Accurate diagnosis of bullous pemphigoid requires multiple health care visits.

Introduction: Accurate use of diagnostic codes is crucial for epidemiological and genetic research based on electronic health record (EHR) data. Methods: This retrospective study validated the International Classification of Diseases (ICD)-10 diagnostic code L12.0 for bullous pemphigoid (BP) using EHR data from two Finnish university hospitals. We found 1225 subjects with at least one EHR entry of L12.0 between 2009 and 2019. BP diagnosis was based on clinical findings characteristic of BP and positive findings on direct immunofluorescence (DIF), BP180-NC16A enzyme-linked immunosorbent assay (ELISA) or indirect immunofluorescence (IIF) assay. Results: True BP was found in 901 patients; the positive predictive value (PPV) for L12.0 was 73.6% (95% CI 71.0-76.0). L12.0 was more accurately registered in dermatology units than any specialized health care units (p<0.001). Including patients with multiple L12.0 registrations (≥3), increased the accuracy of the L12.0 code in both dermatology units and other settings. Discussion: One diagnostic code of L12.0 is not enough to recognize BP in a large epidemiological data set; including only L12.0 registered in dermatology units and excluding cases with <3 L12.0 record entries markedly increases the PPV of BP diagnosis.

Clinical manifestations and comorbidities of pemphigus: a retrospective case-control study in southern Finland

Background: Pemphigus is associated with several autoimmune, dermatological, and psychiatric diseases. Previous studies have reported an increasing incidence of pemphigus in Finland, particularly pemphigus foliaceus and erythematosus. Objectives: The aim of this study was to determine the clinical presentation and associated comorbidities in pemphigus patients. Materials & Methods: We retrospectively assessed 66 pemphigus patients in Helsinki University Hospital and, with an age-standardised control group, performed a comparison of the studied comorbidities. Results: The patients displayed a 0.8 female:male distribution and a mean age of 57.4 years. Pemphigus vulgaris (41%), foliaceus (30%), and erythematosus (15%) were the most common subtypes. Hypertension (30%) and dyslipidaemia (21%) were the most prevalent comorbidities. We found a statistically significant association between pemphigus and a past history of, or concurrent malignancies and atopic dermatitis (p = 0.002 and p = 0.028, respectively). No significant difference was observed in the prevalence of cardiovascular disease, asthma, chronic obstructive lung disease, type I or II diabetes mellitus, inflammatory bowel disease, depression, or anxiety. Erosions (65%), bullae (59%), and crusted lesions (55%) were observed in most patients. Half of the patients experienced pruritus before or at diagnosis. Pruritus was associated with pemphigus without mucosal involvement (p = 0.01). Conclusion: We found a significant association between pemphigus and atopic dermatitis and a history of malignancy. The clinical picture frequently included pruritus. These results support the findings of some recent studies of pruritus occurring more frequently in patients with pemphigus foliaceus and cutaneous pemphigus vulgaris.

Depression and Anxiety in Patients with Bullous Pemphigoid: Impact and Management Challenges.

Bullous pemphigoid is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. It is also associated with high mortality and poor prognosis due to advanced age of the patients and coexisting comorbidities. There is a dearth of data in the literature regarding depression and anxiety among those patients. The objective of this brief review is to discuss the intertwining relationship between depression and anxiety with bullous pemphigoid.

Comorbidities of bullous pemphigoid in a Finnish cohort.

The incidence of bullous pemphigoid (BP) is increasing in Finland. To investigate the clinical presentation, comorbidities, and medications in a cohort of Finnish patients with confirmed BP managed in a university hospital setting. An observational retrospective study of all consecutive patients diagnosed with BP in 2012-2013 at the Department of dermatology, HUCH. The prevalence of the most common comorbidities was compared to that in a sample population aged over 30 years. Seventy patients were included (mean age: 77 years at diagnosis). The most common comorbidities were hypertension (44%), type 2 diabetes (34%), and ischaemic heart disease (26%). Almost half of the cohort had a neurological condition (46%). A statistically significant association was identified between BP and a past history of malignancies (17%; p<0.001), type 2 diabetes (24%; p<0.001), and chronic obstructive lung disorder (COPD) (10%; p=0.004), compared to an age-matched control group. The most common standard drugs were statins, beta-blockers, and diuretics. In total, 83% of the patients with type 2 diabetes took anti-diabetic treatments, mainly metformin (80%) and gliptins (55%). Significant associations were identified between BP and COPD, type 2 diabetes, and a past history of malignancy, compared to the general population. In this study, the specific role of some medications, such as gliptins, may account for the onset of BP in diabetic type 2 patients.