Age: 0.17
Sex: F
Vaccine: IPV, PPV, RVX, TDAP
Symptoms: Rash vesicular, Skin oedema, Skin plaque, Upper respiratory tract infection, White blood cell count increased
Clinical narrative: Bullous pemphigoid; blistering rash; erythematous, edematous,polycyclic plaques distributed over the face, trunk, extremities, hands, and feet; erythematous, edematous,polycyclic plaques distributed over the face, trunk, extremities, hands, and feet; erythematous, edematous,polycyclic plaques distributed over the face, trunk, extremities, hands, and feet; Scattered tense bullae within a widespread annular rash on the leg; upper respiratory infection; This case was reported in a literature article and described the occurrence of bullous pemphigoid in a 2-month-old female patient who received DTPa (DTPa vaccine) for prophylaxis. .  Co-suspect products included Rotavirus vaccine for prophylaxis, POLIOMYELITIS VACCINE INACTIVATED (INACTIVATED POLIO VACCINE) for prophylaxis and PNEUMOCOCCAL VACCINE (PCV13) (batch number Unknown, expiry date unknown) for prophylaxis.   On an unknown date, the patient received DTPa vaccine, Rotavirus vaccine, INACTIVATED POLIO VACCINE and PNEUMOCOCCAL VACCINE (PCV13). On an unknown date, 1 day after receiving DTPa vaccine and Rotavirus vaccine, the patient experienced bullous pemphigoid (serious criteria GSK medically significant), blistery rash, skin plaque, red rash, skin edema, bullae and upper respiratory tract infection. The patient was treated with methylprednisolone (Methylprednisolone Intravenous), prednisolone, erythromycin, niacinamide and dapsone. On an unknown date, the outcome of the bullous pemphigoid, blistery rash and red rash were recovering/resolving and the outcome of the skin plaque, skin edema, bullae and upper respiratory tract infection were unknown.   The reporter considered the bullous pemphigoid, blistery rash, skin plaque, red rash, skin edema, bullae and upper respiratory tract infection to be possibly related to DTPa vaccine and Rotavirus vaccine.  Additional details were provided as follows: This case was reported in a literature article and described the occurrence of Bullous pemphigoid (BP); Blistering rashes originating on acral sites in 2-month-old female patient who had received unspecified diphtheria, tetanus, and acellular pertussis (Dtap), unspecified polio [inactivated polio vaccine (IPV)]; unspecified haemophilus influenzae type B vaccine (Hib); Rotavirus [RV5] and unspecified pneumococcal conjugate vaccine 13 (PCV13) (manufacturer unknown for all vaccines) for prophylaxis). This case correspond to case no 2 reported in literature article.  No information on medical or family history or concurrent condition or concomitant medications.  On unspecified date, at the age of 2 month old, the day before the onset of blistering rash, the patient received unspecified diphtheria, tetanus, and acellular pertussis (Dtap), unspecified polio [inactivated polio vaccine (IPV)]; unspecified haemophilus influenzae type B vaccine (Hib); Rotavirus [RV5] and unspecified pneumococcal conjugate vaccine 13 (PCV13) (administration route unknown; batch number not provided for all vaccines).  On an unspecified date, at the age of 2 month, the day after vaccination, the patient presented with a widespread blistering rash that began on her feet. On examination, the patient had erythematous, edematous, polycyclic plaques distributed over the face, trunk, extremities, hands, and feet (Scattered tense bullae within a widespread annular rash on the leg.), including involvement of both the dorsal and ventral surfaces of the hands and feet. There were numerous overlying tense and ruptured bullae (Ruptured bullae over a bed of erythematous, edematous, polycyclic plaques on the lower leg and foot.). No mucosal lesions were observed.  The patient's skin biopsy for hematoxylin-eosin stain found eosinophilic and neutrophilic spongiosis with small clusters of neutrophils in the papillary dermal tips. Perilesional biopsy for direct immunofluorescence showed linear IgG and C3 along the dermoepidermal junction and weak linear IgA. Serum enzyme-linked immunosorbent assay showed BP180 greater than 100 U and normal BP230. Indirect immunofluorescence performed with the patient's serum showed basement membrane zone IgG antibodies in an epidermal pattern on human salt-split skin substrate. Complete blood count (CBC) was notable for white blood cell (WBC) count of 27.8 K/µL (normal, 6-18 K/µL), platelets of 1,022 K/µL (normal, 150-400 K/µL), and absolute eosinophil count of 4.6 K/µL (normal, 0-0.9 K/µL). Thus, the patient showed common clinical and laboratory features of the disease. Both manifested as a blistering rash on acral skin surfaces followed by widespread skin involvement sparing the mucosae. Subsequently, the patient diagnosed with infantile Bullous pemphigoid (BP).  The patient was treated with intravenous methylprednisolone for 3 days followed by oral prednisolone titrated up to 2 mg/kg/d. Given ongoing new lesions, oral erythromycin, 50 mg/kg/d, and niacinamide, 40 mg 3 times daily were added. 3 weeks after treatment started, she still had widespread, active disease, so dapsone was added at 0.5 mg/kg/d and titrated up to 1.5 mg/kg/d. The patient continued to have active cutaneous disease, and laboratory values still showed elevated WBCs and platelets with absolute eosinophils increasing to 7.34 K/µL. 3 weeks after initiation of dapsone, the patient began to improve. Within days of obtaining disease control, her absolute eosinophil count normalized to 0.01 K/µL with decreases in her WBC and platelet counts. Erythromycin and niacinamide were discontinued. A steroid taper was initiated 3 months later, and a dapsone taper began after another month. A mild flare occurred with an upper respiratory infection, so dapsone was increased back to its prior dose, and erythromycin and niacinamide were restarted.  The patient successfully tapered off systemic steroids while being monitored for adrenal insufficiency 6 months after initial presentation.  The author commented, "The inciting factors of infantile BP are not fully understood, but stimulation of the immune system by vaccination has been proposed as a trigger. As the presented cases and previous literature demonstrate, a history of recent vaccination is not universal, and the time from vaccination to disease manifestation varies greatly. In our case, the patient received vaccinations 1 day before her rash started, which suggests the vaccines may have been a trigger. Infantile BP has near-universal involvement of acral sites often followed by a generalized spread and infrequent mucosal involvmement. The elevated BP180 levels with normal BP230 levels in both cases is representative of infantile BP, which manifests with elevation of BP180 and rarely BP230. The patient showed prominent and persistent eosinophilia on her CBC that only decreased once she stopped getting new skin lesions. Previous case reports similarly show eosinophilia on CBC, suggesting eosinophil counts as a possible marker for disease activity and treatment response."