DERMATOPATHOLOGY CASES: Self-Assessment Cases: Editor - Dr Sampurna Roy MD

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Sunday, February 6, 2011

Answer of Dermatopathology Case 92

Subcorneal Pustular Dermatosis (Sneddon-Wilkinson disease)

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Subcorneal pustular dermatosis: 50 years on.Clin Exp Dermatol. 2008 May;33(3):229-33. Epub 2008 Mar 18.

We review the key developments in our understanding of subcorneal pustular dermatosis (SCPD, also known as Sneddon-Wilkinson disease) over the past 50 years. SCPD is a rare, chronic, sterile pustular eruption that was first described by Sneddon and Wilkinson in 1956. The primary lesions are pea-sized pustules classically described as half-pustular, half-clear flaccid fluid blisters. Histologically the salient feature is a subcorneal accumulation of neutrophils, suggesting the presence of chemoattractants such as tumour necrosis factor (TNF)alpha in the uppermost epidermis. However, to date its exact pathophysiology is unknown. Cases in association with pyoderma gangrenosum, benign monoclonal IgA gammopathy and multiple myeloma are well documented. There are anecdotal reports of SCPD associated with other internal malignancies such as chronic lymphocytic leukaemia, thymoma, apudoma and epidermoid carcinoma of the lung. The treatment of choice is dapsone. Therapeutic alternatives include retinoids, phototreatment with psoralen ultraviolet (UV) A, broadband or narrow band UVB and corticosteroids. Anecdotal uses of tacalcitol, ketoconazole, azithromycin, tetracycline, minocycline, vitamin E, ciclosporin, colchicine, mizoribine, mebhydrolin, infliximab and adalimumab with mycophenolate mofetil have all been reported.

A case of subcorneal pustular dermatosis in association with monoclonal IgA gammopathy successfully treated with acitretin. J Dermatolog Treat. 2010 Mar;21(2):114-6.

Subcorneal pustular dermatosis (SPD), or Sneddon-Wilkinson disease, is a rare, chronic, recurrent, pustular eruption. Association with several diseases is well known, mainly IgA and IgG gammopathies or myelomas. Although dapsone is often considered to be the first-line treatment, some patients fail to respond or cannot tolerate the side effects. For cases that do not respond well to this treatment, acitretin, an excellent second-line treatment, may be used. Herein, a 55-year-old woman with SPD associated with monoclonal IgA gammopathy refractory to dapsone is presented, who was successfully treated with acitretin in a short period.

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.

Subcorneal pustular dermatosis (SPD) [Sneddon-Wilkinson disease] is a benign and uncommon disorder characterized by a chronic, relapsing vesiculopustular eruption of unknown etiology. We present a case of SPD in a young Black woman in whom ELISA was performed to test for desmoglein 1 and 3 antigens (the first reported case of evaluation for these antigens in a patient with SPD). The test revealed the absence of both antibodies. The patient was successfully treated with topical corticosteroids and narrow-band UVB phototherapy. In this report, we review both the pathophysiology of SPD, which has yet to be clarified, and its treatment. Data obtained from our case report add further support to the hypothesis that a non-antibody-mediated mechanism is operative in SPD. The treatment of choice for SPD is dapsone. However, the combination of corticosteroids and UVB phototherapy should be considered a valid therapeutic option in patients who are not appropriate candidates for dapsone therapy.

[Sneddon-Wilkinson disease. Four cases report].Rev Med Interne. 2004 Feb;25(2):154-9.

INTRODUCTION: We report four cases of subcorneal pustular dermatosis or Sneddon-Wilkinson disease. Clinical and histological lesions and immunofluorescence data were presented. This disease is classified among neutrophilic dermatitis.
PATIENTS AND METHODS: All of four patients presented with clinical and histological lesions compatible with the diagnosis of Sneddon-Wilkinson disease. Indeed, direct and indirect immuno-testing were negative. We noted an association with a benign IgA monoclonal gammapathy in one case and with a seronegative polyarthritis in one other case. Three patients correctly responded to dapsone. One of them after transient improvement was resistant to dapsone and then dramatically responded to etretinate.
CONCLUSION: Subcorneal pustular dermatosis is a chronic disease, rarely described in literature. It's a pustular eruption, involving the trunck, axillae and inguinal holds. It's often associated with monoclonal gammapathy, particulary IgA. Its nosological situation is still contested, especially with IgA pemphigus sharing with it the association with IgA monoclonal gammapathy and the same efficacy of dapsone. We discuss relationships between both diseases.

Subcorneal pustular dermatosis type of IgA pemphigus: demonstration of autoantibodies to desmocollin-1 and clinical review.Br J Dermatol.2000;143(1):144-8.

We describe a 40-year-old Japanese man with a 3-year history of vesiculopustular lesions resembling subcorneal pustular dermatosis. Histopathology showed subcorneal pustules containing a few acantholytic cells, and direct immunofluorescence disclosed IgA deposition in the intercellular space of the upper epidermis. Circulating IgA autoantibodies of very low titre were also demonstrated by indirect immunofluorescence. A novel cDNA transfection technique clearly detected IgA autoantibodies reactive with human desmocollin-1. Combined therapy with dapsone and etretinate improved the skin lesions. We review the clinical features of 49 patients in the literature who presented with vesiculopustular lesions and intraepidermal IgA deposition.

Subcorneal pustular dermatosis treated with PUVA therapy. A case report and review of the literature. Dermatology.1999;198(2):203-5.

BACKGROUND: Subcorneal pustular dermatosis (SPD) is a chronic recurrent pustular dermatosis of unknown etiology. Many treatments have been proposed, none of which has been uniformly successful.
OBJECTIVE: Our purpose is to report a patient with SPD successfully treated by PUVA and to review the literature concerning phototherapy treatment of SPD.
METHODS: A patient suffering from SPD resistant to diaminodiphenylsulphone (dapsone) responded well to a combination therapy consisting of dapsone and PUVA. He received 50 mg/day and 3 PUVA sessions a week. Photographs were taken at baseline and after 15 sessions.
RESULTS: The lesions were virtually cleared after 15 sessions. The patient remained free of lesions with a maintenance therapy of dapsone (50 mg/ day) and 1 PUVA session a week.
CONCLUSION: The therapeutic value of phototherapy for the treatment of SPD still has to be confirmed and could be a valuable alternative for treatment-resistant patients.