Aktinik keratoz tanısıyla opera edilen buruna lokalize pemfigus vulgaris

Pemphigus vulgaris localized to the nose operated with the diagnosis of actinic keratosis

Özet

Pemfigus vulgaris deri ve mukozal yüzeylerde yayg›n bül ve erode lezyonlarla seyreden otoimmün büllöz bir hastal›kt›r. Günümüze kadar mukoza tutulumu olmadan belirli bir deri alan›na s›n›rl› pemfigus vakalar› rapor edilmekle beraber, burun derisine s›n›rl› çok az say›da pemfigus vakas› bildirilmifltir. Bunlar›n büyük ço¤unlu¤u da pemfigus foliaceus vakalar›ndan oluflmaktad›r. ‹ngilizce literatürde, lokalize olarak burunda bafllayan ve uzun süreli takiplerinde baflka bölgede lezyon ç›k›fl› olmayan yaln›zca dört pemfigus vulgaris vakas› bildirilmifltir. Bu vakalar›n hiçbiri ilk klinik tan› olarak pemfigus vulgaris tan›s› almam›fl olup, ayr›c› tan›da seboreik dermatit, diskoid lupus eritematosus, impetigo, darier hastal›¤› düflünülmüfltür. Bugüne kadar literatürde yanl›fll›kla aktinik keratoz olarak tedavi edilen yaln›z bir pemfigus vulgaris vakas› sunulmufl olup, bu vakada ilk lezyon kafa derisinde bafllam›fl; 15 ay sonra da oral mukoza lezyonlar› ç›km›flt›r. Burada aktinik keratoz tan›s›yla opere edilen ve iki y›l boyunca baflka bölgede lezyonu ç›kmayan, buruna lokalize bir pemfigus vulgaris olgusunu, çok nadir görüldü¤ü ve klinik branfllar aras›nda konsültasyonun önemini vurgulamak amac› ile sunuyoruz. (Turkderm 2007; 41: 136-8)

Abstract

Pemphigus vulgaris is an autoimmune blistering disease characterized by disseminated bullae and erosions of the mucosal surfaces and skin. Although some cases of localized pemphigus without mucosal involvement have been reported, only a few cases limited to nose were presented. Most of these cases have included the patients with pemphigus foliaceus. In the English literature, only 4 pemphigus vulgaris cases persisting lesions localized to the nose have been described previously. Seborrheic dermatitis, impetigo, discoid lupus erythematosus, Darier’s disease, and actinic keratosis were considered for differential diagnosis in all these cases but pemphigus vulgaris was not considered in the initial clinical diagnosis. To date, only one pemphigus vulgaris case mistakenly treated as actinic keratosis was reported. In this case, initial lesion had first occurred in the scalp and oral mucosal lesions had appeared fifteen months later. Here, we present a pemphigus vulgaris case localized to the nose operated with a previous pathological diagnosis of actinic keratosis that did not show mucosal involvement or dissemination during a follow- up period of 2 years. As it is very rare in the clinical practice, collaboration between clinical branches is very important for correct clinical diagnosis and choosing appropriate treatment modality in these cases. (Turkderm 2007; 41: 136-8)