Clinical Features, Diagnosis and Treatment of Different Types of Paediatric Pemphigus

Authors

  • An-Wei Chen Department of Dermatology, Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, China
  • Li Liu Department of Dermatology, Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, China
  • Huan Yang Department of Dermatology, Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, China
  • Xiao-Yan Luo Department of Dermatology, Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, China
  • Juan Xiang Department of Dermatology, Children’s Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, China

DOI:

https://doi.org/10.2340/actadv.v105.42776

Keywords:

Pemphigus vulgaris, Pemphigus foliaceus, Pemphigus erythematosus, Children, Treatment

Abstract

Pemphigus is a rare and life-threatening autoimmune disease that is easily ignored in children. This study aimed to attract attention to pemphigus in the paediatric population for better understanding and recognition of the clinical characteristics and management in children’s different subtypes of pemphigus. The case series involved a retrospective record review of 3 patients who were referred to a department of dermatology from January 2022 to January 2024. All patients experienced a delayed diagnosis, but were eventually definitively diagnosed with pemphigus vulgaris, pemphigus foliaceus, and pemphigus erythematosus, because they all have intraepidermal blisters and elevated serum antibodies against Dsg1/Dsg3. In all 3 cases, systemic corticosteroids were used in combination with or without steroid-sparing adjuvants. The patient with pemphigus vulgaris experienced a recurrence due to discontinuous treatment; the other 2 patients achieved mostly remission after treatment, with the length of follow-up ranging from 5 to 12 months. Pemphigus in children has its own clinical characteristics and similar literature concerning improved strategies for clinical assessment and treatments are reviewed and discussed.

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References

Mistry BD, Leis M, Lee DM, Levy R. Management of pediatric pemphigus vulgaris with rituximab: A case report and review of the literature. Pediatr Dermatol 2022; 39: 960-966.

https://doi.org/10.1111/pde.15095

Fuertes I, Guilabert A, Mascaró JM Jr, Iranzo P. Rituximab in childhood pemphigus vulgaris: a long-term follow-up case and review of the literature. Dermatology 2010; 221: 13-16.

https://doi.org/10.1159/000287254 DOI: https://doi.org/10.1159/000287254

Baratta A, Camarillo D, Papa C, Treat JR, Payne AS, Rozenber SS, et al. Pediatric pemphigus vulgaris: durable treatment responses achieved with prednisone and mycophenolate mofetil (MMF). Pediatr Dermatol 2013; 30: 240-244.

https://doi.org/10.1111/j.1525-1470.2012.01730.x DOI: https://doi.org/10.1111/j.1525-1470.2012.01730.x

Tallab, T.; Joharji, H.; Bahamdan, K.; Karkashan E, Mourad M, Ibrahim K. The incidence of pemphigus in the southern region of Saudi Arabia. Int. J. Dermatol 2001; 40: 570-572.

https://doi.org/10.1046/j.1365-4362.2001.01247.x DOI: https://doi.org/10.1046/j.1365-4362.2001.01247.x

Miyachi H, Konishi T, Hashimoto Y, Matsui H, Fushimi K, Inozume T, et al. Clinical course and outcomes of pemphigus vulgaris and foliaceus: A retrospective study using a nationwide database in Japan. J Dermatol 2023; 50: 212-221.

https://doi.org/10.1111/1346-8138.16641 DOI: https://doi.org/10.1111/1346-8138.16641

Chiu HY, Chang CJ, Lin YJ, Tsai TF. National trends in incidence, mortality, hospitalizations, and expenditures for pemphigus in Taiwan. J Dermatol Sci 2020; 99: 203-208.

https://doi.org/10.1016/j.jdermsci.2020.08.002 DOI: https://doi.org/10.1016/j.jdermsci.2020.08.002

V'lckova-Laskoska MT, Laskoski DS, Kamberova S, Caca-Biljanovska N, Volckova N. Epidemiology of pemphigus in Macedonia: a 15-year retrospective study (1990-2004). Int J Dermatol 2007; 46: 253-258.

https://doi.org/10.1111/j.1365-4632.2006.03010.x DOI: https://doi.org/10.1111/j.1365-4632.2006.03010.x

Shrivastava P, Mariam S, Abid L, Buch SA, Ahmad SA, Mansoori S, et al. Rituximab in Childhood and Juvenile Pemphigus Vulgaris: A Systematic Review. Cureus 2024; 16: e58288.

https://doi.org/10.7759/cureus.58288

Hübner F, König IR, Holtsche MM, Zillikens D, Linder R, Schmidt E. Prevalence and age distribution of pemphigus and pemphigoid diseases among paediatric patients in Germany. J Eur Acad Dermatol Venereol 2020; 34 :2600-2605.

https://doi.org/10.1111/jdv.16467 DOI: https://doi.org/10.1111/jdv.16467

Metry DW, Hebert AA, Jordon RE. Nonendemic pemphigus foliaceus in children. J Am Acad Dermatol 2002; 46: 419-422.

https://doi.org/10.1067/mjd.2002.119647 DOI: https://doi.org/10.1067/mjd.2002.119647

Hobbs LK, Noland MB, Raghavan SS, Gru AA. Pemphigus erythematosus: A case series from a tertiary academic center and literature review. J Cutan Pathol 2021; 48 :1038-1050.

https://doi.org/10.1111/cup.13992 DOI: https://doi.org/10.1111/cup.13992

Ruocco V, Ruocco E. Pemphigus and environmental factors. G Ital Dermatol Venereol 2003; 138: 299-309.

Asarch A, Gürcan HM, Ahmed AR. A current review of juvenile pemphigus vulgaris: analysis of data on clinical outcomes. Am J Clin Dermatol 2010; 11: 21-33.

https://doi.org/10.2165/11310380-000000000-00000 DOI: https://doi.org/10.2165/11310380-000000000-00000

Melchionda V, Harman KE. Pemphigus vulgaris and pemphigus foliaceus: an overview of the clinical presentation, investigations and management. Clin Exp Dermatol 2019; 44: 740-746.

https://doi.org/10.1111/ced.14041 DOI: https://doi.org/10.1111/ced.14041

Saha A, Dhar S. Pemphigus foliaceous presents as annular pustular lesion: a diagnostic confusion. Indian J Dermatol 2022; 67: 805-807.

https://doi.org/10.4103/ijd.ijd_247_22 DOI: https://doi.org/10.4103/ijd.ijd_247_22

Garza-Dávila VF, Santana-Gutiérrez A, Zapata-Salazar N, Vázquez-Martínez O, Ocampo-Candiani J, Fernández-de Luna ML, et al. Eye and lid involvement as an uncommon feature of pemphigus foliaceus in a pediatric patient. Pediatr Dermatol 2024; 41: 853-856.

https://doi.org/10.1111/pde.15594 DOI: https://doi.org/10.1111/pde.15594

Xie D, Bilgic-Temel A, Abu Alrub N, Murrell DF. Alopecia in autoimmune blistering diseases: a systematic review of pathogenesis and clinical features of disease. Skin Appendage Disord 2019; 5: 263-275.

https://doi.org/10.1159/000496836 DOI: https://doi.org/10.1159/000496836

Yu BT. Juvenile pemphigus foliaceus, exfoliative type--a case report. Proc Chin Acad Med Sci Peking Union Med Coll 1988; 3: 122-123.

Connelly EA, Aber C, Kleiner G, Nousari C, Charles C, Schachner LA. Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment. Pediatr Dermatol 2007; 24: 172-176.

https://doi.org/10.1111/j.1525-1470.2007.00369.x DOI: https://doi.org/10.1111/j.1525-1470.2007.00369.x

Sardana, K.; Garg, V.; Agarwal, P. Is there an emergent need to modify the desmoglein compensation theory in pemphigus on the basis of Dsg ELISA data and alternative pathogenic mechanisms? Br. J. Dermatol 2013; 168: 669-674.

https://doi.org/10.1111/bjd.12012 DOI: https://doi.org/10.1111/bjd.12012

Hans-Filho G, Aoki V, Bittner NRH, Bittner GC. Fogo selvagem: endemic pemphigus foliaceus. An Bras Dermatol 2018; 93: 638-650.

https://doi.org/10.1590/abd1806-4841.20188235 DOI: https://doi.org/10.1590/abd1806-4841.20188235

Laarman R, Horii KA. Concurrent Pemphigus Foliaceus and Graves' Disease. Pediatr Dermatol 2017; 34: e95-e96.

https://doi.org/10.1111/pde.13051 DOI: https://doi.org/10.1111/pde.13051

Moro F, Sinagra JLM, Salemme A, Fania L, Mariotti F, Pira A, et al. Pemphigus: trigger and predisposing factors. Front Med (Lausanne) 2023; 10: 1326359.

https://doi.org/10.3389/fmed.2023.1326359 DOI: https://doi.org/10.3389/fmed.2023.1326359

Zhao CY, Chiang YZ, Murrell DF. Neonatal autoimmune blistering disease: a systematic review. Pediatr Dermatol 2016; 33: 367-374.

https://doi.org/10.1111/pde.12859 DOI: https://doi.org/10.1111/pde.12859

Bjarnason B, Flosadóttir E. Childhood, neonatal, and stillborn pemphigus vulgaris. Int J Dermatol 1999; 38: 680-688.

https://doi.org/10.1046/j.1365-4362.1999.00764.x DOI: https://doi.org/10.1046/j.1365-4362.1999.00764.x

Anand A, Awake P, Bhosale A, Bindu R, Kher A, Bhanap P. Pemphigus vulgaris in a neonate born to a mother with pemphigus vulgaris: A case report. Clin Case Rep 2023; 11: e8343.

https://doi.org/10.1002/ccr3.8343 DOI: https://doi.org/10.1002/ccr3.8343

Malik AM, Tupchong S, Huang S, Are A, Hsu S, Motaparthi K. An Updated Review of Pemphigus Diseases. Medicina (Kaunas) 2021; 57: 1080.

https://doi.org/10.3390/medicina57101080 DOI: https://doi.org/10.3390/medicina57101080

Jamora, M.J.J.; Jiao, D.; Bystryn, J.-C. Antibodies to desmoglein 1 and 3, and the clinical phenotype of pemphigus vulgaris. J. Am. Acad. Dermatol 2003; 48: 976-977.

https://doi.org/10.1067/mjd.2003.438 DOI: https://doi.org/10.1067/mjd.2003.438

McMullan P, Gochnauer H, Brown-Joel Z, O'Donnell P, Belazarian L. Superficial erosions in a pediatric patient. Pediatr Dermatol 2024; 41: 924-926.

https://doi.org/10.1111/pde.15636 DOI: https://doi.org/10.1111/pde.15636

Murrell DF, Peña S, Joly P, Marinovic B, Hashimoto T, Diaz LA, et al. Diagnosis and management of pemphigus: recommendations of an international panel of experts. J Am Acad Dermatol 2020; 82: 575-585.e1.

https://doi.org/10.1016/j.jaad.2018.02.021 DOI: https://doi.org/10.1016/j.jaad.2018.02.021

Lins GT, Barbosa NLS, de Abreu EMV, da Costa KVT, Meneses KCB, Silva RN, et al. Childhood pemphigus vulgaris is a challenging diagnosis. Autops Case Rep 2021; 11: e2021267.

https://doi.org/10.4322/acr.2021.267 DOI: https://doi.org/10.4322/acr.2021.267

Carver C, Kalesinskas M, Dheden N, Ahmed AR. Treatment of Pediatric Pemphigus Foliaceus. Cureus 2023; 15: e45373.

https://doi.org/10.7759/cureus.45373 DOI: https://doi.org/10.7759/cureus.45373

Mahajan R, Handa S, Kumar S, Chatterji D, Saikia UN, De D. Pediatric autoimmune blistering disorders - a five-year demographic profile and therapy experience. Int J Dermatol 2022; 61: 1511-1518.

https://doi.org/10.1111/ijd.16314 DOI: https://doi.org/10.1111/ijd.16314

Shrivastava P, Mariam S, Abid L, Buch SA, Ahmad SA, Mansoori S, et al. Rituximab in childhood and juvenile pemphigus vulgaris: a systematic review. Cureus 2024; 16: e58288.

https://doi.org/10.7759/cureus.58288 DOI: https://doi.org/10.7759/cureus.58288

Kianfar N, Dasdar S, Mahmoudi H, Tavakolpour S, Balighi K, Daneshpazhooh M. Rituximab in childhood and juvenile autoimmune bullous diseases as first-line and second-line treatment: a case series of 13 patients. J Dermatolog Treat 2022; 33: 869-

https://doi.org/10.1080/09546634.2020.1788702 DOI: https://doi.org/10.1080/09546634.2020.1788702

Cheng DR, Barton R, Greenway A, Crawford NW. Rituximab and protection from vaccine preventable diseases: applying the evidence to pediatric patients. Expert Rev Vaccines 2016; 15: 1567-1574.

https://doi.org/10.1080/14760584.2016.1193438 DOI: https://doi.org/10.1080/14760584.2016.1193438

Mistry BD, Leis M, Lee DM, Levy R. Management of pediatric pemphigus vulgaris with rituximab: A case report and review of the literature. Pediatr Dermatol 2022; 39: 960-966.

https://doi.org/10.1111/pde.15095 DOI: https://doi.org/10.1111/pde.15095

Goyal T, Varshney A, Bakshi SK. Incidence of vesicobullous and erosive disorders of neonates: where and how much to worry? Indian J Pediatr 2021; 88: 574-578.

https://doi.org/10.1007/s12098-011-0592-9 DOI: https://doi.org/10.1007/s12098-011-0592-9

Santiago-Vázquez M, González-Molina VJ, Rodriguez-Ramos FM, Colón-Fontanez F, Carrasquillo OY. Successful treatment of recalcitrant pediatric pemphigus vulgaris with rituximab. Clin Pediatr (Phila) 2023; 62: 541-547.

https://doi.org/10.1177/00099228221140801 DOI: https://doi.org/10.1177/00099228221140801

Published

2025-03-09

How to Cite

Chen, A.-W., Liu, L., Yang, H., Luo, X.-Y., & Xiang, J. (2025). Clinical Features, Diagnosis and Treatment of Different Types of Paediatric Pemphigus. Acta Dermato-Venereologica, 105, adv42776. https://doi.org/10.2340/actadv.v105.42776

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