Bullous pemphigoid in a 3-month-old infant: case report and literature\nreview of this dermatosis in childhood
White infant female, three months and fifteen days old, developed multiple tight bullae\nfive days ago, initially on palms and soles, with progressive involvement of trunk,\nlimbs and head, accompanied by papules and plaques associated or not to blisters.\nParents were not consanguineous, denied fever or use of medications, as well as other\ncomorbidities. Personal and family history of atopy, other cutaneous diseases or\nautoimmune disorders was negative. The patient was born vaginally, at term, without\ncomplications. Exclusively breastfed. The patient received the recommended vaccines,\nwith the last dose about 45 days previously.\nDermatological examination revealed the presence of multiple vesicles and widespread\ntight blisters with serous content and in different sizes, affecting areas of normal as\nwell as erythematous skin. She presented serohemorrhagic crusts, papules and annular\nurticarial erythematous plaques, some topped by vesicles and blisters. Lesions were\nlocated predominantly in the hands, feet, scalp, limbs, trunk and back. There were no\nmucosal lesions. Nikolsky sign was negative ( to ). Complete blood count showed\nmild anemia (hemoglobin: 10.8 g / dL), eosinophilia (2,300 eosinophils/mm3 or\n26% of total leukocytes) and thrombocytosis (585,000 platelets). Histopathology showed\nspongiotic dermatitis with subepidermal bullae rich in neutrophils and eosinophils. DIF\ndetected the presence of linear IgG and C3 deposits along the subepidermal basal\nmembrane zone. Immunohistochemical staining for type IV collagen was positive at the\nbase of the blister, demonstrating that cleaving occurred above the lamina densa.\nSaltsplit skin technique and IIFs (indirect immunofluorescence) could not be performed.\n( to )\nThe set of clinical and laboratory data confirmed the diagnosis of infantile bullous\npemphigoid. Treatment was started with prednisolone 1mg/kg. After five days, due to\npartial response, the dose of prednisolone was increased to 2mg/kg/day. Reassessment\nafter fifteen days showed good control of the lesions, with only sparse escape blisters.\nVaccinations were suspended on account of the immunosuppressive doses of\ncorticosteroids. In order to permit corticosteroid weaning, dapsone was initiated at 0.5\nmg / kg. Previous control exams had shown normal G6PD levels and no subsequent worsening\nof anemia occurred with medication. Corticosteroid therapy was reduced by 0.5 mg / kg\nevery two weeks until its full suspension, while dapsone was maintained at 0.5 mg / kg /\nday. Patient remained without skin lesions for three months, and resumed the use of\nvaccines without live antigens according to the national vaccination calendar. At nine\nmonths old, she received acellular conjugated vaccines (DTPA), anti hepatitis B, polio\nand meningococcal diseases, with subsequent display of new bullous lesions. Dapsone was\nincreased to a dose of 1mg/kg and prednisolone 1mg/kg was initiated for a week. With\nregression of the clinical symptoms dapsone was maintained for six months in which\nperiod no new lesions appeared, even with the use of vaccines, so, the medication was\ndiscontinued. The patient is followed-up in the outpatient dermatology service, and she\nhas been lesion-free for six months without any signs of recurrence to date.