Approach Considerations

Dapsone

Dapsone is the treatment of choice. The response is slower than that seen with dermatitis herpetiformis, with resolution usually occurring in about 4 weeks. Once disease control has been established, the dose should be tapered to the lowest dose needed to maintain control. Sulfapyridine and sulfamethoxypyridazine may also be used, but only a few isolated reports support their effectiveness.

Acitretin

Acitretin (and formally etretinate) has been used to successfully treat subcorneal pustular dermatosis (SPD) and should be considered as an alternative or additional treatment for those who are intolerant of, or unresponsive to, dapsone. Once disease control has been established, the dose should be tapered to the lowest dose needed to maintain control. Isotretinoin at 0.5 mg/kg/d appears to be ineffective.

Phototherapy

Phototherapy with psoralen with UVA (PUVA),^[45]broadband UVB, and narrowband UVB alone or in combination with dapsone and/or retinoids can be successful at controlling subcorneal pustular dermatosis.^[46]Long-term maintenance regimens may be needed.^[47]

Additional therapies

Anecdotal case reports support the use of infliximab, tacalcitol,^[48]maxacalcitol,^[49]mizoribine,^[50]ketoconazole,^[51]tetracycline, minocycline, benzylpenicillin, vitamin E,^[52]azithromycin,^[53]cyclosporine,^{[54, 55]}colchicine, pentoxifylline,^[56]intravenous immunoglobulins,^[57]and adalimumab with mycophenolate mofetil.^[58]Antimyeloma treatment should be considered in cases of subcorneal pustular dermatosis associated with IgA monoclonal gammopathy of undetermined significance, refractory to other therapies.^[59]

Systemic and topical corticosteroids are generally ineffective but may provide some control. They have been used in combination with dapsone to treat associated conditions such as pyoderma gangrenosum and multiple myeloma. A good response to systemic corticosteroids is atypical and is suggestive of a diagnosis of pustular psoriasis.

Long-Term Monitoring

Long-term follow-up is recommended. Periodic evaluations with serum protein electrophoresis and direct immunofluorescence should be performed every few years.

Paraproteinemia, myeloma, intraepidermal IgA staining, and pustular psoriasis may develop several years after the initial presentation of subcorneal pustular dermatosis (SPD). Identifying these conditions, as well as other associated diagnoses, can improve the understanding of the etiology and pathogenesis of subcorneal pustular dermatosis, clarify its relationship with IgA pemphigus and pustular psoriasis, and help define its nosologic classification.

Sneddon IB, Wilkinson DS. Subcorneal pustular dermatosis. Br J Dermatol. 1956 Dec. 68(12):385-94. [QxMD MEDLINE Link].
Sneddon IB, Wilkinson DS. Subcorneal pustular dermatosis. Br J Dermatol. 1979 Jan. 100(1):61-8. [QxMD MEDLINE Link].
Bordignon M, Zattra E, Montesco MC, 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):51-5. [QxMD MEDLINE Link].
Hashimoto T, Kiyokawa C, Mori O, et al. Human desmocollin 1 (Dsc1) is an autoantigen for the subcorneal pustular dermatosis type of IgA pemphigus. J Invest Dermatol. 1997 Aug. 109(2):127-31. [QxMD MEDLINE Link].
Grob JJ, Mege JL, Capo C, et al. Role of tumor necrosis factor-alpha in Sneddon-Wilkinson subcorneal pustular dermatosis. A model of neutrophil priming in vivo. J Am Acad Dermatol. 1991 Nov. 25(5 Pt 2):944-7. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link]. [Full Text].
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. [QxMD MEDLINE Link].
Kasha EE Jr, Epinette WW. 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):854-8. [QxMD MEDLINE Link].
Wallach D, Cottenot F, Pelbois G, Cavelier B, Didierjean L, Saurat JH. Subcorneal pustular dermatosis and monoclonal IgA. Br J Dermatol. 1982 Aug. 107(2):229-34. [QxMD MEDLINE Link].
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):687-90. [QxMD MEDLINE Link].
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 1:111-4. [QxMD MEDLINE Link].
Butt A, Burge SM. Sneddon-Wilkinson disease in association with rheumatoid arthritis. Br J Dermatol. 1995 Feb. 132(2):313-5. [QxMD MEDLINE Link].
Lin RY, Schwartz RA, Lambert WC. Subcorneal pustular dermatosis with polyarthritis. Cutis. 1986 Feb. 37(2):123-4, 126. [QxMD MEDLINE Link].
Delaporte E, Colombel JF, Nguyen-Mailfer C, Piette F, Cortot A, Bergoend H. Subcorneal pustular dermatosis in a patient with Crohn's disease. Acta Derm Venereol. 1992 Aug. 72 (4):301-2. [QxMD MEDLINE Link].
García-Salces I, Baldellou R, Hörndler C, Zubiri ML. Subcorneal pustular dermatosis with pathergy phenomenon in a patient with Crohn's disease. J Eur Acad Dermatol Venereol. 2009 Mar. 23 (3):349-50. [QxMD MEDLINE Link].
Cohen PR. Neutrophilic dermatoses: a review of current treatment options. Am J Clin Dermatol. 2009. 10 (5):301-12. [QxMD MEDLINE Link].
Naik HB, Cowen EW. Autoinflammatory pustular neutrophilic diseases. Dermatol Clin. 2013 Jul. 31 (3):405-25. [QxMD MEDLINE Link].
Voigtlander C, Luftl M, Schuler G, Hertl M. Infliximab (anti-tumor necrosis factor alpha antibody): a novel, highly effective treatment of recalcitrant subcorneal pustular dermatosis (Sneddon-Wilkinson disease). Arch Dermatol. 2001 Dec. 137(12):1571-4. [QxMD MEDLINE Link].
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):662-5. [QxMD MEDLINE Link].
Sauder MB, Glassman SJ. Palmoplantar subcorneal pustular dermatosis following adalimumab therapy for rheumatoid arthritis. Int J Dermatol. 2013 May. 52 (5):624-8. [QxMD MEDLINE Link].
Papini M, Cicoletti M, Landucci P. Subcorneal pustular dermatosis and mycoplasma pneumoniae respiratory infection. Acta Derm Venereol. 2003. 83(5):387-8. [QxMD MEDLINE Link].
Scalvenzi M, Palmisano F, Annunziata MC, Mezza E, Cozzolino I, Costa C. Subcorneal pustular dermatosis in childhood: a case report and review of the literature. Case Rep Dermatol Med. 2013. 2013:424797. [QxMD MEDLINE Link]. [Full Text].
Johnson SA, Cripps DJ. Subcorneal pustular dermatosis in children. Arch Dermatol. 1974 Jan. 109 (1):73-7. [QxMD MEDLINE Link].
Koçak M, Birol A, Erkek E, Bozdoğan O, Atasoy P. Juvenile subcorneal pustular dermatosis: a case report. Pediatr Dermatol. 2003 Jan-Feb. 20 (1):57-9. [QxMD MEDLINE Link].
Watts PJ, Khachemoune A. Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress. Am J Clin Dermatol. 2016 Dec. 17 (6):653-671. [QxMD MEDLINE Link].
Scerri L, Zaki I, Allen BR. Pyoderma gangrenosum and subcorneal pustular dermatosis, without monoclonal gammopathy. Br J Dermatol. 1994 Mar. 130(3):398-9. [QxMD MEDLINE Link].
Iobst W, Ingraham K. Sneddon-Wilkinson disease in a patient with rheumatoid arthritis. Arthritis Rheum. 2005 Dec. 52(12):3771. [QxMD MEDLINE Link].
Saulsbury FT, Kesler RW. Subcorneal pustular dermatosis and systemic lupus erythematosus. Int J Dermatol. 1984 Jan-Feb. 23 (1):63-4. [QxMD MEDLINE Link].
Tsuruta D, Matsumura-Oura A, Ishii M. Subcorneal pustular dermatosis and Sjögren's syndrome. Int J Dermatol. 2005 Nov. 44(11):955-7. [QxMD MEDLINE Link].
Mokhtari F, Poostiyan N. Subcorneal Pustular Dermatosis: A Case Report of a Patient with Diffuse Scleroderma. Adv Biomed Res. 2018. 7:83. [QxMD MEDLINE Link]. [Full Text].
Wargo JJ, Adams M, Trevino J. Subcorneal pustular dermatosis and episcleritis associated with poorly controlled ulcerative colitis. BMJ Case Rep. 2017 Jan 30. 2017:[QxMD MEDLINE Link]. [Full Text].
Scarpa R, Lubrano E, Cozzi R, Ames PR, Oriente CB, Oriente P. Subcorneal pustular dermatosis (Sneddon-Wilkinson syndrome): another cutaneous manifestation of SAPHO syndrome?. Br J Rheumatol. 1997 May. 36 (5):602-3. [QxMD MEDLINE Link].
Park BS, Cho KH, Eun HC, Youn JI. Subcorneal pustular dermatosis in a patient with aplastic anemia. J Am Acad Dermatol. 1998 Aug. 39 (2 Pt 1):287-9. [QxMD MEDLINE Link].
Lombart F, Dhaille F, Lok C, Dadban A. Subcorneal pustular dermatosis associated with Mycoplasma pneumoniae infection. J Am Acad Dermatol. 2014 Sep. 71 (3):e85-6. [QxMD MEDLINE Link].
Papini M, Cicoletti M, Landucci P. Subcorneal pustular dermatosis and mycoplasma pneumoniae respiratory infection. Acta Derm Venereol. 2003. 83 (5):387-8. [QxMD MEDLINE Link].
Lombart F, Dhaille F, Lok C, Dadban A. Subcorneal pustular dermatosis associated with Mycoplasma pneumoniae infection. J Am Acad Dermatol. 2014 Sep. 71 (3):e85-6. [QxMD MEDLINE Link].
Iyengar S, Chambers CJ, Chang S, Fung MA, Sharon VR. Subcorneal pustular dermatosis associated with Coccidioides immitis. Dermatol Online J. 2015 Aug 15. 21 (8):[QxMD MEDLINE Link].
Taniguchi S, Tsuruta D, Kutsuna H, Hamada T. Subcorneal pustular dermatosis in a patient with hyperthyroidism. Dermatology. 1995. 190 (1):64-6. [QxMD MEDLINE Link].
Villey MC, Ehrsam E, Marrakchi S, Colombel JF, Thomas P. Apudoma and subcorneal pustular dermatosis (Sneddon-Wilkinson disease). Dermatology. 1992. 185 (4):269-71. [QxMD MEDLINE Link].
Agarwal A, Shivaswamy KN, Barani R et al. Subcorneal pustular dermatosis and thymoma:an association or a coincidence?. Indian J Dermatol. 2006. 51:272-4.
Tajiri K, Nakajima T, Kawai K, Minemura M, Sugiyama T. Sneddon-Wilkinson disease induced by sorafenib in a patient with advanced hepatocellular carcinoma. Intern Med. 2015. 54 (6):597-600. [QxMD MEDLINE Link].
Scalvenzi M, Palmisano F, Annunziata MC, Mezza E, Cozzolino I, Costa C. Subcorneal pustular dermatosis in childhood: a case report and review of the literature. Case Rep Dermatol Med. 2013. 2013:424797. [QxMD MEDLINE Link].
Sauder MB, Glassman SJ. Palmoplantar subcorneal pustular dermatosis following adalimumab therapy for rheumatoid arthritis. Int J Dermatol. 2013 May. 52(5):624-8. [QxMD MEDLINE Link].
Lotery HE, Eedy DJ, McCusker G. Subcorneal pustular dermatosis involving the face. J Eur Acad Dermatol Venereol. 1999 May. 12 (3):230-3. [QxMD MEDLINE Link].
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):203-5. [QxMD MEDLINE Link].
Orton DI, George SA. Subcorneal pustular dermatosis responsive to narrowband (TL-01) UVB phototherapy. Br J Dermatol. 1997 Jul. 137(1):149-50. [QxMD MEDLINE Link].
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):153-5. [QxMD MEDLINE Link].
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):669-72. [QxMD MEDLINE Link].
Hoshina D, Tsujiwaki M, Furuya K. Successful treatment of subcorneal pustular dermatosis with maxacalcitol. Clin Exp Dermatol. 2016 Jan. 41 (1):102-3. [QxMD MEDLINE Link].
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):1328-30. [QxMD MEDLINE Link].
Verma KK, Pasricha JS. Ketoconazole as a therapeutic modality in subcorneal pustular dermatosis. Acta Derm Venereol. 1997 Sep. 77(5):407-8. [QxMD MEDLINE Link].
Ayres S Jr, Mihan R. Letter: Subcorneal pustular dermatosis controlled by vitamin E. Arch Dermatol. 1974 Jun. 109(6):914. [QxMD MEDLINE Link].
Bliziotis I, Rafailidis P, Vergidis P, Falagas ME. Regression of subcorneal pustular dermatosis type of IgA pemphigus lesions with azithromycin. J Infect. 2005 Aug. 51(2):E31-4. [QxMD MEDLINE Link].
Zachariae CO, 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):386-7. [QxMD MEDLINE Link].
Karadogan SK, Aydogan K, Baskan EB, 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):536-7. [QxMD MEDLINE Link].
Falcone LM, Pilcher MF, Kovach RF, Powers R. Pentoxyfilline as a Treatment for Subcorneal Pustular Dermatosis. Dermatol Ther. 2019 Jan 14. e12818. [QxMD MEDLINE Link].
Kundak S, Bağ Ö, Gülez N, Ergin M. A child with subcorneal pustular dermatosis responded to IVIG treatment (Sneddon-Wilkinson disease). Reumatologia. 2017. 55 (6):323-327. [QxMD MEDLINE Link].
Howell SM, Bessinger GT, Altman CE, Belnap CM. 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):541-3. [QxMD MEDLINE Link].
von dem Borne PA, Jonkman MF, van Doorn R. Complete remission of skin lesions in a patient with subcorneal pustular dermatosis (Sneddon-Wilkinson disease) treated with antimyeloma therapy: association with disappearance of M-protein. Br J Dermatol. 2017 May. 176 (5):1341-1344. [QxMD MEDLINE Link].

Media Gallery

Circinate plaques and pustules on nonerythematous base in a patient with subcorneal pustular dermatosis.
Subcorneal pustular dermatosis with numerous pustules on erythematous base.
Subcorneal pustules with neutrophil accumulation and minimal spongiosis.

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Author

Lidija Kandolf Sekulovic, MD, PhD Professor, Head of the Department of Dermatology and Venereology, Medical Faculty, Military Medical Academy, Belgrade, Serbia

Lidija Kandolf Sekulovic, MD, PhD is a member of the following medical societies: American Society of Clinical Oncology, European Academy of Dermatology and Venereology, European Society for Medical Oncology, Serbian Association of Dermatorvenerologists

Disclosure: Nothing to disclose.

Coauthor(s)

Tanja Tirnanić, MD Dermatovenereologist, City Institute for Skin and Venereal Diseases, Serbia

Tanja Tirnanić, MD is a member of the following medical societies: International Society of Dermatology, Serbian Association of Dermatorvenerologists

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Takeji Nishikawa, MD Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc

Disclosure: Nothing to disclose.

Vlada Groysman, MD Medical Director, Cahaba Dermatology and Skin Health Center; Clinical Assistant Professor of Dermatology, University of Alabama at Birmingham School of Medicine

Vlada Groysman, MD is a member of the following medical societies: American Academy of Dermatology, Women's Dermatologic Society, Medical Dermatology Society

Disclosure: Nothing to disclose.

Naveed Sami, MD, FAAD Assistant Professor, Department of Dermatology, University of Alabama School of Medicine

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors John D Wilkinson, MD, MBBS, MRCS, FRCP; John Reed, MBBS, MRCP; Sarah E Dick, MD; and Abby S Van Voorhees, MD, to the development and writing of the source articles.