A case of bullous pemphigoid following pemphigus foliaceus

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Doreen Horton
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1 #2228 A case of bullous pemphigoid following pemphigus foliaceus Priyanka Vedak MD 1, Danielle Levine MD 1,3, Lyn Duncan MD 2,3, Hensin Tsao 1,3, Daniela Kroshinsky MD MPH 1,3 1. Department of Dermatology, Massachusetts General Hospital 2. Department of Pathology, Massachusetts General Hospital 3. Harvard Medical School

2 Disclosures The authors wish to express that there are no conflicts of interest and no financial disclosures to report

3 Background Autoimmune Blistering Disease Bullous Pemphigoid Tense blisters Negative Nikolsky s sign BP180 and BP230 Linear IgG and C3 at dermalepidermal junction Pemphigus Foliaceus Small flaccid blisters and crusted erosions No mucosal involvement + Nikolsky s sign DSG1 Intercellular IgG +/- C3 3 Adapted from Kershenovich et al. Autoimmunity reviews 2014:13;

4 Case Report: Visit 1 71-year-old woman 12-year history of biopsy and DIF confirmed pemphigus foliaceus (PF) Manifested as erythematous plaques with crusting and scale of the trunk and scalp Managed on chronic low dose mycophenolate mofetil Presented to outpatient dermatology clinic with two weeks of new lesions on thighs and forearms Suspected PF flare Increased mycophenolate mofetil to 500 mg daily Initiated clobetasol 0.05% ointment 4

Case Report: Visit 2: 6 days later Intensely pruritic lesions with two morphologies: 1-2 cm bright red annular and arcuate plaques with peripheral edema

5 Case Report: Visit 2: 6 days later Intensely pruritic lesions with two morphologies: 1-2 cm bright red annular and arcuate plaques with peripheral edema without overlying scale or crust on lower extremities Mild erosions with significant overlying scale on back Loratidine initiated for idiopathic urticaria 5

6 Case Report: Visit 3: 1 month later Diffuse blistering of mixed morphology noted with multiple flaccid blisters, associated erosions and numerous intact, tense, Nikolsky negative blisters 6

of IgG(1+) and C3(2+) along the dermal epidermal junction ELISA: antibodies to DSG1 and

7 Case Report: Visit 3 (continued): Right thigh perilesional punch biopsy Subepidermal bullous dermatosis with numerous eosinophils Direct immunofluorescence: linear deposition of IgG(1+) and C3(2+) along the dermal epidermal junction ELISA: antibodies to DSG1 and BP180, but not to DSG3 or BP230 Dx: simultaneous pemphigus foliaceous and bullous pemphigoid 7

8 Case Report: Therapy Mycophenolate mofetil 1500 mg twice daily Prednisone 20mg daily Clobetasol 0.05% ointment to new lesions under occlusion daily 18 months after bullous pemphigoid diagnosis Patient was doing well with no skin lesions Mycophenolate mofetil taper of 500mg each week for three weeks, followed by mycophenolate mofetil discontinuation 8

9 Discussion: Coexistence of two autoimmune blistering diseases First described by Chorzelski T, et al. in patients with pemphigus vulgaris and bullous pemphigoid Subsequent reports: 9 Timespan for development of concomitant autoimmune blistering diseases has ranged from coexistent at time of presentation to years prior to development of second process Pemphigus vulgaris/bullous pemphigoid Pemphigus foliaceus/bullous pemphigoid Both men and women Chorzelski T, et al. Arch Dermatol 1974:109: Korman NJ, et al. Arch Dermatol 1991:127; Maeda JY, et al. Clin Exp Dermatol 2006:31;

10 Discussion Epitope spreading (ES): An epitope is an antigenic determinant to which a specific antibody binds ES refers to the development of an immune response to epitopes distinct from and non-cross-reactive with the disease causing epitope In autoimmunity ES refers to the development of immune responses against endogenous epitopes secondary to the release of self-ag during a chronic auto immune response Implicated in type 1 diabetes, rheumatoid arthritis, multiple sclerosis, systemic lupus erythematosus, and others. Coexistent bullous pemphigoid and pemphigus represents intermolecular progression of antibodies from hemidesmosome to desmoglein protein antigens 10

11 References 1. Kershenovich R, et al. Diagnosis and classification of pemphigus and bullous pemphigoid. Autoimmunity reviews 2014:13; Maeda JY, Moura AKA et al. Changes in the autoimmune blistering response: a clinical and immunopathological shift from pemphigus foliaceus to bullous pemphigoid. Clin Exp Dermatol 2006:31; Korman NJ, Stanley JR, Woodley DT. Coexistence of pemphigus foliaceous and bullous pemphigoid. Arch Dermatol 1991:127; Chorzelski T, et al. Coexistence of pemphigus and bullous pemphigoid. Arch Dermatol 1974:109: Cornaby C, et al. B cell epitope spreading: Mechanisms and contribution to autoimmune disease. Immunology Letters 2015:163:

Autoimmune Diseases with Oral Manifestations

Autoimmune Diseases with Oral Manifestations Martin S. Greenberg DDS, FDS RCSEd Professor Emeritus Department of Oral Medicine University of Pennsylvania Disclosure Statement I have no actual or potential