Pustulosis subcórnea: a propósito de un caso / Subcorneal pustulosis: a case report

Resumen La dermatosis pustulosa subcórnea (DPS), oenfermedad de Sneddon-Wilkinson, es una dermatosis poco frecuente, benigna, de curso crónico y recidivante. En esta oportunidad presentaremos el caso de una paciente femenina de 58 años condiagnóstico de dermatosis pustulosa subcórnea, que fue tratada con dapsona 100mg por día, con resolución de lesiones. Realizamos unarevisión bibliográfica de esta patología describiendo sus características clínicas e histológicas, sus múltiples diagnósticos diferenciales y las posibles terapéuticas.

Abstract Subcorneal pustular dermatosis, or Sneddon-Wilkinson disease, is a rare, benign, chronic and recurrent dermatosis. We reportthe case of a 58-year-old female patient with subcorneal pustular dermatosis, who was treated with dapsone 100mg per day, with goodtherapeutic response. We carried out a bibliographic review of this pathology describing its clinical and histological characteristics, its multiple differential diagnoses and possible therapeutic options.

Dermatosis Pustulosa Subcórnea / Subcorneal pustular dermatosis

RESUMEN La dermatosis pustulosa subcórnea o enfermedad de Sneddon Wilkinson, es una enfermedad poco frecuente, caracterizada por lesiones pustulosas, recurrentes que suelen coalescer. Esta afección también se relaciona con enfermedades neoplásicas o inmunológicas. El propósito de este estudio es describir un caso clínico de ladermatosis referida en una paciente de 54 años de edad inicialmente tratada con dapsona, aunque,si bien, durante dos meses hubo mejoría, posteriormente se observa desarrollo de nuevas lesiones y alteración de las transaminasas. Por este motivo se procede a cambiar de tratamiento, por trimetoprima /sulfametoxazol.

Abstract Subcorneal pustular dermatosis or Sneddon Wilkinson's disease is a rare disease characterized by recurrent pustular lesions that often coalesce. This condition is also associated with neoplastic or immunologic diseases. The purpose of this study is to describe a clinical case of the referred dermatosis in a 54-year-old female patient initially treated with dapsone, although there was improvement for two months, subsequently new lesions developed and transaminase alteration was observed, for this reason the treatment was changed to trimethoprim/sulfamethoxazole.

Clinical and Histopathological Profile of Subcorneal Pustular Dermatosis.

Subcorneal Pustular Dermatosis (SPD) is a chronic, benign, recurrent, pustular eruption of idiopathic nature mainly involving the trunk, intertriginous area and flexor aspects of limbs. It is a neutrophilic dermatosis and the exact aetiology is unknown. There are only limited number of studies on the clinical and histopathological aspects of subcorneal pustular dermatosis as well as the associations of subcorneal pustular dermatosis especially in our country. Hence the study is of significance at present.METHODSA descriptive study was done on 37 patients with clinical features of SPD who attended a tertiary care hospital in Kerala during a period of one and a half years. A detailed history, dermatological and system examination, histopathology, immunofluorescence & relevant laboratory investigations were done, and results were analysed using appropriate biostatistics.RESULTSMajority of the patients were females and belonged to sixth decade. 97.3% of patients had recurrence of the disease during summer season. Trunk and intertriginous areas were predominantly affected. Hypothyroidism was observed in 21.6% of patients. Four patients developed chronic plaque psoriasis during the follow up.CONCLUSIONSSubcorneal Pustular Dermatosis is a chronic relapsing pustular eruption which can mimic various other conditions in our daily practice. Many diseases including inflammatory diseases, malignancies and metabolic diseases have been reported to be associated with SPD. Hence a better understanding of the associations and underlying conditions helps in providing effective treatment for the patients.

A Case of Pediatric Subcorneal Pustular Dermatosis / 대한피부과학회지

Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson's disease, is a rare pustular eruption that occurs mainly in middle-aged women and rarely during childhood. Clinically, the pustules are distributed on the trunk and proximal region of the limbs, and cultures of the pustules consistently do not reveal bacterial growth. Histopathology shows subcorneal pustules containing polymorphonuclear leukocytes and chronic progression. SPD is a rare condition, especially in children, and only one case has been reported in Korea. Here, we report a case of 4-year-old girl as an educational case for the diagnosis and treatment of pediatric SPD.

A Case of Immunoglobulin A Pemphigus: Intraepidermal Neutrophilic Dermatosis Type / 대한피부과학회지

Immunoglobulin A (IgA) pemphigus is a rare variant of an autoimmune bullous disease with IgA antibodies. IgA pemphigus is divided into 2 major subtypes: the subcorneal pustular dermatosis (SPD) type and intraepidermal neutrophilic (IEN) dermatosis type. We documented a case of an 18-year-old woman with recurrent generalized blisters and pustules that were especially severe in the intertriginous areas. Some half-and-half blisters and coalesced pustules in an annular pattern with crusts were simultaneously observed. A biopsy specimen from one of the half-and-half blister lesions showed intraepidermal separation with multiple neutrophils. Direct immunofluorescence staining revealed lace-like intercellular deposition of IgA in the entire epidermis. IgA antibody deposits were also observed in the patient's serum. The eruptions cleared with systemic steroids and colchicine 0.6 mg for 1 week, and the patient remained in partial remission at the 8-month follow-up. Herein, we report a case of IEN-type IgA pemphigus, clinically mimicking SPD with half-and-half blisters.

Clinical and Histopathological Study of Generalized Pustular Psoriasis and Subcorneal Pustular Dermatosis Diagnosed at a Tertiary Hospital in Korea / 대한피부과학회지

BACKGROUND: Generalized pustular psoriasis (GPP) and subcorneal pustular dermatosis (SPD) are clinically and histopathologically difficult to distinguish. There have been no comparative studies examining these two diseases in Korea. OBJECTIVE: To investigate the clinical and histopathological characteristics of GPP and SPD. METHODS: We evaluated the clinical features, laboratory, and histopathological findings in 16 patients with generalized pustular eruption who had visited our hospital over the past 10 years and reviewed the literature. RESULTS: Ten GPP and six SPD patients were included in the study. The mean age at diagnosis was 44.4 years in the GPP group and 50 years in the SPD group. The number of patients with previous personal history of psoriasis vulgaris was 2 (20%) for GPP and 0 (0%) for SPD. The number of patients with history of recent exposure to medications was 1 (10%) and 0 (0%) in the GPP and SPD groups, respectively. Symptoms of fever, arthralgia, and mucosal involvement were reported in 10%, 20%, and 10% of GPP patients and 16.7%, 16.7%, and 0% of SPD patients, respectively. Leukocytosis, eosinophilia, elevated ESR/CRP, and elevated AST/ALT were reported in 25%, 0%, 25%, and 50% of GPP patients and in 20%, 0%, 40%, and 40% of SPD patients, respectively. On histological findings, in the GPP group, spongiosis, and psoriasiform changes including hyperkeratosis/parakeratosis, and rete ridge changes were more apparent than in the SPD group. The mean period of clinical improvement was 32.9 days with 40% recurrence in the GPP group and 38.3 days with 66.7% recurrence in the SPD group. CONCLUSION: Although GPP and SPD exhibit clinical and laboratory findings that are similar and difficult to differentiate, systematic analyses including clinical course, laboratory findings, and histopathological findings are helpful for an accurate differential diagnosis.

Dermatosis pustulosa subcórnea como debut de un paciente con lupus eritematoso sistémico

El lupus eritematoso sistémico es una enfermedad inflamatoria que afecta fundamentalmente a mujeres en edad fértil, aunque en ocasiones su debut llega en edades tempranas de la vida. Múltiples son las manifestaciones clínicas que pueden aparecer en el curso de la enfermedad y que afectan a todos los órganos y sistemas de órganos del cuerpo humano. Se describen diversas formas de debut de la enfermedad, dentro de las cuales los síntomas generales, afectación neurológica, dermatológica y renal han sido reportada como las más frecuentes. Se presenta el caso de un niño de 6 años de edad que debuta con un lupus eritematoso sistémico y que su lesión o signo de debut es una manifestación dermatológica: la dermatosis pustulosa subcornea

Systemic lupus erythematosus is an inflammatory disease that mainly affects women of childbearing age, although sometimes its debut comes at early ages of life. Multiple are the clinical manifestations that can appear in the course of the disease and that affect all organs and organ systems of the human body. Various forms of disease debut are described, within which the general symptoms, neurological, dermatological and renal involvement have been reported as the most frequent. We present the case of a 6-year-old boy who debuted with systemic lupus erythematosus and whose lesion or debut sign is a dermatological manifestation: the subcorneal pustular dermatosis

Enfermedad de Sneddon-Wilkinson / Sneddon-Wilkinson disease

Introducción: la dermatosis pustulosa subcórnea o enfermedad de Sneddon-Wilkinson, es una enfermedad poco común, que se distingue por pústulas flácidas, recurrentes y coalescentes; su mecanismo etiológico no está del todo dilucidado, pero se relaciona con la presencia de enfermedades neoplásicas o inmunológicas. Presentación del caso: se presenta el caso de un niño de 4 años de edad, que acude a consulta de Dermatología del Hospital Andino de Chimborazo, con manifestaciones clínicas que permiten hacer el diagnóstico de dermatosis pustulosa subcórnea. Conclusiones: la enfermedad de Sneddon-Wilkinson es una afección rara que se presenta frecuentemente en pacientes femeninas de 40 años y más. Se caracteriza por una amplia diversidad de manifestaciones cutáneas que dificultan el pronóstico y la evolución del paciente. Esta enfermedad, cuando se presenta en niños, puede confundirse con otras afecciones que cursan con exantemas cutáneos(AU)

Introduction: subcorneal pustular dermatosis or Sneddon-Wilkinson disease is a rare illness characterized by recurrent, coalescent and flaccid pustules; its etiological mechanism is not totally known, but it is related to neoplastic or immunological diseases. Case presentation: a four-year old child who went to the dermatology service of Hospital Andino de Chimborazo. He presented with clinical manifestations that allow making the diagnosis of subcorneal pustular dermatosis. Conclusions: Sneddon-Wilkinson disease is a rare illness that frequently occurs in 40 years-old and older women. It has a wide range of skin manifestations that makes prognosis and recovery of patient difficult. When it appears in children, it may create confusion with other illnesses having skin exanthemas(AU)

Subcorneal Pustular Dermatosis Caused by Darbepoetin-α in Hemodialysis Patients / 대한피부과학회지

Subcorneal pustular dermatosis is a pustular eruption that shows subcorneal pustules containing polymorphonuclear leukocytes and chronic progression. It most commonly affects the trunk, inguinal region, and axilla of middle-aged women, and the pustules are distributed bilaterally. Cultures of the pustules consistently do not reveal bacterial growth. Subcorneal pustular dermatosis rarely happens, and no specific etiology and pathogenesis are known. Thus, we present this case to provide dermatologists with information on its adequate diagnosis and treatment and to inform them of the risk of subcorneal pustular dermatosis in kidney dialysis patients who use darbepoetin-α.

A Case of Subcorneal Pustular Dermatosis / 대한피부과학회지

No abstract available.

Subcorneal pustular dermatosis in a dog / 대한수의학회지

Canine Subcorneal pustular dermatosis (CSPD) represents a sterile, superficial, pustular skin disease of unknown cause but may be a variant of pemphigus foliaceus. A 7-year-old, intact female, mixed dog presented with 3-month history of pruritic multiple pustules and crusts. Direct smears from intact pustules revealed numerous nondegenerate neutrophils, some acantholytic cells, and bacterial culture was negative. Histologic examination of lesional skin showed subcorneal pustules filled with neutrophils and acantholytic cells. The direct immunofluorescence tests stained with IgG, IgA, IgM, C3 were negative. Oral administration of dapsone (1 mg/kg/q8h) was initiated and it was reduced to 1 mg/kg/q12h with good control of the lesions.

A Case of Annular Pustular Psoriasis Showing Features of Subcorneal Pustular Dermatosis / 대한피부과학회지

Annular pustular psoriasis(APP) is a rare variant of the generalized pustular psoriasis characterized by subacute onset of annular lesion with peripheral advancing pustules. Histologically, it is characterized by spongiform and/or subcorneal pustules which are also found in subcorneal pustular dermatosis(SPD). SPD is very difficult to differentiate from APP, although SPD is thought to be a separate and distinctive entity because of its clinical appearance, characteristic histology, and response to dapsone. We herein report a case of APP showing clinical and histologic features of SPD developed in a patient who had a history of generalized pustular psoriasis.

A Case of Subcorneal Pustular Dermatosis with Atypical Distribution / 대한피부과학회지

Subcorneal pustular dermatosis(SPD) or Sneddon-Wilkinson disease is a chronic, relapsing, pustular eruption generally involving the intertriginous area and affecting mainly elderly women. We report a case of SPD presenting in a 24-year-old female with erythematous circinate annular peripheral scaly patches on her right thigh. Histopathologically, the subcorneal pustules contained numerous neutrophils, with only a few eosinophils. Underlying stratum malpighii was slightly edematous and contained small numbers of neutrophils. No acantholytic cells or spongiosis were found. The patient was treated by dapsone successfully.