Pemphigus vulgaris – ein Fallbericht

Zusammenfassung

Pemphigus vulgaris (PV) ist eine der seltenen autoimmunbedingten Dermatosen. Therapeutisches Mittel der Wahl sind Immunsuppressiva, die je nach Krankheitsbild als Monotherapie oder in kombinierter Therapie angewendet werden. Dieser Bericht gibt eine aktuelle Literaturübersicht und beschreibt die Pemphigus-vulgaris-Erkrankung eines 9 Jahre alten, männlich-kastrierten, mittelgroßen Mischlingsrüden, welcher charakteristische Haut- und Schleimhautläsionen in Form von mukosalen Vesikeln und Ulzerationen der Maulschleimhaut sowie multiplen kutanen Ulzerationen periokulär, an Planum nasale, am Schwanzansatz und an den Pfoten aufwies. Bedingt durch die klinische Symptomatik und das Ergebnis der weiterführenden und pathohistologischen Untersuchungen wurde eine gesicherte Diagnose gestellt. Mittels immunsuppressiver Langzeittherapie (Prednisolon, Azathioprin) konnte eine erfolgreiche Behandlung des Patienten bis zum heutigen Zeitpunkt gewährleistet werden.

Abstract

Pemphigus vulgaris (PV) represents a rare autoimmune dermatosis. The therapeutic agents of choice are immunosuppressants, which are used in form of a monotherapy or as a combination treatment depending on the clinical picture. This report provides a current literature review and describes a case of pemphigus vulgaris in a 9-year-old, male, castrated, medium-sized mixed-breed dog. The dog presented with characteristic cutaneous and mucosal lesions showing oral mucosal vesicles and ulcerations as well as ulcerations in the periocular region as well as on the planum nasale, tail base, and the paws. Based on the clinical signs and the results of the further and histological examinations, the diagnosis was confirmed. Using long-term immunosuppressive therapy (Prednisolone, Azathioprine) the dog attained complete remission up to the present time.

Schlüsselwörter

Kleintiermedizin - Autoimmunerkrankung - Dermatose - Dermatologie

Keywords

Small animal medicine - autoimmune disease - dermatosis - dermatology

Publikationsverlauf

Eingereicht: 02. August 2024

Angenommen: 30. Juni 2025

Artikel online veröffentlicht:
15. Oktober 2025

• Literatur

• 1 Werner LL, Brown KA, Halliwell REW. Diagnosis of autoimmune skin disease in the dog: Correlation between histopathologic, direct immunofluorescent and clinical findings. Vet Immunol Immunopathol 1983; 5: 47-64
• 2 Stannard AA, Gribble DH, Baker BB. A mucocutaneous disease in the dog, resembling pemphigus vulgaris in man. J Am Vet Med Assoc 1975; 166: 575-582 PMID:1120727
• 3 Huvritz AI, Feldmann E. A disease in dogs resembling human pemphigus vulgaris: case reports. J Am Vet Med Assoc 1975; 166: 585-590
• 4 Joly P, Litrowski N. Pemphigus Group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol 2011; 29: 432-436
• 5 Kridin K, Zelber-Sagi S, Khamaisi M. et al. Remarkable differences in the epidemiology of pemphigus among two ethnic populations in the same geographic region. J Am Acad Dermatol 2016; 75: 925-930
• 6 Scott DW, Walton DK, Slater MR. et al. Immune-mediated dermatoses in domestic animals – 10 years after. Comp Cont Educ Pract 1987; 9: 424-551 CABI Record Number:19882206965
• 7 Sieben C, Massone AR, Machuca MA. Dermatosis autoinmunes en caninos. Estudio Retrospectivo. Revista Veterinaria 2019; 30: 70-75
• 8 Tham HL, Linder KE, Olivry T. Deep Pemphigus (pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus) in dogs, cats and horses: A comprehensive review. BMC Vet Res 2020; 16
• 9 Rybníček J, Hill PB. Suspected polymyxin b-induced pemphigus vulgaris in a dog. Vet Dermatol 2007; 18: 165-170
• 10 Olivry T. Drug-Associated Canine pemphigus vulgaris? who knows!. Vet. Dermatol 2007; 18: 378-379
• 11 Olivry T. A review of autoimmune skin diseases in domestic animals: I – superficial pemphigus. Vet Dermatol 2006; 17: 291-305
• 12 Olivry T, Linder KE. Dermatoses affecting desmosomes in animals: A mechanistic review of acantholytic blistering skin diseases. Vet Dermatol 2009; 20: 313-326
• 13 Noli C, Scarampella F, Thoma S (eds.). Immunmediierte Erkrankungen. In: Praktische Dermatologie bei Hund und Katze - Klinik, Diagnose, Therapie. Hannover, Niedersachsen: Schlütersche; 201AD 325-343
• 14 Day M, Day M. Immune-mediated skin disease. Clinical Immunology of the Dog and Cat, Second Edition 2011; 122-171
• 15 Medleau L, Hnilica KA. Autoimmune and immune-mediated skin disorders. Small Animal Dermatology 2006; 189-227
• 16 Nishifuji K, Amagai M, Ota T. et al. Cloning of canine desmoglein 3 and immunoreactivity of serum antibodies in human and canine pemphigus vulgaris with its extracellular domains. J Dermatol Sci 2003; 32: 181-191
• 17 Nishifuji K, Olivry T, Ishii K. et al. IGG autoantibodies directed against desmoglein 3 cause dissociation of keratinocytes in canine pemphigus vulgaris and paraneoplastic pemphigus. Vet Immunol Immunopathol 2007; 117: 209-221
• 18 Kasperkiewicz M, Ellebrecht CT, Takahashi H. et al. Pemphigus. Nat Rev Dis Primers 2017; 3
• 19 Bizikova P, Dean GA, Hashimoto T. et al. Cloning and establishment of canine desmocollin-1 as a major autoantigen in canine pemphigus foliaceus. Vet Immunol Immunopathol 2012; 149: 197-207
• 20 Olivry T, Joubeh S, Dunston SM. et al. Desmoglein-3 is a target autoantigen in spontaneous canine pemphigus vulgaris. Exp Dermatol 2003; 12: 198-20
• 21 Williamson L, Raess NA, Caldelari R. et al. Pemphigus vulgaris identifies plakoglobin as key suppressor of c-myc in the skin. EMBO J 2006; 25: 3298-3309
• 22 Rosenkrantz WS. Pemphigus: Current therapy. Vet Dermatol 2004; 15: 90-98
• 23 Dip R, Carmichael J, Letellier I. et al. Concurrent short-term use of prednisolone with cyclosporine a accelerates pruritus reduction and improvement in clinical scoring in dogs with atopic dermatitis. BMC Vet Res 2013; 9: 173
• 24 Ringheim GE, Wampole M, Oberoi K. Bruton’s tyrosine kinase (BTK) inhibitors and autoimmune diseases: Making sense of BTK inhibitor specificity profiles and recent clinical trial successes and failures. Front Immunol 2021; 12
• 25 McDonald C, Xanthopoulos C, Kostareli E. The role of Bruton’s tyrosine kinase in the immune system and disease. Immunology 2021; 164: 722-736
• 26 Murrell DF, Patsatsi A, Stavropoulos P. et al. Proof of concept for the clinical effects of oral rilzabrutinib, the first Bruton tyrosine kinase inhibitor for pemphigus vulgaris: The phase II believe study. Br J Dermatol 2021; 185: 745-755
• 27 Langrish CL, Bradshaw JM, Francesco MR. et al. Preclinical efficacy and anti-inflammatory mechanisms of action of the Bruton tyrosine kinase inhibitor rilzabrutinib for immune-mediated disease. J Immunol 2021; 206: 1454-1468
• 28 Kuter DJ, Boccia RV, Lee E-J. et al. Phase I/II, open-label, Adaptive Study of Oral Bruton tyrosine kinase inhibitor PRN1008 in patients with relapsed/refractory primary or secondary immune thrombocytopenia. Blood 2019; 134: 87-87
• 29 Patsatsi A, Murrell DF. Bruton tyrosine kinase inhibition and its role as an emerging treatment in pemphigus. Front Med (Lausanne) 2021; 8
• 30 Goodale EC, Varjonen KE, Outerbridge CA. et al. Efficacy of a Bruton’s tyrosine kinase inhibitor (PRN-473) in the treatment of canine pemphigus foliaceus. Vet Dermatol Veterinary Dermatology 2020; 31: 291
• 31 Goodale EC, White SD, Bizikova P. et al. Open trial of Bruton’s tyrosine kinase inhibitor (PRN1008) in the treatment of canine pemphigus foliaceus. Vet Dermatol 2020; 31: 410
• 32 Huda S, Chau B, Chen C. et al. Caspase inhibition as a possible therapeutic strategy for pemphigus vulgaris: A systematic review of current evidence. Biology (Basel) 2022; 11: 314
• 33 Hutchison DM, Hosking A-M, Hong EM. et al. Mitochondrial autoantibodies and the role of apoptosis in pemphigus vulgaris. Antibodies (Basel) 2022; 11: 55
• 34 Whitley NT, Day MJ. Immunomodulatory drugs and their application to the management of canine immune-mediated disease. J Small Anim Pract 2011; 52: 70-85
• 35 Viviano KR. Update on immununosuppressive therapies for dogs and cats. Vet Clin North Am Small Anim Pract 2013; 43: 1149-1170
• 36 Atopica. Fachinformation in Form der Zusammenfassung der Merkmale des Tierarzneimittels Atopica^® 50mg, Ciclosporin, Weichkapseln für Hunde 2008
• 37 Archer TM, Boothe DM, Langston VC. et al. Oral cyclosporine treatment in dogs: A review of the literature. J Vet Intern Med 2013; 28: 1-20
• 38 Olszewska M, Kolacinska-Strasz Z, Sulej J. et al. Efficacy and safety of cyclophosphamide, azathioprine, and cyclosporine (ciclosporin) as adjuvant drugs in pemphigus vulgaris. Am J Clin Dermatol 2007; 8: 85-92
• 39 Diagnostik und Therapie des pemphigus vulgaris. Available from https://register.awmf.org/assets/guidelines/013-071l_S2k_Diagnostik-Therapie-Pemphigus-vulgaris-foliaceus-bulloese-Pemphigoids_2019-10-abgelaufen.pdf
• 40 Fellman CL, Archer TM, Wills RW. et al. Effects of cyclosporine and dexamethasone on canine T cell expression of interleukin-2 and Interferon-gamma. Vet Immunol. Immunopathol. 2019; 216
• 41 Archer TM, Fellman CL, Stokes JV. et al. Pharmacodynamic monitoring of caninet-cell cytokine responses to oral cyclosporine. J Vet Intern Med 2011; 25: 1391-1397
• 42 Olivry T, Rivierre C, Murphy KM. Efficacy of cyclosporine for treatment induction of canine pemphigus foliaceus. Vet Rec 2003; 152: 53-54
• 43 Is ciclosporin an effective treatment for canine pemphigus foliaceus?. Vet Rec 2017; 181: 566-568
• 44 Eberhardy AC, Heinrich NA, McFadden RA. et al. Prevalence of hepatotoxicity and myelosuppression with alternate day use of azathioprine and glucocorticoids for treatment of dermatological conditions in dogs. Vet Dermatol 2022; 33: 503-508
• 45 Rodriguez DB, Mackin A, Easley R. et al. Relationship between red blood cell thiopurine methyltransferase activity and myelotoxicity in dogs receiving azathioprine. J Vet Intern Med 2004; 18: 339
• 46 Wallisch K, Trepanier LA. Incidence, timing, and risk factors of azathioprine hepatotoxicosis in dogs. J Vet Intern Med 2015; 29: 513-518
• 47 Antibiotic Stewardship. RKI Available from https://www.rki.de/DE/Content/Infekt/Antibiotikaresistenz/Antibiotic_Stewardship.html
• 48 Marks SL, Kook PH, Papich MG. et al. ACVIM consensus statement: Support for rational administration of gastrointestinal protectants to dogs and cats. J Vet Intern Med 2018; 32: 1823-1840