Case Of Subcorneal Pustular Dermatosis Research Articles

Three cases of subcorneal pustular dermatosis with immunohistochemical examinations.

Subcorneal pustular dermatosis, a rare, benign skin disease, is a type of neutrophilic dermatosis. The authors reported three cases of subcorneal pustular dermatosis. In case 1, a 9-year-old girl developed a skin rash with blisters following a mycoplasma infection and had a flare-up due to a common cold. She was successfully treated with a topical corticosteroid. In case 2, a 70-year-old woman who had been treated for rheumatoid arthritis with adalimumab, salazosulfapyridine, and leflunomide developed 3- to 5-mm pustules on her trunk and thighs 4 days after flu vaccination. The rash disappeared with drug withdrawal and treatment with diaminodiphenyl sulfone. In case 3, an 81-year-old man, who was diagnosed with pyoderma gangrenosum at 61 years old, developed multiple small flaccid pustules on his trunk and extremities due to an infection in the arteriovenous shunt area on the forearm. The pustule disappeared with intravenous antibiotic therapy; however, the pustules subsequently flared up along with ulcers typical of pyoderma gangrenosum. He was given oral prednisolone therapy, which was effective for the small pustules and some ulcers. Immunohistochemical examination of the three cases revealed neutrophilic infiltration in the subcorneal layer of the epidermis. The pustules contained neutrophils as well as some CD68+ and a few CD1a+ cells. The epidermis and dermis were more predominantly infiltrated by CD4+ cells than by CD8+ cells. Positive stainings for interleukin 8, interleukin 36γ, and phospho-extracellular signal-regulated kinases 1 and 2 were observed in the upper layers of the epidermis below the pustules. Although the pathogenesis of subcorneal pustular dermatosis has not been clarified, the current results suggest that a variety of inflammatory cells, including those responsible for both innate and acquired immunity, are involved in the accumulation of neutrophils in subcorneal pustular dermatosis.

Subcorneal pustular dermatosis associated with IgG monoclonal gammopathy of undetermined significance

Subcorneal pustular dermatosis is a rare chronic relapsing bullous neutrophilic dermatosis. Because it can be associated with monoclonal gammopathy of undetermined significance and multiple myeloma, screening for these conditions is necessary. Herein, we present a case of subcorneal pustular dermatosis, with concurrent monoclonal gammopathy of undetermined significance, successfully treated with acitretin.

Subcorneal pustular dermatosis: Comprehensive review and report of a case presenting during pregnancy

Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a rare, relapsing, sterile pustular eruption of unknown etiology that develops most commonly in middle-aged or mature women. This article reviews the presentation, associations, and management of the condition and highlights advances in pathophysiology. Onset of SPD during pregnancy has not been reported. Herein, we report a case of SPD that developed during pregnancy. The patient was treated with dapsone without complications for her or the fetus. An association between T helper (Th) 17 and Th2 environments in the development of SPD has been advocated. Pregnancy is characterized by a predominance of Th2 responses and increased interleukin-17 levels and thus may favor the development of the condition

Subcorneal Pustular Dermatosis Successfully Treated with Acitretin in a Patient with Glucose-6-Phoshate Dehydrogenase Deficiency

Subcorneal pustular dermatosis (SPD) is a rare neutrophilic dermatosis. SPD most commonly presents as a serpiginous pattern of pustules on the trunk or intertriginous areas of middle-aged females. It tends to have a chronic disease course and patients may experience relapses. Dapsone currently remains the treatment of choice for SPD. However, in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency, alternative therapies are required. We report a case of subcorneal pustular dermatosis in a G6PD deficient patient successfully treated with acitretin.

Subcorneal pustular dermatosis: An unusual presentation in pediatric age group

Subcorneal pustular dermatosis (SPD), first described by Sneddon and Wilkinson, represents a rare chronic and relapsing sterile pustular eruptions generally involving the trunk and flexor aspect of limbs. Usually, it affects middle-aged or elderly women. Histologically, it is characterized by the accumulation of subcorneal neutrophils with an absence of spongiosis and acantholysis. Here, we are reporting a rare case of SPD in the pediatric age group, which was diagnosed based on clinical and histological findings. We gave dapsone in treatment with a positive outcome. To the best of our knowledge, there has not been any case report in the pediatric age group from India so far.

Subcorneal Pustular Dermatosis: A Case Report of a Patient with Diffuse Scleroderma

Subcorneal pustular dermatosis (SPD) or Sneddon-Wilkinson disease is a rare, benign, chronic, sterile pustular eruption which is associated with various systemic diseases including immunoglobinopathies, neoplasms, and autoimmune disorders. This paper reports a case of SPD in a patient with diffuse scleroderma in a 37-year-old woman. The hypothesis that immune dysregulation may play a role in the pathogenesis of SPD was supposed by the coexistence of diffuse scleroderma and SPD in our patient.

A case of concomitant subcorneal pustular dermatosis and erythema nodosum leprosum in borderline lepromatous leprosy-relapses

Subcorneal pustular dermatosis (SPD) is a rare, chronic, and recurrent pustular eruption characterized histopathologically by subcorneal pustules that contain neutrophils. SPD has been clearly reported conjunction with other diseases. Leprosy reactions are acute inflammatory process that immunologically driven on the chronic course of leprosy. Erythema nodosum leprosum (ENL) is a type II of leprosy reaction putatively can initiate SPD lesions. We report one case of concomitant SPD and ENL in borderline lepromatous leprosy-relapses. A 41-year-old man with the history of using multidrug therapy-multibacillary for leprosy presented with painful erythematous nodules on the trunk and extremities, accompanied by pustules on erythematous base on the face, arms, buttocks, and legs. There were thickening of both ulnar nerves with gloves and stocking hypesthesia. The bacterial index was 3+ and morphological index was 20\%. Histopathological examination on the pustule revealed subcorneal pustules with exocytosis of neutrophils which supported the diagnosis of SPD. A possible immunologic mechanism has been suggested in the induction of the occurence both SPD and ENL.

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.

Intercellular IgA dermatosis (IAD) is a subset of autoimmune bullous disease exclusively with IgA antikeratinocyte cell-surface antibodies. The classification and pathogenesis of this condition are still obscure. To classify IAD and study its pathogenesis. From our cohort of 5402 cases of autoimmune bullous disease, we selected 49 cases of various types of intercellular IgA dermatosis (IAD) and 13 cases of classical subcorneal pustular dermatosis (SPD), for which sera and information were available. We studied these cases clinically and immunologically. There were 17 SPD-type IAD, 12 intraepidermal neutrophilic IgA dermatosis (IEN)-type IAD, two IgA-pemphigus vegetans, four IgA-pemphigus foliaceus, six IgA-pemphigus vulgaris and eight unclassified IAD cases. There was no sex predominance, and the average age at disease onset was 45·9years. Clinically, bullous and pustular skin lesions developed on various sites, particularly intertriginous areas. Histopathology showed intraepidermal blisters or pustules at the upper epidermis in the SPD-type and at the midepidermis in the IEN-type. Immunological studies revealed that direct immunofluorescence, indirect immunofluorescence of normal human skin and enzyme-linked immunosorbent assays (ELISAs) of recombinant proteins of desmogleins and desmocollins frequently showed positive results, although no antigens were detected in many cases. All cases of classical SPD, which showed no positive immunological results, were indistinguishable clinically and histopathologically from SPD-type IAD. The present study of the largest cohort of cases of IAD showed that themajor subtypes are SPD and IEN, and that the combination of indirect immunofluorescence and ELISAs of desmogleins and desmocollins, in addition to direct immunofluorescence, was useful for the diagnosis of IAD and its subtypes.

本態性血小板血症に合併し血中 TNF-α の上昇を伴った角層下膿疱症の 1 例

本態性血小板血症に合併し血中 TNF-α の上昇を伴った角層下膿疱症の 1 例

トイ・プードルにみられた角層下膿疱症の1例

トイ・プードルにみられた角層下膿疱症の1例

Palmoplantar subcorneal pustular dermatosis following adalimumab therapy for rheumatoid arthritis

Tumour necrosis factor (TNF)-α inhibitors represent potent new therapies for severe forms of psoriasis, psoriatic arthritis, and several other immune-mediated disorders. Paradoxical worsening or de novo development of psoriasis has been documented with their use. Palmoplantar pustulosis has been one of the commoner presentations of this unusual side effect. Subcorneal pustular dermatosis (SPD) has some similarity to pustular psoriasis, particularly the acral form of SPD. Thus far there have been no biopsy-proven cases of SPD associated with TNF-α inhibitor use. We describe clinical and histopathological features of a pustular skin condition which occurred in a 48-year-old woman with rheumatoid arthritis who had started adalimumab four months prior. The adalimumab had been added to her usual treatment with methotrexate because of incomplete symptom control. Painful and pruritic skin lesions were noted on her palms and soles primarily, with some extension to the limbs and abdomen. Examination revealed relatively non-inflamed pustules with fluid levels, together with sparse crusted papules. Histopathology showed subcorneal pustules more suggestive of SPD than pustular psoriasis. The eruption resolved completely when adalimumab was withdrawn; methotrexate was continued. Subcorneal pustular dermatosis, in addition to psoriasis vulgaris and pustular psoriasis, may occur in patients treated with TNF-α inhibitors like adalimumab.

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

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.

Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) occurring in association with nodal marginal zone lymphoma: A case report

Sneddon-Wilkinson disease or subcorneal pustular dermatosis (SPD) is a rare, benign inflammatory skin disorder of unknown etiology. SPD is associated with various systemic disorders, including immunoglobinopathies and lymphoproliferative disorders. The relationship between SPD and immune dysfunction is unlikely to be incidental, although it still remains a mystery whether the associated gammopathies are primary or secondary to the pathogenesis of the disease. Herein, we report the first case of SPD in association with marginal zone lymphoma.

Subcorneal Pustular Dermatosis (Sneddon-Wilkinson Disease) with Absence of Desmoglein 1 and 3 Antibodies

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.

A case of subcorneal pustular dermatosis treated successfully with a combination of cyclosporin and prednisolone

A case of subcorneal pustular dermatosis treated successfully with a combination of cyclosporin and prednisolone

Maladie de Sneddon-Wilkinson. À propos de quatre cas

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.

Dermatosis pustulosa subcórnea en un paciente con espondilitis anquilosante

Abstract—We present the case of a 33-year-old patient, diagnosed with ankylosing spondylitis 12 years before, who came to our practice after having had skin symptoms consistent with subcorneal pustular dermatosis (SPD) for a month. Although SPD is considered a purely cutaneous disease, associations with other entities have been described. We describe a case of SPD appearing along with a 12-year case of ankylosing spondylitis, and we review the literature of cases described to date associated or simultaneous with SPD.

Subcorneal pustular dermatosis involving the face

We report an interesting case of subcorneal pustular dermatosis in a young woman with prominent involvement of the face. Facial involvement in this condition has not previously been reported.

Subcorneal pustular dermatosis involving the face

We report an interesting case of subcorneal pustular dermatosis in a young woman with prominent involvement of the face. Facial involvement in this condition has not previously been reported.

Subcorneal pustular dermatosis in a patient with aplastic anemia

Subcorneal pustular dermatosis (SPD) is a chronic, relapsing vesiculopustular eruption that typically affects the trunk and intertriginous areas and usually occurs in women more than 40 years of age. 1 Sneddon IB Wilkinson DS. Subcorneal pustular dermatosis. Br J Dermatol. 1979; 100: 61-68 Crossref PubMed Scopus (104) Google Scholar We describe a case of SPD in a patient with aplastic anemia induced by antituberculosis medications. Although the association of SPD with paraproteinemia, myeloma, and inflammatory bowel diseases such as ulcerative colitis and Crohn’s disease has been recognized, 2 Kasha EE 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: 854-858 Abstract Full Text PDF PubMed Scopus (83) Google Scholar , 3 Delaporte E Colombel JF Nguyen-Mailfer C et al. Subcorneal pustular dermatosis in a patient with Crohn’s disease. Acta Derm Venereol (Stockh). 1992; 72: 301-302 PubMed Google Scholar an association with aplastic anemia has not been previously reported.