Hopkins Christina R, Ren Vicky, Grover Raminder, Cockerell Clay, Hsu Sylvia
Dermatology, Baylor College of Medicine, Houston, USA.
Laboratory Medicine, Beutner Laboratories, Buffalo, USA.
Cureus. 2022 Feb 14;14(2):e22201. doi: 10.7759/cureus.22201. eCollection 2022 Feb.
Bullous pemphigoid (BP) is the most common autoimmune bullous disease, but rarer forms of pemphigoid may appear identical to BP on routine histopathology and direct immunofluorescence (DIF). Here, we present the case of a 60-year-old man, who was initially thought to have BP, with supportive findings on routine histopathology and DIF. However, prominent oral involvement and cutaneous lesions refractory to conventional treatment suggested an alternate diagnosis. Further workup was performed, including indirect immunofluorescence (IIF) on salt-split skin, which showed binding of antibodies to the dermal floor rather than to the blister roof, and enzyme-linked immunosorbent assay for pemphigus and pemphigoid antibodies. With these additional tests, we concluded that the patient does not have BP but rather anti-p200 pemphigoid, anti-p105 pemphigoid, or a yet undiscovered form of pemphigoid. We reached a presumptive diagnosis of anti-p200 pemphigoid, as it is the most common pemphigoid with serum antibodies to the dermal floor of human salt-split skin by IIF. This case demonstrates that suspicion for other autoimmune bullous diseases in cases of treatment-refractory and clinically aberrant BP is essential. A limited workup may lead to a missed diagnosis and ultimately less efficient disease management.
大疱性类天疱疮（BP）是最常见的自身免疫性大疱性疾病，但在常规组织病理学和直接免疫荧光（DIF）检查中，罕见的类天疱疮形式可能与BP表现相同。在此，我们报告一例60岁男性病例，该患者最初被认为患有BP，常规组织病理学和DIF检查有支持性结果。然而，明显的口腔受累以及对传统治疗无效的皮肤病变提示可能存在其他诊断。于是进行了进一步检查，包括对盐裂皮肤进行间接免疫荧光（IIF）检查，结果显示抗体与真皮底层而非疱顶结合，以及针对天疱疮和类天疱疮抗体的酶联免疫吸附测定。通过这些额外检查，我们得出结论，该患者并非患有BP，而是患有抗p200类天疱疮、抗p105类天疱疮或一种尚未发现的类天疱疮形式。我们初步诊断为抗p200类天疱疮，因为它是通过IIF检测血清中存在针对人盐裂皮肤真皮底层抗体的最常见类天疱疮。该病例表明，对于治疗难治且临床异常的BP病例，怀疑存在其他自身免疫性大疱性疾病至关重要。有限的检查可能导致漏诊，最终降低疾病管理的效率。
