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Subcorneal pustular dermatosis in a patient with rheumatoid arthritis and diffuse scleroderma

  • Author(s): Brantley, Elise I
  • Sheth, Pranav
  • et al.
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Subcorneal Pustular Dermatosis in a Patient with Rheumatoid Arthritis and Diffuse Scleroderma
Elise I Brantley MD1, Pranav Sheth MD2
Dermatology Online Journal 15 (3): 5

1. Dermatology Resident
2. Assistant Professor of Dermatology
Department of Dermatology, University of Cincinnati, Ohio. pranav.sheth@uc.edu


Abstract

We report a case of subcorneal pustular dermatosis (SPD) in a patient with rheumatoid arthritis (RA) and diffuse scleroderma. Although SPD has been described in association with RA, it has not been reported in association with diffuse scleroderma to our knowledge. This observation supports the hypothesis linking immune dysfunction as a common pathogenesis of these three entities.



Introduction

Subcorneal pustular dermatosis (SPD) of Sneddon and Wilkinson was first described in 1956 [1]. It is a rare disorder characterized by recurrent "crops" of discrete sterile vesiculopustules. SPD has been reported alone and in association with multiple systemic autoimmune disorders, hypergammaglobulinemias, and neoplasms. We report a case of subcorneal pustular dermatosis associated with diffuse scleroderma and rheumatoid arthritis.


Case Report

A 37-year-old African-American female presented with a pruritic, occasionally painful, chronic blistering eruption of one-year duration involving the abdomen, back, and thighs. She had received several short courses of systemic steroid tapers followed by rapid recurrence, and subsequently longer term tapering prednisone. At the time of her initial visit, she was on prednisone 2.5 mg for 3 months with continued moderate activity. Her past medical history was significant for rheumatoid arthritis and diffuse scleroderma for 10 years. Her medications included atenolol, valsartan, hydroxychloroquine, furosemide, nifedipine, and naprosyn, all of which had been given for over one year.


Figure 1aFigure 1b
Figure 1a. Abdomen: On the trunk and proximal extremities are symmetric, diffusely scattered vesicopustules
Figure 1b. Close-up of abdomen: Some exhibit the meniscus sign on a background of hyperpigmentation

On physical exam, involving the chest, back, abdomen, and thighs there were numerous, 2-6 mm superficial vesicles and pustules, some of which exhibited the meniscus sign (Figs. 1a & 1b). The lesions were on a background of hyperpigmented round macules. Her fingertips had a slight, firm taper and early sclerodactyly. She had marked joint deformities of the proximal interphalangeal joints and slight ulnar deviation at the metacarpophalangeal joints bilaterally. Her mucous membranes were clear.


Figure 2Figure 3
Figures 2-4. A subcorneal pustule containing few neutrophils and acantholytic cells

Figure 4

The differential diagnosis included pustular psoriasis, dermatitis herpetiformis, pemphigus foliaceus, subcorneal-type IgA pemphigus, and subcorneal pustular dermatosis. Histopathology revealed a subcorneal vesicle with few neutrophils and epidermal acantholytic cells (Figs. 2-4). The superficial dermis revealed a moderate lymphocytic infiltrate with neutrophils and eosinophils. Direct immunofluorescence was negative on two occasions. Indirect immunofluorescence using salt split skin and monkey esophagus was negative. These findings were consistent with subcorneal pustular dermatosis of Sneddon and Wilkinson.

Given the patient's multiple other medical problems and medications, she was started on Dapsone 50 mg a day in addition to the prednisone 2.5 mg. She had rapid clearance of her skin within two weeks. After two months, a flare with new lesions prompted an increase of the Dapsone to 100 mg daily. This resulted in complete and sustained clearance at all subsequent visits during a follow up period of nine months.


Discussion

Subcorneal pustular dermatosis (SPD) of Sneddon and Wilkinson is a rare disorder presenting with recurrent sterile vesicopustules. It generally affects women in the third decade or older and runs a benign yet chronic course. Subcorneal pustular dermatosis typically responds rapidly to dapsone, though other treatment options have been reported including colchicine, PUVA, UVB, cyclosporine, and infliximab [2, 3, 4, 5].

Differentiating SPD from other causes of superficial vesiculopustular eruptions can be challenging. Clinically, the lack of systemic symptoms, the lack of confluence of the pustules, and the larger size of the pustules puts pustular psoriasis lower on the differential. Histopathology and immunofluorescence are needed to rule out the other entities. On histopathology, lesions demonstrating a sterile subcorneal pustule rule out dermatitis herpetiformis. The minimal findings of acantholysis do not lend themselves to the various forms of pemphigus, nor do the negative immunofluorescence studies.

Subcorneal pustular dermatosis has been reported alone and in association with many disorders including seropositive and seronegative rheumatoid arthritis (RA), Sjögren disease, Crohn disease, systemic lupus erythematosus, Grave disease, multiple sclerosis, pyoderma gangrenosum, synovitis-acne-palmoplantar-pustulosis-hyperosteosis-osteitis (SAPHO) syndrome, benign monoclonal IgA, IgG, and IgM gammopathy, acquired immune deficiency syndrome, multiple myeloma, apudoma, and pancreatic cancer [6-26]. The etiology of SPD is largely unknown, but given its association with several autoimmune disorders and hyperimmunoglobulinemia, some have proposed an underlying autoimmune etiology or systemic immunologic defect.

The association between RA and SPD has been noted for some time. There are many proposed theories linking the pathogenesis of the two entities. The activation and chemotaxis of neutrophils is intimately involved in the pathogenesis of both conditions, as is up regulation of many cytokines and chemokines, including interleukin (IL)-8 and tumor necrosis factor (TNF)-alpha [9, 14, 27, 28].

The pathogenesis of diffuse scleroderma involves immune dysregulation and a cascade of synergistic events including endothelial cell damage, activation of T cells, proinflammatory and profibrotic cytokine production, inflammation, and activation of fibroblasts [29]. Immune dysregulation plays a key role in many of the other diseases found to be associated with SPD, including multiple sclerosis, monoclonal IgA gammopathies, and SLE [15, 17, 20, 22, 30]. The coexistence of diffuse scleroderma and SPD in our patient supports the hypothesis that immune dysregulation may play a role in the pathogenesis of SPD.


Summary

Subcorneal pustular dermatosis is a chronic blistering disorder that affects young to middle-aged women. In our patient, SPD was associated with underlying RA and diffuse scleroderma. The rapid response to dapsone probably speaks to the presence of neutrophils seen histologically. Though the etiology is poorly understood, its association with immunologic disorders such as RA and scleroderma may offer insight into its pathogenesis.

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