Erasmus Syndrome: A Case Report and Literature Review.

BACKGROUND Erasmus syndrome is a rare disease entity characterized by the development of systemic sclerosis (SSc) in a background of silica exposure or silicosis. CASE REPORT We report the case of a 40-year-old Filipino man who previously worked in a silica grind mill for 10 years and eventually developed Erasmus syndrome. The patient initially presented with chronic back pain in 2018 associated with findings of pulmonary tuberculosis on chest X-ray, with notable improvement after 6 months of anti-tuberculosis treatment. However, his back pain recurred in 2021; this time with arthralgia, Raynaud's phenomenon, thickening of both hands, skin hypopigmentation on the chest, back, and forehead, and exertional dyspnea. Physical examination revealed salt-and-pepper dermopathy and skin tightening over the back, chest, and extremities. Mobility of his hands was limited, associated with sclerodactyly and digital pitting. Antinuclear antibody-immunofluorescence and anti-scleroderma-70 antibodies were strongly positive, confirming the diagnosis of SSc. Chest computed tomography illustrated multiple subcentimeter nodules and enlarged mediastinal lymph nodes with eggshell calcifications, consistent with silicosis. Spirometry with body plethysmography was normal but diffusing capacity for carbon monoxide was severely reduced. Histopathology of the skin showed markedly thickened collagen bundles in the dermis. CONCLUSIONS Chronic silica exposure is a risk factor for the development of silicosis. The clinical course of patients with silicosis may be complicated by SSc. Maintaining a high index of suspicion is key to the diagnosis of Erasmus syndrome. The present report emphasizes the importance of preventive measures and surveillance among those with occupational exposure to silica. To our knowledge, this is the first documented case of Erasmus syndrome in the Philippines.

Atopy patch tests may identify patients at risk for systemic contact dermatitis.

BACKGROUND: A subset of patients with positive patch tests demonstrates systemic contact dermatitis (SCD) upon ingestion or inhalation of the allergen. Concern has been raised about the use of patch tests for protein allergens (APTs) to detect SCD in atopic dermatitis (AD) patients. METHODS: We present atopy patch test (APT) data for 97 people. We reviewed APTs and tests for antigen-specific immunoglobulin E (IgE) to the same allergen in pediatric AD patients. We compared the frequency of APTs as a function of age in AD patients. To study the irritancy potential of APTs, we prospectively tested consenting non-AD dermatitis patients undergoing evaluation for allergic contact dermatitis and healthy controls to an APT panel. RESULTS: APT demonstrated fewer positive results than serum-specific IgE or skin prick tests to the same allergen. Positive APT to food was more common in children under 3 years, whereas positive APT to aeroallergens were more common in teens and adults. Only positive APTs to dust mite were significantly more common positive in subjects without AD. CONCLUSION: Our aggregate findings suggest that most APTs, but not dust mite, behave like conventional patch tests to low-potency allergens. They are more likely to be positive in patients with chronically inflamed skin and to identify allergens that cause SCD. The higher prevalence of APT positivity to foods in young children is consistent with food allergy as a trigger of AD (also known as SCD) being more common in children than adults. Positive APTs define patients who may have SCD; negative APTs may guide elimination diets.