Bullous pemphigoid in adolescence

Abstract

Bullous pemphigoid (BP) is the most common autoimmune blistering disease affecting the elderly but is quite rare in childhood. The majority of pediatric cases have been reported during early childhood. Adolescence is divided into three phases: early (10‐13 years), middle (14‐17), and late (18‐21). This review aimed to identify BP cases in adolescence and demonstrate their clinical features and course. Our literature search was performed in Medline with the terms “bullous pemphigoid in childhood and adolescence,” “childhood bullous pemphigoid,” “juvenile bullous pemphigoid,” and “autoimmune blistering and autoimmune bullous diseases in childhood.” The data extraction for late adolescence was limited by the fact that this age group is included in adult BP registries. We identified nine cases in early adolescence. Mucosa were affected in 5 of 9 cases. Treatment consisted of systemic prednisone (8/9), in combination with dapsone (2/9), azathioprine (2/9), or erythromycin/nicotinamide (1/9). Relapses were reported in 3 of 9 cases. We identified five cases occuring in middle adolescence. Mucosa were not affected. Treatment consisted of systemic prednisone (5/5), in combination with dapsone (3/5), azathioprine (2/5), doxycycline/nicotinamide (1/5), or mycophenolate mofetil (1/5). Relapses were reported in two of five cases. No case of BP in the late adolescence was included in the results, as only one case met the search criteria, and overlapped with pemphigus vulgaris. With only 14 cases found in our review, BP in adolescence appears even rarer than in earlier childhood. Despite its low prevalence, BP should be included in the differential diagnosis of autoimmune blistering diseases in adolescents.

Bullous pemphigoid in adolescence, a rare demographic

“…Of the nine reported cases in early adolescence, six had generalised eruptions, one was localised to the vulva, and four had mucosal involvement. The lesions in the middle adolescence group tended to be more generalised with no mucosal involvement reported prior to our case study 1 …”

“…Late adolescents are often classified as adults so any unique features in this group are unknown. It is very rare in early and middle adolescence with only nine cases in early adolescence and five cases in middle adolescence reported in the literature 1 . Of the nine reported cases in early adolescence, six had generalised eruptions, one was localised to the vulva, and four had mucosal involvement.…”

“…Bullous pemphigoid is an autoimmune blistering disorder which is rarely seen in adolescence. A recent review of bullous pemphigoid in middle adolescence identified only 14 published cases 1 …”

Ein ungewöhnlicher Fall von Lichen ruber planus mit vorausgegangenem bullösem Pemphigoid bei einem 20‐jährigen Patienten

“…Lebensjahr) betrifft . In sehr seltenen Fällen kann es auch Kinder und in extrem seltenen Fällen auch Jugendliche betreffen; insgesamt wurden bislang nur 9 Fälle publiziert, in denen das BP in einem Alter zwischen 10–13 Jahren erstmals auftrat .…”

Bullous pemphigoid: can it occur in pediatric age?

“…1 Its incidence and prevalence during childhood is unknown, being extremely rare with only nine reports of cases occurred during early adolescence. [2][3][4] Autoimmune bullous disorders are due to autoantibodies generated against target antigens; in this way one typical serologic characteristic of BP is the presence of circulating autoantibodies against BP180 (collagen XVII) and BP230. 5 BP180 is a 180 kDa transmembrane glycoprotein with a 16th non-collagenous domain, and BP230 is an intracellular constituent of the hemidesmosomal plaque which belongs to the spectraplakin family.…”