Sex: F
Vaccine: VARZOS, VARZOS
Symptoms: Autoimmune blistering disease, Bacterial test negative, Biopsy skin normal, Blister, Burning sensation
Clinical narrative: Blistering autoimmune skin reaction; Bullous pemphigoid; Blistering autoimmune skin reaction; Bullous pemphigoid; pemphigus vulgaris; acantholytic dermatosis; Erythematous rash/erythematous plaque-like eruption; raised pustules; papular outbreaks around both ears, left upper arm, and right hand; developed redness, blisters, and burning on the right side of her face; developed redness, blisters, and burning on the right side of her face/spongiotic vesicles contain eosinophils; developed redness, blisters, and burning on the right side of her face; mildly weepy; rash on her face (forehead, cheeks, ears), anterior chest, arms, legs, and abdomen); Erythematous rash; Pustular rash; Papular rash; This case was reported in a literature article and described the occurrence of autoimmune blistering disease in a 74-year-old female patient who received Herpes zoster (Shingrix) for prophylaxis. Blistering autoimmune skin reaction following SHINGRIX vaccination in an ulcerative colitis patient:  Co-suspect products included Herpes zoster (Shingrix) for prophylaxis.   The patient's past medical history included ulcerative (chronic) proctosigmoiditis. Previously administered products included diphenoxylate/atropine, mesalamine, levothyroxine (25mcg), gabapentin (300 mg), nortriptyline (25 mg), naratriptan (2.5 mg), hydroxyzine (25 mg), nitrofurantoin (100 mg), pentosan polysulfate (100 mg) and multiple vitamins. Concurrent medical conditions included alcoholic (drank one glass of wine per week).   In April 2019, the patient received the 1st dose of Shingrix. On an unknown date, the patient received the 2nd dose of Shingrix. On an unknown date, 40 days after receiving Shingrix, not applicable after receiving Shingrix and an unknown time after starting terbinafine, the patient experienced autoimmune blistering disease (serious criteria GSK medically significant), bullous pemphigoid (serious criteria GSK medically significant), autoimmune blistering disease (serious criteria GSK medically significant), bullous pemphigoid (serious criteria GSK medically significant), pemphigus vulgaris (serious criteria GSK medically significant), transient acantholytic dermatosis, red rash, pustular rash, papular rash, red face, blister, burning sensation in face, weepy, rash, red rash, pustular rash and papular rash. The patient was treated with valaciclovir hydrochloride (Valacyclovir), terbinafine, cephalexin and corticosteroid nos (Corticosteroids Nos). Rechallenge with Shingrix was positive. On an unknown date, the outcome of the autoimmune blistering disease, bullous pemphigoid, autoimmune blistering disease, bullous pemphigoid, pemphigus vulgaris, transient acantholytic dermatosis, red rash, pustular rash, papular rash, red face, blister, burning sensation in face and weepy were recovered/resolved and the outcome of the rash, red rash, pustular rash and papular rash were unknown.   The reporter considered the autoimmune blistering disease, bullous pemphigoid, pemphigus vulgaris, transient acantholytic dermatosis, red rash, pustular rash, papular rash, red face, blister, burning sensation in face and weepy to be related to Shingrix. The reporter considered the autoimmune blistering disease, bullous pemphigoid, rash, red rash, pustular rash and papular rash to be related to Shingrix.  Additional details were provided as follows: This case was reported in a literature article and described the occurrence of blistering autoimmune skin reaction/Bullous pemphigoid (BP) in a 74-years old female patient, who was vaccinated with SHINGRIX (GlaxoSmithKline) for prophylaxis. The patient was female. The patient had history of ulcerative proctosigmoiditis, controlled with fiber supplement, diphenoxylate/atropine, and mesalamine as needed. The patient drank one glass of wine per week and had never smoked or used any illicit drugs. Other long-term medications include levothyroxine 25mcg, gabapentin 300 mg, nortriptyline 25 mg, naratriptan 2.5 mg, hydroxyzine 25 mg, nitrofurantoin 100 mg, pentosan polysulfate 100 mg and multiple vitamins. No information on patient's family history was provided. On an unspecified date in April 2019, the patient received 1st dose of SHINGRIX (administration route and site unspecified; batch number not provided). The age of vaccination was not provided but it could be 73 years or 74 years. On an unspecified date in 2019, 40 days after the 1st dose of vaccination, new rash initially started and appeared erythematous with raised pustules. [The new rash initially started 40 days after her first SHINGRIX vaccination and appeared erythematous with raised pustules]. The patient also developed redness, blisters, and burning on the right side of her face. The patient denied fevers, joint pains, chest pain, shortness of breath or cough. The patient was initially prescribed valacyclovir 1000 mg three times daily for presumed diagnosis of shingles. Despite compliance, the patient was noted to have erythematous plaque-like eruption over the right eye that crossed the midline and was mildly weepy", along with papular outbreaks around both ears, left upper arm, and right hand. The patient was then treated with cephalexin and terbinafine, however the rash continued to worsen. The patient underwent a biopsy and culture of the rash which showed no growth of aerobic bacteria or fungi. On histology, the biopsy showed intra-epidermal blister with eosinophils" with comment of spongiotic vesicles contain eosinophils, as does the upper part of the dermis" and that the findings were evidence for either re-epithelialized lesions of bullous pemphigoid or an intra-epidermal acantholytic dermatosis such as pemphigus vulgaris." Direct immunofluorescence studies, likely for antibodies such as Desmoglein 1, 3, bullous pemphigoid (BP) 180 and BP 230 for the autoimmune bullous diseases, were recommended by the pathology laboratory, but not obtained by the clinician. It was recommended to correlate with clinical findings. The rash completely resolved after a 20-day steroid taper with topical corticosteroid for 1-2 weeks and then only as needed for flares. On an unspecified date, less than two months after finishing the steroid taper, the patient received 2nd dose of SHINGRIX (administration route and site unspecified; batch number not provided). On an unspecified date, within less than 2 months after the 2nd dose of vaccination, the patient had recurrence of the same rash. The patient was treated with oral corticosteroid for one week, and the rash resolved. [Less than two months after finishing the steroid taper, the patient received the second dose of SHINGRIX and had recurrence of the same rash. The second rash occurrence, treatment, and resolution all occurred within less than 2 months of receiving the second vaccination. The patient presented on two occasions with a rash on her face (forehead, cheeks, ears), anterior chest, arms, legs, and abdomen after receiving the SHINGRIX vaccine in April 2019]. The author commented, "There have been multiple accounts of inflammatory Bowel Disease (IBD) patients developing autoimmune blistering skin disorders, and in some cases, these have been associated with specific triggers such as vaccination. Some studies have proposed mechanisms for the pathophysiology of these associations, however there is not a clear consensus on the cause. In terms of overall frequency, one review of 48 cases of patients with autoimmune skin diseases and IBD reported that the most frequently occurring autoimmune skin diseases were linear IgA disease and BP. Another study concluded that pemphigus was significantly associated with ulcerative colitis (UC) with an odds ratio of 1.9 but did not hypothesize a mechanism. BP has also been reported in multiple infants after HSV-1 herpetic stomatitis and after vaccination against diphtheria, tetanus, pertussis, poliomyelitis, hepatitis B, and H. influenzae type b. This study confirmed the presence of BP antigen 180 and resolution of symptoms with oral prednisone treatment. The authors thought that trauma from vaccine injection could have led to activation of Th17 cells and IL-17 production causing proteolytic enzyme and cytokine induced blister formation. These proposed mechanisms share the unifying theme of a specific environmental trigger from vaccination causing an increased immune response that manifests on the skin. We believe there to be enough evidence amongst these studies in the current literature to support a possible association between a vaccination and the development of specific autoimmune disorders, particularly in those who may be predisposed to autoimmune conditions. Additionally, the recurrence of the same reaction after each dose of a vaccine (a positive re-challenge) is further confirmation of a causal association between the vaccine and an adverse reaction. Given the temporal relationship between vaccination, the development of BP, and resolution with steroids with each dose of SHINGRIX, we believe that the SHINGRIX vaccine led to the development of BP in this patient."