Subcorneal pustular dermatosis
- Arianne Shadi Kourosh, MD
Arianne Shadi Kourosh, MD
- Director of Community Health
- Department of Dermatology, Massachusetts General Hospital and Harvard Medical School
Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a rare neutrophilic dermatosis in which recurrent crops of sterile pustules appear in the most superficial (subcorneal) layers of the skin (picture 1A-B). The pustules are usually distributed in annular or serpiginous patterns and are most commonly located on flexural surfaces and on intertriginous skin. SPD is characterized by its clinical presentation, as its etiology is unknown and its nosology remains controversial.
In the overwhelming majority of cases, SPD is a chronic and benign condition for which the primary concerns are minimizing morbidity, improving quality of life, and ruling out the presence of an associated internal disease. Oral dapsone is the treatment of choice for SPD.
The clinical features, diagnosis, and management of SPD will be reviewed here. Other neutrophilic dermatoses and pustular disorders are reviewed separately. (See "Neutrophilic dermatoses" and "Approach to the patient with pustular skin lesions".)
Since Sneddon and Wilkinson first described SPD in the 1950s, the majority of cases have been reported in women, and generally in women over the age of 40 [1,2]. SPD is considered a rare condition; however, no estimate of global incidence or prevalence is available.
Although most of the reported cases have occurred in Caucasians, there is no known ethnic or geographic predilection. SPD is observed worldwide [3,4]. The relatively few cases reported in children tend to be atypical, and some have subsequently been reclassified as forms of psoriasis .To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- SNEDDON IB, WILKINSON DS. Subcorneal pustular dermatosis. Br J Dermatol 1956; 68:385.
- 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 2017; 176:168.
- Leppard B. An atlas of African dermatology, Radcliffe Medical Press, Abingdon, UK 2002.
- ISE S, OFUJI S. SUBCORNEAL PUSTULAR DERMATOSIS; A FOLLICULAR VARIANT? Arch Dermatol 1965; 92:169.
- Scalvenzi M, Palmisano F, Annunziata MC, et al. Subcorneal pustular dermatosis in childhood: a case report and review of the literature. Case Rep Dermatol Med 2013; 2013:424797.
- 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; 25:944.
- Cheng S, Edmonds E, Ben-Gashir M, Yu RC. Subcorneal pustular dermatosis: 50 years on. Clin Exp Dermatol 2008; 33:229.
- Bonifati C, Trento E, Cordiali Fei P, et al. 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; 30:662.
- Reed J, Wilkinson J. Subcorneal pustular dermatosis. Clin Dermatol 2000; 18:301.
- 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; 9:51.
- Robinson ND, Hashimoto T, Amagai M, Chan LS. The new pemphigus variants. J Am Acad Dermatol 1999; 40:649.
- Ishii N, Ishida-Yamamoto A, Hashimoto T. Immunolocalization of target autoantigens in IgA pemphigus. Clin Exp Dermatol 2004; 29:62.
- 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; 109:127.
- Düker I, Schaller J, Rose C, et al. Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3. Arch Dermatol 2009; 145:1159.
- Stone MS, Lyckholm LJ. Pyoderma gangrenosum and subcorneal pustular dermatosis: clues to underlying immunoglobulin A myeloma. Am J Med 1996; 100:663.
- Kohl PK, Hartschuh W, Tilgen W, Frosch PJ. Pyoderma gangrenosum followed by subcorneal pustular dermatosis in a patient with IgA paraproteinemia. J Am Acad Dermatol 1991; 24:325.
- Miyakawa K, Miyamoto R, Baba S, et al. Vesiculopustular dermatosis with ulcerative colitis. Concomitant occurrence of circulating IgA anti-intercellular and anti-basement membrane zone antibodies. Eur J Dermatol 1995; 5:122.
- Delaporte E, Colombel JF, Nguyen-Mailfer C, et al. Subcorneal pustular dermatosis in a patient with Crohn's disease. Acta Derm Venereol 1992; 72:301.
- Dallot A, Decazes JM, Drouault Y, et al. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) with amicrobial lymph node suppuration and aseptic spleen abscesses. Br J Dermatol 1988; 119:803.
- Iobst W, Ingraham K. Sneddon-Wilkinson disease in a patient with rheumatoid arthritis. Arthritis Rheum 2005; 52:3771.
- Butt A, Burge SM. Sneddon-Wilkinson disease in association with rheumatoid arthritis. Br J Dermatol 1995; 132:313.
- Lin RY, Schwartz RA, Lambert WC. Subcorneal pustular dermatosis with polyarthritis. Cutis 1986; 37:123.
- Lautenschlager S, Itin PH, Hirsbrunner P, Büchner SA. Subcorneal pustular dermatosis at the injection site of recombinant human granulocyte-macrophage colony-stimulating factor in a patient with IgA myeloma. J Am Acad Dermatol 1994; 30:787.
- Takata M, Inaoki M, Shodo M, et al. Subcorneal pustular dermatosis associated with IgA myeloma and intraepidermal IgA deposits. Dermatology 1994; 189 Suppl 1:111.
- Vaccaro M, Cannavò SP, Guarneri B. Subcorneal pustular dermatosis and IgA lambda myeloma: a uncommon association but probably not coincidental. Eur J Dermatol 1999; 9:644.
- 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; 19:854.
- Ratnarathorn M, Newman J. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) occurring in association with nodal marginal zone lymphoma: a case report. Dermatol Online J 2008; 14:6.
- Guggisberg D, Hohl D. Intraepidermal IgA pustulosis preceding a CD30+ anaplastic large T-cell lymphoma. Dermatology 1995; 191:352.
- Yamamoto T, Katayama I, Nishioka K. A variant of subcorneal pustular dermatosis with monoclonal IgG gammopathy of undetermined significance. Eur J Dermatol 1995; 5:640.
- Agarwal A, Shivaswamy KN, Barani R, et al. Subcorneal pustular dermatosis and thymoma: an association or a coincidence? Indian J Dermatol 2006; 51:272.
- Woof JM, Russell MW. Structure and function relationships in IgA. Mucosal Immunol 2011; 4:590.
- O'Connell M, Goulden V. Images in clinical medicine. "Half-half" blisters. N Engl J Med 2012; 366:e31.
- Naretto C, Baldovino S, Rossi E, et al. The case of SLE associated Sneddon-Wilkinson pustular disease successfully and safely treated with infliximab. Lupus 2009; 18:856.
- Tsuruta D, Matsumura-Oura A, Ishii M. Subcorneal pustular dermatosis and Sjögren's syndrome. Int J Dermatol 2005; 44:955.
- Taniguchi S, Tsuruta D, Kutsuna H, Hamada T. Subcorneal pustular dermatosis in a patient with hyperthyroidism. Dermatology 1995; 190:64.
- Pujol RM, Wang CY, el-Azhary RA, et al. Necrolytic migratory erythema: clinicopathologic study of 13 cases. Int J Dermatol 2004; 43:12.
- Roujeau JC, Bioulac-Sage P, Bourseau C, et al. Acute generalized exanthematous pustulosis. Analysis of 63 cases. Arch Dermatol 1991; 127:1333.
- Chao SC, Lee JY. Brittle nails and dyspareunia as first clues to recurrences of malignant glucagonoma. Br J Dermatol 2002; 146:1071.
- Nakashima H, Komine M, Sasaki K, et al. Necrolytic migratory erythema without glucagonoma in a patient with short bowel syndrome. J Dermatol 2006; 33:557.
- 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 2016.
- Firkin FC, Mariani AF. Agranulocytosis due to dapsone. Med J Aust 1977; 2:247.
- Foucauld J, Uphouse W, Berenberg J. Dapsone and aplastic anemia. Ann Intern Med 1985; 102:139.
- Björkman A, Phillips-Howard PA. Adverse reactions to sulfa drugs: implications for malaria chemotherapy. Bull World Health Organ 1991; 69:297.
- Richardus JH, Smith TC. Increased incidence in leprosy of hypersensitivity reactions to dapsone after introduction of multidrug therapy. Lepr Rev 1989; 60:267.
- Todd DJ, Bingham EA, Walsh M, Burrows D. Subcorneal pustular dermatosis and IgA paraproteinaemia: response to both etretinate and PUVA. Br J Dermatol 1991; 125:387.
- Cameron H, Dawe RS. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) treated with narrowband (TL-01) UVB phototherapy. Br J Dermatol 1997; 137:150.
- Orton DI, George SA. Subcorneal pustular dermatosis responsive to narrowband (TL-01) UVB phototherapy. Br J Dermatol 1997; 137:149.
- Park YK, Park HY, Bang DS, Cho CK. Subcorneal pustular dermatosis treated with phototherapy. Int J Dermatol 1986; 25:124.
- Bauwens M, De Coninck A, Roseeuw D. Subcorneal pustular dermatosis treated with PUVA therapy. A case report and review of the literature. Dermatology 1999; 198:203.
- Laifaoui JA, Guillen E, Worret WI, Ring J. A case of subcorneal pustular dermatosis (Sneddon-Wilkinson disease) not responding to dapsone: Therapeutic alternatives. Acta Dermatoven APA 2003; 12:109.
- Kawaguchi M, Mitsuhashi Y, Kondo S. A case of subcorneal pustular dermatosis treated with tacalcitol (1alpha,24-dihydroxyvitamin D3). J Dermatol 2000; 27:669.
- Handler MZ, Hamilton H, Aires D. Treatment of peristomal pyoderma gangrenosum with topical crushed dapsone. J Drugs Dermatol 2011; 10:1059.
- Zachariae CO, Rossen K, Weismann K. An unusual severe case of subcorneal pustular dermatosis treated with cyclosporine and prednisolone. Acta Derm Venereol 2000; 80:386.
- Karadoğan SK, Aydoğan K, Başkan EB, Tunali S. A case of subcorneal pustular dermatosis treated successfully with a combination of cyclosporin and prednisolone. J Eur Acad Dermatol Venereol 2007; 21:536.
- Bedi MK. Successful treatment of long-standing, recalcitrant subcorneal pustular dermatosis with etanercept. Skinmed 2007; 6:245.
- Marlière 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; 199:153.
- Voigtländer C, Lüftl 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; 137:1571.
- Berk DR, Hurt MA, Mann C, Sheinbein D. Sneddon-Wilkinson disease treated with etanercept: report of two cases. Clin Exp Dermatol 2009; 34:347.
- Sauder MB, Glassman SJ. Palmoplantar subcorneal pustular dermatosis following adalimumab therapy for rheumatoid arthritis. Int J Dermatol 2013; 52:624.
- 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; 53:541.
- CUTANEOUS MANIFESTATIONS
- ASSOCIATED DISORDERS
- Physical examination
- Skin biopsy
- EVALUATION FOR ASSOCIATED DISORDERS
- DIFFERENTIAL DIAGNOSIS
- First-line therapy
- - Oral dapsone
- Second-line therapies
- - Phototherapy
- - Systemic glucocorticoids
- Other therapies
- - Topical agents
- - Systemic agents
- SUMMARY AND RECOMMENDATIONS