Meola T, Lim H W
Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York.
Dermatol Clin. 1993 Jul;11(3):583-96.
The vesiculobullous porphyrias are a group of blistering diseases with systemic as well as cutaneous manifestations ranging from mild to disabling, secondary to endogenous photosensitizing porphyrins. The characteristic patterns of porphyrin accumulation in erythrocytes, plasma, urine, and feces are invaluable for differentiating the vesiculobullous porphyrias from other blistering diseases and from one another. Most importantly, these porphyrin profiles are essential for distinguishing the acute vesiculobullous porphyrias, HCP and VP, from PCT, which is not associated with acute attacks. This distinction may prevent the potentially fatal administration of porphyrinogenic drugs to patients with HCP or VP. Histopathology is not helpful in differentiating the vesiculobullous porphyrias, as all typically show subepidermal bullae. Avoidance of sun exposure and precipitating factors are the initial steps in both the prevention and treatment of these entities. In addition, more specific therapy may be indicated, as discussed in the preceding pages.
水疱大疱性卟啉病是一组水疱性疾病，具有全身和皮肤表现，从轻度到致残不等，继发于内源性光敏卟啉。红细胞、血浆、尿液和粪便中卟啉积累的特征模式对于区分水疱大疱性卟啉病与其他水疱性疾病以及它们彼此之间的区别非常重要。最重要的是，这些卟啉谱对于区分急性水疱大疱性卟啉病、遗传性粪卟啉病（HCP）和变异性卟啉病（VP）与迟发性皮肤卟啉病（PCT）至关重要，后者与急性发作无关。这种区分可以防止对患有HCP或VP的患者给予潜在致命的卟啉生成药物。组织病理学无助于区分水疱大疱性卟啉病，因为所有病例通常都表现为表皮下水疱。避免阳光照射和诱发因素是预防和治疗这些疾病的初始步骤。此外，如前所述，可能需要更具体的治疗方法。
