Subacute Cutaneous Lupus Erythematosus-Like Eruption Induced by Durvalumab: A Case Report and Literature Review.

Reports of immune-related adverse events caused by programmed cell death-ligand 1 (PD-L1) inhibitor have been emerging. Herein, we report a subacute cutaneous lupus erythematosus (SCLE)-like eruption presented after the treatment of durvalumab in a patient with extensive-stage small cell lung carcinoma. A 74-year-old Thai man was referred to our department after experiencing multiple dusky red to brownish papules and patches with scale and erosions on photo-distributed areas after receiving 3 infusion cycles of durvalumab. Histological finding revealed epidermal atrophy with interface changes and superficial perivascular infiltration of lymphocytes. Serum antinuclear antibodies (ANA) was 1:320 and anti-Ro/Sjogren's-syndrome-related antigen A (anti-Ro/SSA) antibodies were positive (2+). Based on the history and clinicopathological correlation, the diagnosis of SCLE-like eruption due to durvalumab was made. To the best of our knowledge, this is the first case of durvalumab-induced SCLE.

Comparative Analyses of Clinical Features, Histopathology, and CD123 Immunohistochemistry of Oral Lupus Erythematosus, Lichen Planus, and Other Lichenoid Lesions.

BACKGROUND: Oral lupus erythematosus (OLE) and oral lichen planus (OLP) are among the common causes of oral lichenoid lesions (OLLs). The differential diagnosis among causes of OLLs, particularly between OLE and OLP, is challenging as they have significant clinical and histopathological overlap. OBJECTIVES: To compare and summarize the clinical, histopathological, and direct immunofluorescence (DIF) findings between OLE, OLP, and other OLLs and to explore the diagnostic value of CD123 immunohistochemistry. METHODS: A retrospective study on patients with OLE, OLP, and other OLLs was performed between January 2014 and December 2019. The baseline characteristics, the clinical, histopathological, and DIF features, as well as CD123 immunohistochemistry for plasmacytoid dendritic cells (PDCs) were statistically analyzed and compared between groups. RESULTS: Of 70 patients, 12 had OLE, 39 had OLP, and 19 had other OLLs. Oral erosions/ulcers were the most common findings in all three groups. Red macules, telangiectases, and discoid plaques were more common in OLE patients, while OLP cases were typified by reticulated patches (p < 0.05). Additionally, white patches were found more often in other OLLs than in both OLE and OLP (p = 0.002). Histologically, mucosal atrophy, basal vacuolization, and perivascular infiltrate were observed in OLE, whereas OLP specimens possessed mucosal hyperplasia, hypergranulosis, and compact orthokeratosis (p < 0.05). Mucosal spongiosis was a histologic feature that favored other OLLs over OLE and OLP (p < 0.001). Data on DIF were nonspecific for all three conditions. For immunohistochemical staining, the median number of total CD123+ PDCs was observed to be higher in OLE than OLP in the mucosal-submucosal junction (MSJ) (p = 0.021), the superficial perivascular area (p = 0.026), and the superficial and deep perivascular areas (p = 0.001). Likewise, PDCs in clusters ≥2+ were seen in significantly higher numbers on OLE than OLP along the MSJ (p = 0.002), the superficial perivascular area (p < 0.001), as well as the superficial and deep perivascular areas (p = 0.011). CD123+ PDCs were found to be significantly more numerous in both OLE and OLP than other OLLs in all of the abovementioned areas (all p < 0.05). CONCLUSION: While there are some differences in the clinicopathological features between OLE, OLP, as well as other OLLs, a significant overlap remains. The quantity and distribution pattern of CD123 immunohistochemical staining has a diagnostic implication in differentiating OLE from OLP and other OLLs.

Rituximab Therapy for Treatment of Pemphigus in Southeast Asians.

BACKGROUND: Rituximab provides more effective and less adverse effects than the standard dose of corticosteroids, but evidence on its efficacy and safety in the Thai population is lacking. OBJECTIVE: To evaluate the efficacy and safety of rituximab in the treatment of pemphigus and also to determine prognostic factors linked to the treatment outcomes. METHODS: Pemphigus patients who received rituximab from November 2017 to December 2020 were retrospectively reviewed. The outcome was evaluated by using early (end of consolidation phase [ECP]) and late endpoints (complete remission [CR] on/off therapy, immunological remission [IR], and relapse). Adverse events were noted. Prognostic factors associated with remission and relapse were analyzed. RESULTS: Of 53 pemphigus patients, all attained ECP within 1.61 months. Almost 80% achieved CR on therapy within a median time of 6.36 months, while 33.9% reached CR off therapy in 19.74 months. Nearly half had IR within a median time of 6.88 months. Relapse occurred in 33.3% with a median time of 14 months. In multivariate analysis, receiving rituximab within 12 months of disease duration was more likely to achieve CR off therapy and IR (hazard ratio [HR] 3.79; 95% confidence interval [CI] 1.38, 10.42; P = 0.01 and HR 2.74; 95% CI 1.12, 6.69; P = 0.027, respectively), whereas older patients and positive anti-desmoglein 1 levels at the time of CR were predictive indicators for relapse (HR 1.07; 95% CI 1.01, 1.13; P = 0.036 and HR 4.38; 95% CI 1.24, 15.46; P = 0.022, respectively). The treatment-related adverse effects occurred in 33.9%. CONCLUSION: Rituximab is effective and safe in Thai pemphigus patients. Early administration of rituximab was a predictor of clinical and immunological remission. Older age and persistently positive anti-Dsg1 were correlated with disease relapse.

Dermatologic Manifestations, Histologic Features and Disease Progression among Cutaneous Lupus Erythematosus Subtypes: A Prospective Observational Study in Asians.

INTRODUCTION: Cutaneous manifestations are central to the primary diagnosis of systemic lupus erythematosus (SLE). However, information on the clinical, histopathologic, and direct immunofluorescence (DIF) features among subtypes of cutaneous lupus erythematosus (CLE), as well as longitudinal prospective observational study to evaluate the natural history and the progression to SLE, is lacking among Asians. Our objectives are to summarize the differences in the clinical, histopathologic, and DIF characteristics and serological profiles between various subtypes of CLE, and to provide its natural history and the association with disease activity in our Asian population. METHODS: A prospective observational study on CLE patients was performed between May 2016 and May 2020. Patients underwent full physical/dermatologic examination, skin biopsy for histology, and DIF. Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores and laboratory data were evaluated. Time schedule and characteristics for resolution and/or the disease progression to SLE were recorded in subsequent follow-ups. RESULTS: Of 101 biopsy-proven CLE patients, 25 had acute CLE (ACLE), 8 had subacute CLE (SCLE), 39 had chronic CLE (CCLE) only, 22 had CCLE with SLE, and 7 had LE-nonspecific cutaneous lesions only. Patients with exclusive CLE showed lower female preponderance, serological abnormalities, and correlation to systemic disease. However, when CLE was accompanied with any LE-nonspecific cutaneous manifestations, they were associated with high antinuclear antibody (ANA) titer, renal, hematologic, joint involvement, and greater SLEDAI score. Of 207 biopsy sections, SCLE/CCLE regardless of systemic involvement showed significantly higher percentage of superficial/deep perivascular and perieccrine infiltration than ACLE. On DIF, deposition of multiple immunoreactants was associated with higher systemic disease. Approximately 10% of CLE-only patients later developed SLE but had mild systemic involvement. CONCLUSION: Our findings support that each CLE subtype has a diverse and unique character. Comprehensive understanding of the differences among CLE subtypes is important for achieving the correct diagnosis and providing appropriate disease monitoring and management.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis-Like Lupus Erythematosus.

BACKGROUND/OBJECTIVE: Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN)-like lupus erythematosus is a hyperacute and life-threatening form of cutaneous lupus erythematosus. Because of its rarity, little is known about this entity. We aimed to evaluate the clinical characteristics, laboratory findings, systemic manifestations, treatments, and outcome of SJS/ TEN-like lupus erythematosus. METHODS: We conducted a chart review study from July 2002 to September 2016 of all patients diagnosed with SJS/TEN-like lupus erythematosus who presented with gradual epidermal necrolysis without clear drug or infectious culprit. We evaluate for clinical features, extracutaneous involvement, Systemic Lupus Erythematosus Disease Activity Index, histologic findings, immunofluorescence pattern, serologic abnormalities, treatment, outcome, and recurrence of SJS/TEN-like lupus erythematosus. RESULTS: Of 9074 patients diagnosed with cutaneous lupus erythematosus and/or systemic lupus erythematosus, 6 patients justified the diagnosis of SJS/TEN-like lupus erythematosus (5 SJS/TEN-like acute cutaneous lupus erythematosus, 1 TEN-like subacute cutaneous lupus erythematosus), accounting for 0.07%. Fifty percent had epidermal necrolysis as the initial presentation of lupus with a median time from onset of 1.5 months (0-48 months). The median duration between initial rash and epidermal detachment was 4.5 days (3-14 days). All had internal organ involvement (hematologic and renal) and high Systemic Lupus Erythematosus Disease Activity Index score (median, 19.5 [16-24]). Most recovered with systemic corticosteroids, antimalarial drugs, and/or immunosuppressants. None had disease recurrence. CONCLUSIONS: This is the largest single series of patients with SJS/TEN-like lupus erythematosus. Skin damage is an indicator of disease activity, and careful search for extracutaneous involvement to prevent further complications is mandatory.