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Biopsy showed eosinophilic pustules and spongiosis without acantholysis, with perivascular eosinophil and lymphocyte infiltration. Direct and indirect immunofluorescence and immunoblotting supported paraneoplastic pemphigus features despite clinical and histologic consistency with pemphigus herpetiformis; desmoglein 3 was positive while BP180 and other ELISAs were negative. 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Indirect immunofluorescence was positive for the patient’s IgG on rat transitional epithelium and the epidermal side of 1M NaCl split skin, and desmoglein 3 measured 928 U/mL; BP180, Dsg1, and ELISA for BP230 were negative.",{"name":119,"@type":110,"acceptedAnswer":120},"What treatment was started and how did the patient respond?",{"text":121,"@type":113},"Because the patient had pneumothorax, treatment began with low-dose prednisolone (10 mg/day) plus diaminodiphenyl sulfone (50 mg/day). Skin and oral manifestations resolved within 3 weeks, and prednisolone was tapered.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},459514,1790756106,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":19,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":14,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":129,"read_time":24,"preview_page_count":4},962084931830,"https://ap-avatar.wpscdn.com/davatar_a8503ba1806abce46bf441b54a3ca4cd","1/2  \nRESEARCH LETTER  \nParaneoplastic Pemphigus Mimicking Pemphigus Herpetiformis – Overlap of Two Diseases  \nSakura MAEDA1, Masahiro TSUTSUMI1, Kwesi TEYE2, Norito ISHII1 and Hiroshi KOGA1*  \n1Department of Dermatology, Kurume University School of Medicine, Fukuoka, Japan, and 2Kurume University Institute of Cutaneous Cell  \nBiology, Fukuoka, Japan. *Email: [hiroshi_koga@med.kurume-u.ac.jp](hiroshi_koga@med.kurume-u.ac.jp)  \n[Submitted Oct 16](Submitted Oct 16), 2025. Accepted after revision Oct 30, 2025  \nPublished Dec 22, 2025. DOI: 10.2340/actadv.v105.adv-2025-0106 Acta Derm Venereol 2025; 105: adv-2025-0106 .  \nTo the Editor,  \nA 53-year-old woman was referred to our department due to blisters on both lower limbs. She had been diagnosed with diffuse large B-cell lymphoma and bronchiolitis obliterans 2 years earlier. A month before the initial visit to our department, the patient developed pneumothorax. Her medical history included uterine myoma, Hashimoto’s disease, bilateral retinal detachment, bronchial asthma and hypertension. Physical examination revealed erythema annulare with blisters on both thighs and erosions on the soft palate, buccal mucosa and lips (Fig. 1a) . Thepemphigus disease area index was 9. A biopsy of  \nFig. 1. Clinical, histological and immunological findings in this case. (A) Erythema annulare with blisters on the right thigh.(B) Eosinophilic pustulosis and eosinophil infiltration in the dermis (HE, x200) (scale bar: 100 μm) . (C) Deposition of patient’s IgG on the cell surface of keratinocytes in direct immunofluorescence. The patient’s IgG reaction to the rat bladder epithelium (D) and the epidermal side of 1M split skin (E) in indirect immunofluorescence. Immunoblotting using epidermal extracts showing 210 kDa, 190 kDa and 130 kDa bands (F), and that using the recombinant protein (RP) of the BP180 NC16A domain showing the expected band as the RP (G) for the patient’s IgG. Arrowheads indicate bands positive for the patient’s IgG. Abbreviations: PV, pemphigus vulgaris; BP, bullous pemphigoid.  \nthe thigh revealed eosinophilic pustules and spongiosis without acantholysis in the epidermis. Eosinophils and lymphocytes infiltrated the perivascular region of the upper dermis (Fig. 1b) . Blood tests revealed eosinophilia (1183/μL) . Direct immunofluorescence showed deposits of the patient’s IgG and C3 on the cell surface of the epidermis (Fig. 1c) . The patient’s IgG reacted with the transitional epithelium of the rat bladder and on the epidermal side of the 1M NaCl split skin in indirect immunofluorescence (Fig. 1d ande) . The chemiluminescent enzyme immunoassay fordesmoglein (Dsg) 3 was 928 U/mL, whereas those for Dsg1 and BP180 were negative. Enzyme-linked immunosorbent assay (ELISA) for BP230 was negative. ELISAs for desmocollin 1–3 established previously (1) were negative. Immunoblotting using human epidermal extracts revealed the patient’s IgG reaction to a 210 kDa envoplakin, 190 kDa periplakin and 130 kDa Dsg3 . Immunoblotting using BP180 NC16A recombinant protein was positive for the patient’s IgG (Fig. 1fand g) . Although the clinical and histological findings were consistent with pemphigus herpetiformis (PH), the final diagnosis of paraneoplastic pemphigus (PNP) was made according to the criteria proposed by the European Academy of Dermatology and Venereology (EADV) guidelines (2) . Because the patient had a pneumothorax, the treatment was initiated with a low dose of prednisolone (10 mg/day) and diaminodiphenyl sulfone 50 mg/day. The skin and oral manifestations  \nresolved in 3 weeks, and prednisolone was tapered.  \nPH is a form of pemphigus reported by Jablonska et [al. in](al. in) 1975 and characterized by intraepidermal blistering and eosinophilic sea surface conditions with a clinical and histologic picture resembling dermatitis herpetiformis (3) . To our knowledge, 10 cases of PH with neoplasms including haematologic tumours have been reported (4) . Upon our detailed review of the 10 ca","cbCaifYIGjNYfirT","https://ap.wps.com/l/cbCaifYIGjNYfirT","pdf",1004688,"English","# Clinical presentation and history\n# Histology and immunological findings\n# Diagnostic criteria and differential diagnosis\n# Treatment and outcome\n# Literature cases and comparison","[{\"question\":\"Why was paraneoplastic pemphigus diagnosed instead of pemphigus herpetiformis?\",\"answer\":\"Although clinical and histological findings resembled pemphigus herpetiformis, the final diagnosis of paraneoplastic pemphigus was made using the EADV guideline criteria and supported by the patient’s immunological test results.\"},{\"question\":\"What were the key immunological and laboratory findings?\",\"answer\":\"Direct immunofluorescence showed IgG and C3 deposits on the epidermal cell surface. Indirect immunofluorescence was positive for the patient’s IgG on rat transitional epithelium and the epidermal side of 1M NaCl split skin, and desmoglein 3 measured 928 U/mL; BP180, Dsg1, and ELISA for BP230 were negative.\"},{\"question\":\"What treatment was started and how did the patient respond?\",\"answer\":\"Because the patient had pneumothorax, treatment began with low-dose prednisolone (10 mg/day) plus diaminodiphenyl sulfone (50 mg/day). Skin and oral manifestations resolved within 3 weeks, and prednisolone was tapered.\"}]","Paraneoplastic Pemphigus Mimicking Pemphigus Herpetiformis - Overlap of Two Diseases | PDF"]