The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present some clinical, histological and immunopathological characteristics that are different from the classical types. These are reviewed in this article. Future research may help definitively to locate the position of these forms in the pemphigus group, especially with regard to pemphigus herpetiformis and the IgG/ IgA pemphigus.

Desde 1991, há 8 casos relatados de síndrome da fragilidade capilar pulmonar associada à psoríase pustulosa ou eritrodérmica, induzida ou não pelo uso de acitretina ou sirolimus - um dos casos, fatal. Reportamos um caso de psoríase pustulosa de von Zumbusch em paciente feminina com antecedente de várias internações por quadro semelhante, evoluindo nesta ocasião com acometimento pulmonar e resolução com corticosteroide. A paciente não estava em uso de quaisquer medicações previamente descritas como capazes de precipitar o quadro pulmonar (metotrexate e acitretina). Esta é uma complicação rara associada à psoríase, não descrita em pacientes brasileiros até o momento

Since 1991, eight cases of pulmonary leak capilary syndrome have been described associated with pustular or erythrodermic psoriasis induced or not by the use of acitretin or sirolimus - being one of the cases, fatal. We report the case of a female patient with diagnosed GPP or von Zumbusch and multiple hospitalizations due to such condition. At that time, the condition was evolving with pulmonary onset and resolution with corticosteroids. The patient was not using any previously described medication that could precipitate pulmonary condition (methotrexate and acitretin). This is a rare complication associated with psoriasis which has not been described in Brazilian patients so far