Bullous Pemphigoid Associated with Multiple System Atrophy: Case Series.

BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering dermatosis associated with a number of neurological conditions, including idiopathic Parkinson's disease (IPD). Only 1 case of BP in a patient with multiple system atrophy (MSA) has been reported. CASES: We report 3 cases of men with probable MSA who developed bullous pemphigoid at a latency of 4-6 years from MSA symptom onset. CONCLUSIONS: Skin α-synuclein deposition in neurodegenerative conditions such as IPD and MSA may be a potential substrate for the exposure of BP-related antigens. Alternatively, central neurodegeneration may expose antigens as a substrate for cross-reactivity and BP pathogenesis. Our report suggests an association between BP and MSA, in addition to the previously documented association with IPD.

Systemic drug photosensitivity-Culprits, impact and investigation in 122 patients.

BACKGROUND: Systemic drugs are a potentially reversible cause of photosensitivity. We explore prevalence, impact, phototest findings and culprit drugs. METHODS: Retrospective review of patients was diagnosed with drug-induced photosensitivity in a specialist photoinvestigation centre (2000-2016), using data recorded in standardized pro forma. Patients underwent detailed clinical evaluation. Monochromator phototesting was performed to 300 ± 5 nm, 320 ± 10 nm, 330 ± 10 nm, 350 ± 20 nm, 370 ± 20 nm, 400 ± 20 nm, 500 ± 20nm and 600 ± 20 nm. Broadband UVA and solar-simulated radiation (SSR) testing were performed, and photopatch testing and laboratory tests examined for other causes of photosensitivity. DLQI was evaluated. RESULTS: Prevalence of drug-induced photosensitivity was 5.4% (122/2243) patients presenting with photosensitivity. Patients with drug-induced photosensitivity were 52.5% female; median 62 years (range 11-86); phototype I (17.2%), II (39.3%), III (26.2%), IV (6.5%), V (4.1%). Fifty-five (45.1%) patients had reduced erythemal thresholds on monochromator phototesting: 83.6%% to UVA alone, 14.5% to both UVA and UVB, 1.8% to UVA and visible light; 61.4% (n = 75) showed abnormal response to broadband UVR. Drugs implicated: quinine (11.5%), diuretics (10.7%; thiazide 9.8%), antifungals (9.8%), proton-pump-inhibitors (9.8%), angiotensin-converting enzyme inhibitors (7.4%), anti-inflammatory drugs (6.6%), statins (5.7%), selective serotonin reuptake inhibitors (4.9%), calcium channel antagonists (3.3%), anti-epileptics (3.3%), tricyclic antidepressants (3.3%), beta-blockers (2.5%), antibiotics (2.5%), others (≤1.6% cases each). Emerging culprits included azathioprine (2.5%) and biologics (TNF-α inhibitors, denosumab; 2.5%). Median DLQI was 11 (range 2-27) for the past year. CONCLUSION: Classically described photosensitizing drugs such as thiazides and quinine remain common offenders, while emerging culprits include biologics such as TNF-a inhibitors and proton-pump-inhibitors. There is very large impact on life quality; identification facilitates measures including drug cessation and implementation of appropriate photoprotection.

Dermatology emergencies: handy hints for the acute medical team.

This article will help the general physician recognize and manage acute dermatology presentations. This can be challenging for non-dermatology doctors owing partly to the difficulty in providing an extensive dermatology undergraduate education and the lack of exposure to dermatology patients. The problem is further compounded at many hospital trusts because of the lack of on-site dermatology 'on-call'. The general physician must be able to recognize dermatology emergencies in order to provide initial management and maintain appropriate referrals to acute dermatology services. The emergency presentations discussed are erythroderma, life-threatening drug eruptions, cutaneous vasculitis, eczema herpeticum and bullous disorders.