Joseph Jonathan M, Kilgore Jacob, Culotta Nicholas
Department of Dermatology, Louisiana State University (LSU) Health, New Orleans, USA.
Cureus. 2025 Feb 24;17(2):e79567. doi: 10.7759/cureus.79567. eCollection 2025 Feb.
Cutaneous lupus erythematosus (CLE) encompasses a broad range of clinical and histopathologic variants that can overlap with other dermatologic entities, complicating accurate diagnosis. We report the case of a 42-year-old male patient who initially presented with a diffuse pruritic eruption presumed to be atopic dermatitis, for which dupilumab was initiated. Within the following weeks, the patient developed a fever of unknown origin and diarrhea, raising concern for an atypical drug-related reaction or an unmasked autoimmune process. Subsequent biopsies demonstrated evolving histopathologic features, including superficial and deep perivascular dermatitis suggestive of drug eruption. In addition, a dermal mucin deposition with mixed neutrophilic and lymphocytic infiltrates is suggestive of cutaneous lupus, such as tumid lupus or lupus-related neutrophilic urticarial dermatosis. Despite negative direct immunofluorescence and fluctuating autoantibodies, partial and sustained clinical improvement occurred with hydroxychloroquine therapy. The patient's variable serologic profile (including intermittent positivity for antiribonucleoprotein and anti-Smith), transient urticarial lesions, and evolving histopathology highlight the difficulties in definitively categorizing cutaneous lupus subtypes. While a direct causal link between dupilumab and lupus-like disease remains unproven, the temporal association raises the possibility that T helper type 1/T helper type 2 immune modulation may unmask subclinical autoimmune conditions. This case underscores the importance of repeated clinicopathologic correlation and multidisciplinary surveillance in patients presenting with atypical or treatment-refractory dermatitis. Ongoing dermatologic and rheumatologic evaluation is critical for early detection of systemic involvement, especially when autoimmune etiologies are suspected. Hydroxychloroquine remains a cornerstone of therapy for many CLE variants and can provide substantial improvement, even in complex or overlapping clinical scenarios.
皮肤红斑狼疮（CLE）涵盖了广泛的临床和组织病理学变体，这些变体可能与其他皮肤病实体重叠，使准确诊断变得复杂。我们报告了一例42岁男性患者，该患者最初表现为弥漫性瘙痒性皮疹，推测为特应性皮炎，并因此开始使用度普利尤单抗治疗。在接下来的几周内，患者出现不明原因发热和腹泻，引发了对非典型药物相关反应或潜在自身免疫过程的担忧。随后的活检显示组织病理学特征不断演变，包括提示药物疹的浅表和深部血管周围性皮炎。此外，伴有中性粒细胞和淋巴细胞混合浸润的真皮粘蛋白沉积提示皮肤狼疮，如肿胀性狼疮或狼疮相关的嗜中性荨麻疹性皮肤病。尽管直接免疫荧光检查结果为阴性且自身抗体波动，但羟氯喹治疗使临床症状部分且持续改善。患者多变的血清学特征（包括抗核糖核蛋白和抗史密斯抗体间歇性阳性）、短暂性荨麻疹皮损以及不断演变的组织病理学突出了明确分类皮肤狼疮亚型的困难。虽然度普利尤单抗与狼疮样疾病之间的直接因果关系尚未得到证实，但时间上的关联增加了1型辅助性T细胞/2型辅助性T细胞免疫调节可能掩盖亚临床自身免疫状况的可能性。该病例强调了在患有非典型或治疗难治性皮炎的患者中反复进行临床病理相关性分析和多学科监测的重要性。持续的皮肤科和风湿科评估对于早期发现系统性受累至关重要，尤其是在怀疑自身免疫病因时。羟氯喹仍然是许多CLE变体治疗的基石，即使在复杂或重叠的临床情况下也能带来显著改善。
