A Case of Herpetiform Pemphigus with Anti‐Desmoglein 3 IgG Autoantibodies

Abstract

Herpetiform pemphigus (HP) is a rare variant of pemphigus characterized by a unique clinical phenotype of erythematous or urticarial plaques and vesicles that present in a herpetiform arrangement. Most HP cases have circulating anti-desmoglein 1 (Dsg1) IgG autoantibodies, but some HP cases have anti-desmoglein 3 (Dsg3) IgG. A 92-year-old Japanese woman presented with severely pruritic annular erythema and vesicles in a herpetiform arrangement on the trunk. No oral mucosal lesions were present. Histopathologically, these vesicles showed eosinophilic spongiosis as well as suprabasilar acantholysis. Direct immunofluorescence showed in vivo IgG deposition on keratinocyte cell surfaces, and indirect immunofluorescence showed circulating IgG autoantibodies against keratinocyte cell surfaces at a titer of 1:30. Enzyme-linked immunosorbent assay using recombinant Dsg1 and Dsg3 revealed the presence of anti-Dsg3 IgG but no anti-Dsg1 IgG autoantibodies. The lack of oral mucosal involvement and the unique clinical features favored the diagnosis of HP. It remains to be clarified why the anti-Dsg3 IgG autoantibodies in this patient induced this unique features of HP, rather than the mucosal dominant type of pemphigus vulgaris.

Pemphigus herpetiformis: analysis of the autoantibody profile during the disease course with changes in the clinical phenotype

“…However, a number of reports have been published, reporting that the clinical phenotype of pemphigus was in apparent contrast with that expected based on the autoantibody profile, contradicting the Dsg compensation theory. 3,[11][12][13] How can the apparent discordance of our findings with the Dsg compensation theory be explained? The pathogenic potential of pemphigus antibodies may be affected by several factors, such as the quantity of IgG autoantibodies 14 and their subclass profile.…”

Histomorphology of healthy oral mucosa in untreated celiac patients: unexpected association with spongiosis

“…Spongiotic reaction has been found in several skin lesions (60–64) and in some oral diseases, such as oral melanoacanthosis (melanoacanthoma) (65), oral psoriasis (66), allergic contact stomatitis (67), plasma cell gingivitis, intra‐oral fixed drug eruption, leukoedema and white sponge nevus (68). In most of these oral lesions, the pathogenetic mechanism involved in the collection of the intraepithelial fluid is not clear and remains to be elucidated: spongiosis could be caused by extravasations of fluids from blood vessels located in the lamina propria or by the presence of an osmotic gradient developed towards the epithelium, drawing fluid into it subsequent to various immunological reactions (59).…”

Paraneoplastic Pemphigus Herpetiformis With IgG Antibodies to Desmoglein 3 and Without Mucosal Lesions

“…4,5 Direct immunofluorescence (IF) shows IgG antibodies on keratinocyte cell surfaces, and the target antigen has been shown to be desmoglein (Dsg) 1 in most cases, 3,6 with a few patients demonstrating autoantibodies to Dsg3. 1,3,[6][7][8] Anti-Dsg antibodies in PH are thought to induce spongiosis with eosinophil infiltration but rarely produce acantholysis, in contrast to classic pemphigus. Although the association of cancer with immunobullous diseases can be seen in patients with paraneoplastic pemphigus (PNP) and pemphigus vulgaris (PV), [9][10][11] it is rare in patients with PH.…”