Please login or create an account. If you do not have access to this content, you will be shown a 30 second preview and licensing options.
- Presentation
What's That Blistering Disease?
Description
The presentation focuses on blistering diseases, particularly in the context of connective tissue diseases like systemic lupus erythematosus (SLE). The first case discusses a patient with a severe blistering condition initially thought to be toxic epidermal necrolysis (TEN) but later identified as Rowl Syndrome, characterized by an overlap of lupus and erythema multiforme (EM) lesions. Key clinical findings included distinctive blistering patterns and specific antibody profiles. The second case described a woman with systemic lupus who presented with erosions and blisters resembling pemphigus, ultimately diagnosed as pemphigus erythematosus, a milder variant linked with lupus. Throughout the presentation, distinctions between these conditions and common rashes were highlighted, emphasizing the connection between sun exposure and the onset of these blistering diseases. The importance of careful diagnosis and management of each condition was underlined, including the usage of specific medications like hydroxychloroquine to achieve resolution in patients.
View moreConclusions
- Rowell Syndrome involves the overlap of lupus erythematosus and erythema multiforme-like lesions, often triggered by sun exposure.
- The diagnosis of Rowell Syndrome can be supported by the presence of a speckled ANA pattern and positive anti-Ro/SSA antibodies.
- Pemphigus Erythematosus is characterized by features of pemphigus foliaceus with lupus-like implications, particularly in photodistributed areas.
- Both Rowell Syndrome and Pemphigus Erythematosus may present with blistering rashes that involve the malar region of the face.
- Histopathological evaluations for these conditions may not always provide clear distinctions, necessitating clinical correlation.
- Patients with Rowell Syndrome may present without prior lupus history, indicating that the syndrome can be the initial presentation of lupus.
- Medication triggers for Rowell Syndrome include terbinafine and PPIs, while Pemphigus Erythematosus can be associated with drugs like penicillamine and captopril.
- Effective management of Rowell Syndrome involves transitioning to hydroxychloroquine and methotrexate after initial treatment with cyclosporine.
- Kim SS et al. HSSJ. 2013;9:289-92.
- Shteyngarts AR et al. J Am Acad Dermatol. 1999;40:773-7.
- Champagne et al. CIn Exp Dermatol 2012; 37:822-3.
- Murad et al. BMJ Case Rep 2015.
- Branisteanu et al. Exp Ther Med 2018; 15: 785-8.
- Pozharashka J, Acta Dermatovernerol Croat 2019; 27:124-6.
- Baron et al. J Dtsch Dermatol Ges 2014; 12:1039-42.
- Kacalak-Rzepka et al. Clin Exp Dermatol 2009; 34:702-4.
- Amerian M and Ahmed RA. Int J Dermatol. 1985;24(1):16-25.
- Kridin et al. Immunol Res. 2019 Feb;67(1):116-122.
- Oktarina DAM et al. Arch Dermatol. 2012;148(10):1173-1178.
- Steffen C and Thomas D. Am J Dermatopathol. 2003;25(5):432-36.
- Jablonska et al. Arch Dermatol Res. 1977;259(2):135-40.
- Chandan et al. Dermatol Online J. 2018;24(1):13030.
- Errichetti E, Lupus. 2019 Nov;28(13):1583-1588.