Sneddon — Wilkinson disease: Review of the modern literature

Cover Page

Cite item


The article provides a current overview of the correlation between a rare dermatosis — subcorneal pustulosis (Sneddon — Wilkinson disease) — and various diseases of internal organs; features of immunological changes; the role of desmocollin 1, chemoatractants, interleukin-1β, interleukin-6, interleukin-8, interleukin-10, leukotriene B4, tumour necrosis factor-alpha and K5a complement fraction in the pathogenesis of the disease. In this work, the authors describe the features of the clinical picture of the disease, as well as diagnostic criteria, including the use of serum protein electrophoresis. Histological features of the disease are discussed in detail. In addition, modern methods for treating this disease, including the use of dapsone, acitretin, infliximab, PUVA therapy are presented.

About the authors

M. B. Drozhdina

Kirov State Medical University, Ministry of Health of the Russian Federation

Author for correspondence.
ORCID iD: 0000-0002-7689-8350
Cand. Sci. (Med.), Associate Professor, Department of Dermatovenereology and Cosmetology Russian Federation

S. V. Koshkin

Kirov State Medical University, Ministry of Health of the Russian Federation

ORCID iD: 0000-0002-6220-8304
Dr. Sci. (Med.), Prof., Departmental Head, Department of Dermatovenereology and Cosmetology Russian Federation


  1. Sneddon I. B., Wilkinson D. S. Subcorneal pustular dermatosis. Br J Dermatol. 1956 Dec.;68(12):385–394. doi: 10.1111/j.1365-4362.1977. tb01874.x
  2. Sneddon I. B., Wilkinson D. S. Subcorneal pustular dermatosis. Br J Dermatol. 1979Jan.;100(1):61–68. doi: 10.1111/j.1365-2133.1979. tb03570.x
  3. Bordignon M., Zattra E., Montesco M. C., Alaibac M. Subcorneal pustular dermatosis (Sneddon — Wilkinson disease) with absence of desmoglein 1 and 3 antibodies: case report and literature review. Am J Clin Dermatol. 2008;9(1):51–55. doi: 10.2165/00128071-200809010-00006
  4. Ono S., Otsuka A., Miyachi Y., Kabashima K. Subcorneal Pustular Dermatosis Exhibiting a High Serum TARC/CCL17 Level. Case Rep Dermatol. 2013 Jan.;5(1):38–42. doi: 10.1159/000348241
  5. Hashimoto T., Teye K., Ishii N. Clinical and immunological studies of 49 cases of various types of intercellular IgA dermatosis and 13 cases of classical subcorneal pustular dermatosis examined at Kurume University. Br J Dermatol. 2016 Jun 3. doi: 10.1111/bjd.14780
  6. Kasha E. E. Jr., Epinette W. W. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) in association with a monoclonal IgA gammopathy: a report and review of the literature. J Am Acad Dermatol. 1988 Nov.;19(5 Pt 1):854–858.
  7. Cohen P. R. Neutrophilic dermatoses: a review of current treatment options. Am J Clin Dermatol. 2009;10(5):301–312. doi: 10.2165/11310730-000000000-00000
  8. Naik H. B., Cowen E. W. Autoinflammatory pustular neutrophilic diseases. Dermatol Clin. 2013 Jul.;31(3):405–425. DOI: 10.1016/j. det.2013.04.001
  9. Puechguiral-Renaud I., Carpentier O., Piette F., Delaporte E. Subcorneal pustulosis and Pyoderma gangrenosum associated with a biclonal gammopathy. Eur J Dermatol. 2006 Nov-Dec.;16(6):687–690. doi: 10.2165/00128071-200809010-00006
  10. Takata M., Inaoki M., Shodo M., Hirone T., Kaya H. Subcorneal pustular dermatosis associated with IgA myeloma and intraepidermal IgA deposits. Dermatology. 1994:189(Suppl 1):111–114. doi: 10.1159/000246947
  11. Butt A., Burge S. M. Sneddon — Wilkinson disease in association with rheumatoid arthritis. Br J Dermatol. 1995 Feb.;132(2):313–315.
  12. García-Salces I., Baldellou R., Hörndler C., Zubiri M. L. Subcorneal pustular dermatosis with pathergy phenomenon in a patient with Crohn’s disease. J Eur Acad Dermatol Venereol. 2009 Mar.;23(3):349–350. doi: 10.1111/j.1468-3083.2008.02866.x
  13. Voigtlander C., Luftl M., Schuler G., Hertl M. Infliximab (antitumor necrosis factor alpha antibody): a novel, highly effective treatment of recalcitrant subcorneal pustular dermatosis (Sneddon — Wilkinson disease). Arch Dermatol. 2001 Dec.;137(12):1571–1574. doi: 10.1001/archderm.137.12.1571
  14. Bonifati C., Trento E., Cordiali Fei P., Muscardin L., Amantea A., Carducci M. Early but not lasting improvement of recalcitrant subcorneal pustular dermatosis (Sneddon — Wilkinson disease) after infliximab therapy: relationships with variations in cytokine levels in suction blister fluids. Clin Exp Dermatol. 2005 Nov.;30(6):662–665. doi: 10.1111/j.1365-2230.2005.01902.x
  15. Scarpa R., Lubrano E., Cozzi R., Ames P. R., Oriente C. B., Oriente P. Subcorneal pustular dermatosis (Sneddon — Wilkinson syndrome): another cutaneous manifestation of SAPHO syndrome? Br J Rheumatol. 1997 May;36(5):602–603. doi: 10.1093/rheumatology/36.5.602
  16. Park B. S., Cho K. H., Eun H. C., Youn J. I. Subcorneal pustular dermatosis in a patient with aplastic anemia. J Am Acad Dermatol. 1998 Aug.;39(2 Pt 1):287–289. doi: 10.1016/S0190-9622(98)70093-3
  17. Lombart F., Dhaille F., Lok C., Dadban A. Subcorneal pustular dermatosis associated with Mycoplasma pneumoniae infection. J Am Acad Dermatol. 2014 Sep.;71(3):85–86. doi: 10.1016/S0190-9622(98)70093-3
  18. Iyengar S., Chambers C. J., Chang S., Fung M. A., Sharon V. R. Subcorneal pustular dermatosis associated with Coccidioides immitis. Dermatol Online J. 2015 Aug 15;21(8).
  19. Taniguchi S., Tsuruta D., Kutsuna H., Hamada T. Subcorneal pustular dermatosis in a patient with hyperthyroidism. Dermatology. 1995;190(1):64–66. doi: 10.1159/000246638
  20. Agarwal A., Shivaswamy K. N., Barani R. et al. Subcorneal pustular dermatosis and thymoma: an association or a coincidence? Indian J Dermatol. 2006;51:272–274.
  21. Bauwens M., De Coninck A., Roseeuw D. Subcorneal pustular dermatosis treated with PUVA therapy. A case report and review of the literature. Dermatology. 1999;198(2):203–205.
  22. Marliere V., Beylot-Barry M., Beylot C., Doutre M. Successful treatment of subcorneal pustular dermatosis (Sneddon — Wilkinson disease) by acitretin: report of a case. Dermatology. 1999;199(2):153–155. doi: 10.1159/000018224
  23. Kawaguchi M., Mitsuhashi Y., Kondo S. A case of subcorneal pustular dermatosis treated with tacalcitol (1alpha, 24-dihydroxyvitamin D3). J Dermatol. 2000 Oct.;27(10):669–672.
  24. Hoshina D., Tsujiwaki M., Furuya K. Successful treatment of subcorneal pustular dermatosis with maxacalcitol. Clin Exp Dermatol. 2016 Jan.;41(1):102–103. doi: 10.1111/ced.12680
  25. Kono T., Terashima T., Oura H., Ishii M., Taniguchi S., Muramatsu T. Recalcitrant subcorneal pustular dermatosis and bullous pemphigoid treated with mizoribine, an immunosuppressive, purine biosynthesis inhibitor. Br J Dermatol. 2000 Dec.;143(6):1328–1330. doi: 10.1046/j.1365-2133.2000.03917.x
  26. Verma K. K., Pasricha J. S. Ketoconazole as a therapeutic modality in subcorneal pustular dermatosis. Acta Derm Venereol. 1997 Sep.;77(5):407–408.
  27. Ayres S. Jr., Mihan R. Letter: Subcorneal pustular dermatosis controlled by vitamin E. Arch Dermatol. 1974 Jun.;109(6):914. DOI: 10.1001/ archderm.109.6.914b
  28. Bliziotis I., Rafailidis P., Vergidis P., Falagas M. E. Regression of subcorneal pustular dermatosis type of IgA pemphigus lesions with azithromycin. J Infect. 2005 Aug.;51(2):E31–E34. doi: 10.1016/j.jinf.2004.08.009
  29. Zachariae C. O., Rossen K., Weismann K. An unusual severe case of subcorneal pustular dermatosis treated with cyclosporine and prednisolone. Acta Derm Venereol. 2000 Sep-Oct.;80(5):386–387.
  30. Karadogan S. K., Aydogan K., Baskan E. B., Tunali S. A case of subcorneal pustular dermatosis treated successfully with a combination of cyclosporin and prednisolone. J Eur Acad Dermatol Venereol. 2007 Apr.;21(4):536–537.
  31. Howell S. M., Bessinger G. T., Altman C. E., Belnap C. M. Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil. J Am Acad Dermatol. 2005 Sep.;53(3):541–543. doi: 10.1016/j.jaad.2005.02.051

Copyright (c) 2019 Drozhdina M.B., Koshkin S.V.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
Регистрационный номер и дата принятия решения о регистрации СМИ: серия ПИ № ФС 77 - 60448 от 30.12.2014.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies