Intercellular IgA dermatosis of childhood. Selective deposition of monomer IgA1 in the intercellular space of the epidermis

Subcorneal pustular dermatosis type of IgA pemphigus: demonstration of autoantibodies to desmocollin-1 and clinical review

“…Since the first report by Varigos 1 in 1979, a total of 49 cases 1–42 (20 males and 29 females, including the present case) characterized by vesiculobullous or vesiculopustular lesions with intercellular IgA deposition has been reported. Various terms have been used for this condition, such as intraepidermal neutrophilic IgA dermatosis, 6 intercellular IgA dermatosis, 10 IgA pemphigus foliaceus, 13 IgA herpetiform pemphigus, 18 intraepidermal IgA pustulosis 44 and intercellular IgA vesiculopustular dermatosis, 45 of which the most simple term, IgA pemphigus, is used in this report.…”

Generalized Annular Pustular Lesions in a Child

“…The antigen in IEN type is unknown, but autoantibodies to desmoglein-1 or desmoglein-3 have been reported [1][2][3][4]7].…”

“…The IEN type shows a characteristic clinical feature, the so‐called “sunflower‐like” configuration—a circinate configuration with central crusting and peripheral vesiculation [3]. Pediatric cases of IGAP also include a report of an unusual pemphigus vegetans type [3] and cases of extensive oropharyngeal mucosal involvement [4, 5].…”

“…The antigen associated with the SCPD type is desmocollin‐1. The antigen in IEN type is unknown, but autoantibodies to desmoglein‐1 or desmoglein‐3 have been reported [1–4, 7].…”

“…The age of all the reported cases was less than 11 years, with female predominance. Around 33.3% of pediatric cases had mucosal involvement [1, 3, 4].…”