trialOrogenital ulcers
Sharquie (1984) [54]Open study

Interferon alpha 2a6 million units subcutaneously 3 times per weekAlpsoy et al. (2002) [57]50Randomized placebo-controlled and double-blind studyOral ulcers, genital ulcers, and papulopustular lesion
9 million units/day three times a week versus colchicine 1,5 mg/dayBoyvat et al. (2000) [58]20/16Randomized controlled trial
3 million units subcutaneously daily for 6 monthsO’Duffy et al. (1998) [59]10Randomized controlled trial
6 million units per day 3 times per week for 2 monthsGeorgiou et al. (1998) [60]12Oral aphthae, genital ulcers, erythema nodosum, and pseudofolliculitis
3 million units/day in the first week (three times a week), 6 million units/day in the second week (three times a week), 9 million units IU/day in the third week and thereafter (three times a week)Azizlerli et al. (1996) [61]18Oral aphthae, genital ulcers, erythema nodosum, and pseudofolliculitis
3 million units three times per week versus 9 million units once a monthAlpsoy et al. (1994) [62]14Randomized controlled trial
9 million units three times a week for 6 months Zouboulis et al.  (1993) [64]10Oral aphthae, genital ulcers, erythema nodosum, pseudofolliculitis
5 million units 3 times a week for 6 weeks followed by 5 million units once a week for 10 weeksHamuryudan et al. (1994) [63]21Randomized controlled trialOral aphthae, genital ulcers, erythema nodosum, and pseudofolliculitis

Alemtuzumab Perez-Pampin et al. (2013) [67]1Case reportOrogenital aphthosis and erythema nodosum
134 mg Lockwood et al. (2003) [68]18Open studyOrogenital ulcerations

Infliximab5 mg/kg every 8 weeks Olivieri et al. (2009) [74]Case reportMucocutaneous lesions
5 mg/kg for 4th infusion 0, 2, 6, and 15 weeks Haugeberg et al. (2004) [71]Case reportGenital ulcers
5 mg/kg every 8 + sulfasalazine Ordahan et al. (2014) [72]1Case reportOral and genital aphthosis
5 mg/kg every 8 + cyclosporineOlivieri et al. (2008) [73]1Case reportPapulopustulosa, erythema nodosum

Etanercept50/mg/weekly for 12 months [azatioprine + colchicine]Mohammed (2014) [75]15Open studyMucocutaneous lesions
25 mg twice a weekMelikoglu et al. (2005) [76]40Randomized controlled trialOral ulcers, papulopustular lesions, and modular lesions
25 mg twice a weekAtzeni et al. (2005) [77]1Case reportGenital ulcers

Apremilast30 mg/twice a dayHatemi et al. (2013) [33]55/56Placebo-controlled double-blind trialOral and genital ulcers

Rebamipide300 mg/dayMatsuda et al. (2003) [56]14/17Placebo-controlled double-blind trialOral ulcers

Anakinra100 mg/dayBotsios et al. (2008) [19]1Case reportOral and genital ulcers
1 mg/kg/day + colchicineBilginer et al. (2010) [79]1Case reportOral ulcers and erythema nodosum

Canakinumab150 mg every 8 weeksCantarini et al. (2012) [80]1Case report Recurrent oral and genital aphthosis, erythema nodosum, and pseudofolliculitis
150 mg every 6 weeksVitale et al. (2014) [21]3Case seriesMucosal ulcers