TY - JOUR
T1 - Bullous Diseases in Children
T2 - A Review of Clinical Features and Treatment Options
AU - Schultz, Brittney
AU - Hook, Kristen
N1 - Publisher Copyright:
© 2019, Springer Nature Switzerland AG.
PY - 2019/10/1
Y1 - 2019/10/1
N2 - Bullous diseases are uncommon in children; however, as they have the potential to affect quality of life, occasionally have long-term side effects in the setting of scarring processes, and carry a rare risk of underlying malignancy [e.g., with paraneoplastic pemphigus (PNP)], knowledge of their clinical presentation and treatment options is essential. Given the rarity of these conditions, our current state of knowledge is largely derived from case reports and case series, with a paucity of evidence-based recommendations. In this review, we discuss the clinical presentation of and treatment options for linear immunoglobulin A disease, dermatitis herpetiformis, pemphigus vulgaris, pemphigus foliaceus, PNP, bullous pemphigoid, mucus membrane pemphigoid, epidermolysis bullosa acquisita, and inherited epidermolysis bullosa. In general, when these conditions, except for PNP, occur in childhood, they have a better prognosis than when they occur in adults. Clinical, histopathological, and immunologic features frequently overlap, but distinct differences have also been reported, most commonly in clinical presentation. Treatment is often similar to that in adults, although specific considerations are necessary for a pediatric population.
AB - Bullous diseases are uncommon in children; however, as they have the potential to affect quality of life, occasionally have long-term side effects in the setting of scarring processes, and carry a rare risk of underlying malignancy [e.g., with paraneoplastic pemphigus (PNP)], knowledge of their clinical presentation and treatment options is essential. Given the rarity of these conditions, our current state of knowledge is largely derived from case reports and case series, with a paucity of evidence-based recommendations. In this review, we discuss the clinical presentation of and treatment options for linear immunoglobulin A disease, dermatitis herpetiformis, pemphigus vulgaris, pemphigus foliaceus, PNP, bullous pemphigoid, mucus membrane pemphigoid, epidermolysis bullosa acquisita, and inherited epidermolysis bullosa. In general, when these conditions, except for PNP, occur in childhood, they have a better prognosis than when they occur in adults. Clinical, histopathological, and immunologic features frequently overlap, but distinct differences have also been reported, most commonly in clinical presentation. Treatment is often similar to that in adults, although specific considerations are necessary for a pediatric population.
UR - http://www.scopus.com/inward/record.url?scp=85072718192&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85072718192&partnerID=8YFLogxK
U2 - 10.1007/s40272-019-00349-3
DO - 10.1007/s40272-019-00349-3
M3 - Review article
C2 - 31506891
AN - SCOPUS:85072718192
SN - 1174-5878
VL - 21
SP - 345
EP - 356
JO - Pediatric Drugs
JF - Pediatric Drugs
IS - 5
ER -