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- Presentation
Challenging Adult and Pediatric Autoimmune Connective Tissue Disease Cases: Pearls for Diagnosis and Management
Description
The presentation discusses two complex cases related to autoimmune connective tissue diseases, focusing on Raoul syndrome and pemphigus erythematosus (senior Usher syndrome). The first case involves a 31-year-old pregnant woman with systemic lupus, presenting with a worsening rash characterized by violaceous plaques and erosions, leading to a diagnosis of Raoul syndrome, a rare condition where lupus coexists with erythema multiforme-like lesions. The patient experienced complications, including infections and severe preeclampsia, ultimately receiving treatment with high-dose steroids and mycophenolate, resulting in significant improvement in her skin lesions. The second case describes a patient with systemic lupus and blindness, suffering from pemphigus erythematosus, presenting with scaly erosions and lesions predominantly in sun-exposed areas. Histological analysis confirmed the diagnosis, with the patient responding well to corticosteroids and hydroxychloroquine. Key aspects of diagnosis and management for both conditions are highlighted, including the significance of identifying characteristic skin presentations and recognizing potential drug-related triggers. The importance of early treatment and sun protection in managing these autoimmune conditions is emphasized, given their potential for exacerbation and complex overlap.
View moreConclusions
- Rowell syndrome is an association between lupus erythematosus and erythema multiforme that can occur without clear precipitating factors.
- The diagnosis of Rowell syndrome is more common in women and can be exacerbated by sun exposure and certain medications.
- Rowell syndrome may be accompanied by a positive speckled pattern of antinuclear antibodies and can occur in the absence of known triggers.
- Pemphigus erythematosus is a variant of pemphigus foliaceus with features of lupus and is characterized by photodistributed cutaneous lesions.
- Both Rowell syndrome and pemphigus erythematosus can induce severe skin conditions and may be drug-induced.
- Treatment for Rowell syndrome includes systemic corticosteroids and immunosuppressive therapy, often resulting in significant improvement of lesions.
- Pemphigus erythematosus can also be effectively treated with corticosteroids and other therapies, leading to rapid improvement of skin lesions.
- Both conditions are sensitive to UV light and require careful management to prevent exacerbations and complications.
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