eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses

Subcorneal Pustular Dermatosis

Author: Vlada Groysman, MD, Staff Physician, Department of Dermatology, University of Alabama School of Medicine
Coauthor(s): Naveed Sami, MD, FAAD, Assistant Professor Department of Dermatology, University of Alabama School of Medicine
Contributor Information and Disclosures

Updated: Jul 10, 2009

Introduction

Background

Subcorneal pustular dermatosis was first described by Sneddon and Wilkinson in 1956.1,2 It is a rare, benign, chronic relapsing sterile pustular eruption typically involving the flexural sites of the trunk and proximal extremities. It most commonly affects woman aged 40 years or older. The etiology of this entity is unknown, and its exact nosologic classification is still controversial. Studies also suggest that some cases of subcorneal pustular dermatosis represent a variant of pustular psoriasis. These cases have been reported to evolve clinically from initially presenting as subcorneal pustular dermatosis to lesions that are more typical of pustular or plaque psoriasis. 

Direct and indirect immunofluorescence results are commonly negative in the subcorneal pustular dermatosis Sneddon-Wilkinson subtype. These findings suggest that subcorneal pustular dermatosis Sneddon-Wilkinson subtype may not be an autoantibody-mediated disease. A 2008 report also tested patients for autoantibodies to desmogleins 1 and 3. These autoantibodies are considered pathogenic in pemphigus. Patients with subcorneal pustular dermatosis Sneddon Wilkinson subtype tested negative to the autoantibodies for desmogleins 1 and 3, further leading to a conclusion of alternate  pathogenic mechanisms.3

However, other studies that have identified another subgroup of subcorneal pustular dermatosis. This has been classified as the immunoglobulin A (IgA) pemphigus subtype. These patients have been shown to have IgA deposition against desmocollin 1 on immunofluorescence study results. Some experts have considered this subgroup to be a rare variant of pemphigus rather than subcorneal pustular dermatosis.4

Pathophysiology

The exact pathophysiology is unknown. The accumulation of neutrophils in the subcorneal layer suggests the presence of chemoattractants in the uppermost epidermis. Interleukin (IL)-1 beta, IL-6, IL-8, IL-10, leukotriene B4, and complement fragments C5a and C5a are neutrophil chemoattractants that have been found at increased levels in scale extracts of patients with subcorneal pustular dermatosis compared with that of controls.
 
Tumor necrosis factor-alpha levels have been found to be significantly elevated in the serum and blister fluid of patients with subcorneal pustular dermatosis.5 Successful treatment of recalcitrant subcorneal pustular dermatosis with an antitumor necrosis factor-alpha antibody has been reported.6 However, this observation was not confirmed in a recent report in which infliximab failed to achieve long-lasting control of the disease.7 The stimulus for these chemoattractants is unknown. No etiologic pathogen has been identified.

Immunofluorescence studies are negative in the subcorneal pustular dermatosis Sneddon Wilkinson subtype. However, a rare subtype of subcorneal pustular dermatosis has been reported to have a positive immunofluorescence with IgA deposition restricted to the upper epidermis and directed against desmocollin. As noted above, the clinical and histopathological characteristics have led experts to classify this variant as the subcorneal pustular dermatosis IgA pemphigus subtype. 

Additionally, despite many attempts, no infectious agent or other immunogenic trigger has yet been identified in subcorneal pustular dermatosis patients. The eruption is regarded as sterile, although it may sometimes become secondarily infected with Staphylococcus aureus or streptococcal species. Preceding Mycoplasma pneumoniae infection was implicated in one report, but this case had an acute presentation that responded to 3 months of dapsone without relapse.8

Frequency

International

Subcorneal pustular dermatosis is a rare condition. No estimate of prevalence or incidence is available. Cases are reported worldwide; no particular geographical predominance is apparent.

Mortality/Morbidity

Subcorneal pustular dermatosis is benign but chronic. The association of subcorneal pustular dermatosis with paraproteinemia or lymphoproliferative disorders, especially multiple myeloma, may alter the prognosis.

Race

No racial predisposition is reported.

Sex

Subcorneal pustular dermatosis affects middle-aged or elderly women more commonly than men.

Age

Subcorneal pustular dermatosis is most common in individuals aged 40 years or older. It has been reported in children, but these cases tend to have atypical features more suggestive of psoriasis. Several reported cases of subcorneal pustular dermatosis in children have evolved into psoriasis.

Clinical

History

  • Patients typically present with a history of a relapsing pustular eruption involving the flexural areas of the trunk and proximal extremities. Individual pustular lesions arise within a few hours. Pruritus and irritation can occur but are not usually prominent symptoms. Systemic and toxic symptoms are not associated with acute episodes. Patients typically do not have any symptoms or signs of mucosal involvement.
  • Patients may present with histories notable for monoclonal gammopathies (IgA more often than immunoglobulin G),9,10 lymphoproliferative disorders (especially multiple myeloma),11 and pyoderma gangrenosum.12,13 These conditions are well-recognized associations with subcorneal pustular dermatosis (developing both before and after the diagnosis of subcorneal pustular dermatosis). Other anecdotally associated conditions include inflammatory bowel disease, rheumatoid arthritis,14,15,16 systemic lupus erythematosus, hyperthyroidism and hypothyroidism, APUDoma (amine precursor uptake and decarboxylation cell–derived tumor), polycythemia rubra vera, Sjögren syndrome,17 thymoma,18 and SAPHO (synovitis, acne, pustulosis, osteitis)syndrome.
  • Patients should be queried about a personal and family history of psoriasis because differentiating subcorneal pustular dermatosis from pustular psoriasis can be difficult. Similarly, patients should be questioned about recent drug exposure because acute generalized exanthematous pustulosis is also in the differential diagnosis.

Physical

  • The primary lesions are flaccid pustules, measuring several millimeters in diameter, on normal or mildly erythematous skin.
  • The classic lesion has been described as a "half-half" blister, in which purulent fluid accumulates in the lower half of the blister.
  • The pustules can be isolated or grouped. They tend to coalesce and form annular, circinate, or serpiginous patterns. The pustules are superficial and rupture easily, resulting in a superficial crust.
  • Mild hyperpigmentation often remains after pustular lesions have resolved.
  • The most commonly affected areas include the axillae, groin, neck, and the submammary regions. The proximal flexural aspects of the extremities are sometimes affected.
  • Palmar, plantar, face and mucous membrane involvement is unusual.

Causes

  • The etiology of subcorneal pustular dermatosis is unknown. Subcorneal pustular dermatosis is a sterile eruption. Because multiple subtypes have been recognized, subcorneal pustular dermatosis has more than one etiology.
  • Some cases of subcorneal pustular dermatosis have been considered a variant of pustular psoriasis. Note that clinical and histological differentiation of subcorneal pustular dermatosis from pustular psoriasis can be difficult, although spongiform changes on histology favor the latter. Furthermore, a significant number of cases initially diagnosed as subcorneal pustular dermatosis are later diagnosed as psoriasis.
  • Other cases of subcorneal pustular dermatosis are argued to be a rare variant of pemphigus, known as subcorneal pustular dermatosis type IgA pemphigus. This subgroup of patients shows positive immunofluorescence with epidermal intercellular IgA deposits. The positive immunofluorescence can develop years after the initial diagnosis of subcorneal pustular dermatosis. Unlike pemphigus, a predominance of neutrophils and an absence or moderate acantholysis is observed; additionally, the condition is usually responsive to dapsone.

More on Subcorneal Pustular Dermatosis

Overview: Subcorneal Pustular Dermatosis
Differential Diagnoses & Workup: Subcorneal Pustular Dermatosis
Treatment & Medication: Subcorneal Pustular Dermatosis
Follow-up: Subcorneal Pustular Dermatosis
References
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References

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Further Reading

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Keywords

subcorneal pustular dermatosis, Sneddon-Wilkinson disease, subcorneal pustulosis of Sneddon and Wilkinson, paraproteinemia, immunoglobulin A monoclonal gammopathies, immunoglobulin G monoclonal gammopathies, lymphoproliferative disorders, multiple myeloma, pustular psoriasis, subcorneal pustular dermatosis type IgA pemphigus

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Contributor Information and Disclosures

Author

Vlada Groysman, MD, Staff Physician, Department of Dermatology, University of Alabama School of Medicine
Vlada Groysman, MD is a member of the following medical societies: American Academy of Dermatology, Medical Dermatology Society, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Coauthor(s)

Naveed Sami, MD, FAAD, Assistant Professor Department of Dermatology, University of Alabama School of Medicine
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey P Callen, MD, Professor of Medicine, 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, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Genetech Honoraria Consulting; Celgene Honoraria Consulting

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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