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At the patient's first presentation, chemiluminescent enzyme immunoassay was positive for anti-BP180 NC16A antibodies (265.3 U/mL; normal value < 9 U/mL). GLO:DF2/01oct19:ced13962-fig-0002.jpg PHOTO (COLOR): The time courses of the treatments, the skin conditions and the anti-bullous pemphigoid (BP)180 NC16A antibody titres. In the present case, BP that had been stable on a low dose of steroid showed exacerbation of exanthemas with a rapid rise in the level of anti-BP180 NC16A autoantibodies 1 month after administration of DPP4i. [Extracted from the article]

The article presents a case study of a 69-year-old Japanese man with a history of psoriasis vulgaris. He was treated with ustekinumab, a therapeutic agent for psoriasis that biologically blocks p40 protein of interleukin-12. It is inferred that the treatment has caused both bullous and psoriatic lesions to completely disappear. The Antilamininγ1 pemphigoid autoimmune bullous disease is also explored.

Dear Editor, Mucous membrane pemphigoid (MMP) is a heterogeneous group of subepidermal autoimmune bullous skin disease, which affects mainly various mucous membranes and, occasionally, skin.1-3 Although the oral mucosa is most commonly affected, ocular, nasal, pharyngeal, laryngeal, oesophageal and genital mucosae are also involved.2 Anti-laminin 332 (LM332)-type MMP is characterized by IgG autoantibodies to various subunits of LM332. Considering that autoantibodies against LM332 react with the dermal side of split skin, the high sensitivity of ssIIF further confirms that ssIIF has diagnostic value in anti-LM332-type MMP. In summary, patients with anti-LM332-type MMP develop mucosal lesions on various sites, most frequently on the oral mucosae, and cutaneous lesions develop more frequently in males. [Extracted from the article]

Summary: We report a unique case of a Japanese woman with herpetiform pemphigus (HP) who had IgG autoantibodies reactive with nondesmosomal sites of keratinocytes and presented characteristic transmission electron microscopic (TEM) findings of various‐sized vacuoles in keratinocytes without acantholysis. The patient presented with pruritic annular oedematous erythemas with small blisters lining the margins on the trunk and extremities. Histopathological examinations showed intraepidermal blisters with prominent infiltrations of eosinophils. Direct and indirect immunofluorescence tests revealed the presence of in vivo bound and circulating IgG autoantibodies to the keratinocyte cell surfaces. However, enzyme‐linked immunosorbent assays for desmoglein (Dsg) 1, Dsg3 and desmocollins 1–3 showed negative results. Immunoblotting using the full‐length human Dsg1 recombinant protein showed a positive band. TEM examination showed various‐sized vacuoles squashing the nuclei in many keratinocytes, resulting in rupture of the cells. Immunoelectron microscopic examination revealed IgG deposition over the entire keratinocyte cell surfaces, which spared the desmosomes. IgG antibodies were also present on the inside walls of the vacuoles around the nuclei of keratinocytes and on the cell surfaces of infiltrating eosinophils. This patient also had marked eosinophilia and high levels of thymus and activation‐regulated chemokine and interleukin‐5 in the serum. These results indicated a novel autoantigen on the nondesmosomal keratinocyte cell surfaces and the pathogenesis of bullous spongiotic change with inflammation in HP. What's already known about this topic? Herpetiform pemphigus (HP) is a rare variant of pemphigus characterized clinically by pruritic annular erythemas with vesicles resembling dermatitis herpetiformis and histopathologically by eosinophilic spongiosis without apparent acantholysis.The targets of IgG autoantibodies in patients with HP are usually desmoglein 1 and 3, which are both components of desmosomes. What does this study add? This study reports the unique case of a patient with HP with IgG autoantibodies reactive to nondesmosomal sites of keratinocytes and characteristic transmission electron microscopic findings of various‐sized vacuoles in keratinocytes, while desmosomal adhesion remained.Regarding pathogenesis, we speculate that the ballooning vacuoles might result in the bullous spongiotic changes without acantholysis seen in this patient.This study also focuses on the relationships between blood eosinophil counts, serum thymus and activation‐regulated chemokine levels and HP disease activity. Linked Comment: Yuan and Pan. Br J Dermatol 2019; 180:22. [ABSTRACT FROM AUTHOR]

Background Despite the established pathogenic role of anti-desmoglein (Dsg) antibodies in classical pemphigus, the significance of autoantibodies to another desmosomal cadherin, desmocollin (Dsc) is at present unknown. No consistent immunoassay for immunoglobulin (Ig) G autoantibodies to Dscs has been developed. Objectives The aim of this study was to develop reliable assays to detect anti-Dsc autoantibodies. Methods We expressed soluble recombinant proteins (RPs) of human Dsc1-3 in mammalian cells and examined sera of various types of pemphigus, including 79 paraneoplastic pemphigus (PNP) sera, by novel enzyme-linked immunosorbent assays (ELISAs) using the RPs. We also performed ELISAs of Dsc baculoproteins and used the complementary DNA (cDNA) transfection method, and compared the results with those of mammalian ELISAs. Results Through mammalian ELISAs, IgG autoantibodies to Dsc1, Dsc2 and Dsc3 were detected in 16·5%, 36·7% and 59·5% of PNP sera, respectively, and considerable numbers of pemphigus herpetiformis (PH) and pemphigus vegetans (PVeg) sera reacted strongly with Dsc1 and Dsc3. Mammalian ELISAs were highly specific and more sensitive than baculoprotein ELISAs or the cDNA transfection method. Several Dsc-positive sera, particularly PH sera, showed no reactivity with Dsgs. The reactivity of PNP serum and PVeg serum with Dscs was not abolished by pre-absorption with Dsg RPs. Conclusions The results of these novel ELISAs indicated that IgG anti-Dsc autoantibodies were frequently detected and potentially pathogenic in nonclassical pemphigus. [ABSTRACT FROM AUTHOR]

The article presents the case of a 64-year-old Japanese man with antilaminin γ1 pemphigoid, an autoimmune sub-epidermal bullous disease, with no past history of psoriasis. The association of pruritic reddish-brown papules and plaques as initial lesions in a case of antilaminin γ1 pemphigoid without psoriasis is not conceived as coincidental. The patient was initially diagnosed with prurigo chronica multiformis, and was prescribed prednisolone.