Downregulation of aquaporin 3 in bullous pemphigoid patients

  • Mona Mostafa Korany
  • Marwa Ahmed Amer
  • Laila Ahmed Rashed
  • Marwah Adly Saleh
Original Paper


Bullous pemphigoid (BP) is a chronic autoimmune skin disease. Aquaporin 3 (AQP 3) has a possible role in the pathogenesis of many dermatological diseases. In this work, we aimed to evaluate the expression of AQP 3 in BP. Perilesional skin biopsies were taken from 24 BP patients and 13 controls. The biopsies were stained by direct immunofluorescence using rabbit anti-human AQP 3 FITC antibody. The expression of AQP 3 was weak in 5 patients (20.8%), moderate in 18 patients (75%), strong in 1 patient (4.2%) in the suprabasal layers. It was negative in 4 patients (16.7%), weak in 18 patients (75%), moderate in 2 patients (8.3%) and no strong fluorescence was seen in the basal layers. In the controls, the expression was strong in ten controls (76.9%), moderate in three controls (23.1%) and no controls showed weak fluorescence in the suprabasal layer. The basal layer showed strong fluorescence in 11 controls (84.6%), moderate in 2 controls (15.4%) and no controls showed mild or no fluorescence. There was a statistically significant difference in the expression of AQP 3 between basal and suprabasal layers of BP patients but not of the controls. There was statistically significant difference in the expression of AQP 3 between patients and controls in both the basal (P value < 0.001) and the suprabasal layers (P value < 0.001). In conclusion, AQP 3 was downregulated in BP patients especially in the basal cell layer. This suggests that AQP 3 plays a role in the pathogenesis of BP.


Aquaporin 3 Basal Bullous pemphigoid Epidermis Immunofluorescence Suprabasal 




Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

Informed consent was obtained from all individual participants included in the study.


  1. 1.
    Bakry OA, Hagag MM, Kandil MA, Shehata WA (2016) Aquaporin 3 and E-cadherin expression in perilesional vitiligo skin. J Clin Diagn Res 10(12):WC01–WC06PubMedPubMedCentralGoogle Scholar
  2. 2.
    Boury-Jamot M, Sougrat R, Tailhardat M, Le Varlet B, Bonte F, Dumas M et al (2006) Expression and function of aquaporins in human skin: is aquaporin-3 just a glycerol transporter? Biochim Biophys Acta 1758(8):1034–1042CrossRefPubMedGoogle Scholar
  3. 3.
    Calonje E, Brenn A, Lazar A, McKee P (2012) McKee’s pathology of the skin. In: Schmitt W (ed) Acantholytic disorders, Chap 5. Saunders Elsevier, Amsterdam, pp 151–179. Google Scholar
  4. 4.
    Csorba K, Sesarman A, Oswald E, Feldrihan V, Fritsch A, Hashimoto T et al (2010) Cross-reactivity of autoantibodies from patients with epidermolysis bullosa acquisita with murine collagen VII. Cell Mol Life Sci 67(8):1343–1351CrossRefPubMedGoogle Scholar
  5. 5.
    Di Zenzo G, Della Torre R, Zambruno G, Borradori L (2012) Bullous pemphigoid: from the clinic to the bench. Clin Dermatol 30(1):3–16CrossRefPubMedGoogle Scholar
  6. 6.
    Furukawa F, Takigawa M, Matsuyoshi N, Shirahama S, Wakita H, Fujita M et al (1994) Cadherins in cutaneous biology. J Dermatol 21(11):802–813CrossRefPubMedGoogle Scholar
  7. 7.
    Hara-Chikuma M, Verkman AS (2008) Roles of aquaporin-3 in the epidermis. J Investig Dermatol 128(9):2145–2151CrossRefPubMedGoogle Scholar
  8. 8.
    Joly P, Baricault S, Sparsa A, Bernard P, Bedane C, Duvert-Lehembre S et al (2012) Incidence and mortality of bullous pemphigoid in France. J Investig Dermatol 132(8):1998–2004CrossRefPubMedGoogle Scholar
  9. 9.
    Kim NH, Lee AY (2010) Reduced aquaporin3 expression and survival of keratinocytes in the depigmented epidermis of vitiligo. J Investig Dermatol 130(9):2231–2239CrossRefPubMedGoogle Scholar
  10. 10.
    Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J (2008) Bullous pemphigoid and pemphigus vulgaris-incidence and mortality in the UK: population based cohort study. BMJ 337:a180CrossRefPubMedGoogle Scholar
  11. 11.
    Murrell DF, Daniel BS, Joly P, Borradori L, Amagai M, Hashimoto T et al (2012) Definitions and outcome measures for bullous pemphigoid: recommendations by an international panel of experts. J Am Acad Dermatol 66(3):479–485CrossRefPubMedGoogle Scholar
  12. 12.
    Mysorekar VV, Sumathy TK, Shyam Prasad AL (2015) Role of direct immunofluorescence in dermatological disorders. Indian Dermatol Online J 6(3):172–180CrossRefPubMedPubMedCentralGoogle Scholar
  13. 13.
    Nousari HC, Anhalt GJ (1999) Pemphigus and bullous pemphigoid. Lancet 354(9179):667–672CrossRefPubMedGoogle Scholar
  14. 14.
    Nguyen VT, Arredondo J, Chernyavsky AI, Kitajima Y, Pittelkow M, Grando SA (2004) Pemphigus vulgaris IgG and methylprednisolone exhibit reciprocal effects on keratinocytes. J Biol Chem 16(3):2135–2146 279(CrossRefGoogle Scholar
  15. 15.
    Olsson M, Broberg A, Jernas M, Carlsson L, Rudemo M, Suurkula M et al (2006) Increased expression of aquaporin 3 in atopic eczema. Allergy 61(9):1132–1137CrossRefPubMedGoogle Scholar
  16. 16.
    Puviani M, Marconi A, Cozzani E, Pincelli C (2003) Fas ligand in pemphigus sera induces keratinocyte apoptosis through the activation of caspase-8. J Investig Dermatol 120(1):164–167CrossRefPubMedGoogle Scholar
  17. 17.
    Santarelli A, Di Fede O, Carinci F, Rubini C, Ganzetti G, Perrone D et al (2011) Reciprocal altered expression of E-cadherin and P-cadherin in mucous membrane pemphigoid. Int J Immunopathol Pharmacol 4(2 Suppl):103–106CrossRefGoogle Scholar
  18. 18.
    Schmidt E, Goebeler M, Hertl M, Sardy M, Sitaru C, Eming R et al (2015) S2k guideline for the diagnosis of pemphigus vulgaris/foliaceus and bullous pemphigoid. J Dtsch Dermatol Ges 13(7):713–727PubMedGoogle Scholar
  19. 19.
    Voss KE, Bollag RJ, Fussell N, By C, Sheehan DJ, Bollag WB (2011) Abnormal aquaporin-3 protein expression in hyperproliferative skin disorders. Arch Dermatol Res 303(8):591–600CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Mona Mostafa Korany
    • 1
  • Marwa Ahmed Amer
    • 1
  • Laila Ahmed Rashed
    • 2
  • Marwah Adly Saleh
    • 1
  1. 1.Department of DermatologyFaculty of Medicine Cairo UniversityCairoEgypt
  2. 2.Department Biochemistry and Molecular BiologyFaculty of Medicine Cairo UniversityCairoEgypt

Personalised recommendations