Childhood Bullous Pemphigoid: Report of Three Cases

Voltan, Erika; Maeda, Juliana Yumi; Silva, Mariana Andrade Muniz; Maruta, Celina Wakisaka; Santi, Cláudia Giuli; Zimbres, Silvia de Almeida; Aoki, Valéria

doi:10.1111/j.1346-8138.2005.tb00912.x

Cited by 14 publications

(13 citation statements)

References 34 publications

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“…Bullous pemphigoid is an autoimmune blistering disease that typically affects the elderly. It is rare in children 1 and even more so during the infantile period 1–11 . To date, less than 30 cases have been reported in infants 1–11 .…”

Section: Discussionmentioning

confidence: 99%

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Bullous pemphigoid (BP) in an infant complicated by tuberculous meningoencephalitis

Nanda

Dvořák

et al. 2007

Int J Dermatology

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A 13-month-old female infant was referred to us in April 2005 with a progressive, wide spread eruption of 1 month's duration. She was noticed to have perioral vesicobullous lesions that soon spread to affect the extremities and trunk. The parents denied a history of fever, throat infection, vaccination, or drug intake prior to onset of her rash. She was delivered at term by normal vaginal delivery and the antenatal history of her mother was unremarkable. She was observed to have normal developmental milestones and there was no history of associated medical ailments. On examination, her height was > 25th centile and weight was at 50th centile. She was observed to have multiple tense blisters, crusted lesions, and erosions on the face, trunk (Fig. 1), and extremities ( Fig. 2) including palms and soles, urticarial papules and plaques on the back, and erosions of the oral mucosa.Skin biopsy specimen from a vesicobullous lesion showed a subepidermal blister with mixed inflammatory cell infiltrate in upper dermis containing many eosinophils. Direct immunofluorescence on the perilesional skin showed linear deposition of IgG ( Fig. 3) and C3 at the basement membrane zone. Indirect immunofluorescence on saline split skin revealed immunoreactants on the epidermal side of the split (Fig. 4). Bullous phempigoid (BP) enzyme-linked immunosorbent assay (recombinant NC16A domain of 180 kDa) was positive = 219.9 (control ≤ 20). Complete blood counts revealed mild leukocytosis = 15.2 × 10 9 /L ( n = 4-11 × 10 9 / L), mild eosinophilia = 7% ( n = 2-6%), microcytic hypochromic anemia = 9.5 g / L ( n = 11.5-17.5 g / L), and mildly elevated erythrocyte sedimentation rate = 28 mm / h. Iron profile showed a low serum iron = 1.2 µ mol/L ( n = 6 -27.0) and decreased serum iron saturation = 2% ( n = 20 -40). Ultrasound of the abdomen and pelvis showed grade II hydronephrosis that improved after a prolonged course of antibiotics (cephalosporins). A follow-up technetium-99 m dimercaptosuccinic acid (Tc-99 m DMSA) renal cortical scintigraphy was normal. Various other investigations including serum biochemistry, liver and renal function tests, urinalysis, Hb electrophoresis, chest X-ray, glucose 6-phosphate dehydrogenase, serum immunoglobulins, and hepatitis screening were within the normal limits. She was given intravenous immunoglobulins (IVIG) 400 mg / kg /day for 5 consecutive days along with prednisolone (1 mg / kg /day). Within 5 days of treatment she stopped getting new lesions and showed resolution of most of the earlier skin lesions. Three weeks later while receiving prednisolone 0.75 mg / kg /day, she presented back to us with an acute relapse. The course of IVIG was repeated and dapsone (1 mg / kg /day) was added. A third course of IVIG was given a month later and the dose of prednisolone was reduced to 0.5 mg / kg /day. A week later, she was brought with persistent vomiting and high-grade fever and was hospitalized. Prednisolone and dapsone were temporarily discontinued. A few days later, she was observed to develop weakness of ri...

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Section: Discussionmentioning

confidence: 99%

“…In 1998, Amos et al 2 . summarized the data on 10 cases of infantile BP and subsequently more cases were reported 1,5–11 . BP in infants and children may present either as a localized variant with lesions localized to palms and soles or vulva, or a generalized vesicobullous eruption 1,2,9,10 .…”

Section: Discussionmentioning

confidence: 99%

Bullous pemphigoid (BP) in an infant complicated by tuberculous meningoencephalitis

Nanda

Dvořák

et al. 2007

Int J Dermatology

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“…Systemic corticosteroids are the best choice for the initial treatment of JBP. In children, as in adults, a minimal dose of drugs that controls the disease is recommended (2,10). Powell et al analyzed immunobullous disease in children and its good response to sulfa drugs and macrolides (11).…”

Section: Discussionmentioning

confidence: 99%

Juvenile Bullous Pemphigoid – a Case Report

Kostić¹,

Sekulović²,

Zecević³

2013

Serbian Journal of Dermatology and Venerology

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Bullous pemphigoid is an autoimmune blistering disease that predominantly affects elderly persons and rarely children. We present a 12-year-old girl with sudden appearance of tense blisters on an erythematous base on the trunk, neck, hands and legs with intense pruritus. Standard laboratory test results were within the normal range except for blood eosinophilia of 12% of the total white cell count. Skin biopsy specimens showed evolving subepidermal blisters with perivascular lymphohistiocytic, eosinophil and neutrophil infiltrations in the papillary dermis. Direct immunofluorescence of perilesional skin showed linear, continuous deposits of IgG and C3 along the dermoepidermal junction. Indirect immunofluorescence showed circulating anti-basement membrane zone IgG autoantibodies at a titer of 1:80. We started treatment with systemic corticosteroids, methylprednisolone 0,5 mg/kg per day and 500 mg erythromycin 4 times a day during 10 days. After 3 days 50 mg dapsone (DDS, 4,4-diaminodiphenylsulphone) per day was added. After a few days, there were no new changes on the skin and pruritus disappeared completely.

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“…The age ranged from 2.5 months to 14 years, with 15 cases at the age of 1 year or younger and 41 cases younger than 8 years. To the best of our knowledge, up to November 2008, there were 28 additional cases, giving 81 as the total number of published cases of JBP 5–16 . The disease most often occurs in children older than 8 years.…”

Section: Discussionmentioning

confidence: 99%

“…To the best of our knowledge, up to November 2008, there were 28 additional cases, giving 81 as the total number of published cases of JBP. [5][6][7][8][9][10][11][12][13][14][15][16] The disease most often occurs in children older than 8 years. The average age at JBP onset in our study was 10 years, with a female to male ratio of 2 : 1.…”

Section: Discussionmentioning

confidence: 99%