Nikkels A F, Nikkels-Tassoudji N, Piérard G E
Department of Dermatopathology, University of Liège, B-4000 Liège, Belgium.
Eur J Dermatol. 1999 Mar;9(2):126-8.
We report the case of a 74-year-old man who suffered for four years from recurrent generalized pustular eruptions associated with acrodermatitis continua of Hallopeau (ACH). Initial therapy using topical corticosteroids, tars and PUVA failed to improve the acral lesions. The disease was also unresponsive to systemic therapy using corticosteroids, methotrexate, etretinate, acitretine and cyclosporin A. Serious side-effects were experienced with these drugs. A marked improvement of the generalized pustules was observed under treatment combining etretinate and methotrexate, without having any effect on the acropustulosis. During hospitalization, a topical tar therapy cleared the pustular rash. Again no effect was observed on the acral lesions. Disulone (DDS) therapy, initiated at a dose of 200 mg/day, completely cleared ACH after 4 weeks. The disease relapsed when the dose was reduced to 100 mg/day. Increasing the DDS to 150 mg/day was rapidly followed by a new clearance of the acral lesions. After a 3-month treatment, the patient has remained lesion free and no DDS-related side-effects have occurred. In conclusion, sulfones may be considered as an alternative therapeutic option in drug-resistant ACH.
我们报告了一例74岁男性患者,其患有与Hallopeau连续性肢端皮炎(ACH)相关的复发性全身性脓疱疹达四年之久。最初使用外用皮质类固醇、焦油和光化学疗法(PUVA)治疗未能改善肢端病变。该疾病对使用皮质类固醇、甲氨蝶呤、依曲替酯、阿维A和环孢素A的全身治疗也无反应。使用这些药物出现了严重的副作用。在联合使用依曲替酯和甲氨蝶呤治疗期间,观察到全身性脓疱有明显改善,但对肢端脓疱病没有任何效果。住院期间,外用焦油疗法清除了脓疱性皮疹。同样,对肢端病变没有观察到效果。以每日200毫克的剂量开始使用氨苯砜(DDS)治疗,4周后ACH完全消退。当剂量减至每日100毫克时疾病复发。将DDS增加至每日150毫克后,肢端病变迅速再次消退。经过3个月的治疗,患者一直无皮损,且未出现与DDS相关的副作用。总之,对于耐药性ACH,砜类药物可被视为一种替代治疗选择。