A Case of Subacute Cutaneous Lupus Erythematosus That Simultaneously Presented with Papulosquamous and Annular Lesions / 대한피부과학회지

Subacute cutaneous lupus erythematosus (SCLE) includes the papulosquamous and annular-polycyclic variants that present with psoriasiform lesions and annular/polycyclic erythema, respectively. Most patients predominantly have either one or the other subtype, but it is rare that patients have a combination of both lesions. A 13-year-old boy had both erythematous, well-defined, annular-shaped patches and erythematous, ill-defined, hyperkeratotic scaly patches that were scattered on the entire body. Although his clinical manifestations seemed typical for SCLE, his laboratory findings were all negative for LE-specific autoantibodies, including ANA, anti-Ro/SS-A antibody, anti-La/SS-B antibody, anti-dsDNA antibody, etc. The characteristic histopathological features for lupus erythematosus and the granular deposition of IgG and C3 along the dermo-epidermal junction, as detected by direct immunofluorescence testing of lesional skin biopsy specimens, could lead us to the diagnosis of SCLE. We herein report on a case of SCLE that simultaneously presented with both papulosquamous and annular-shaped lesions.

Lupus eritematoso cutáneo subagudo (LECSA): a propósito de 17 casos con anticuerpo anti-Ro positivo / Subacute cutaneous lupus erythematosus (SCLE): 17 patients with anti-Ro antibodies

Se presentan, analizan y comparan con otras series los hallazgos dermatológicos, clínicos, histopatológicos e inmunológicos de 17 casos de lupus eritematoso cutáneo subagudo (LECSA) con anticuerpos (Ac) anti-Ro positivos.Las manifestaciones dermatológicas de nuestros pacientes fueron lesiones papuloescamosas policíclicas, más frecuentes que las psoriasiformes. Se localizaban preferentemente en miembros superiores, dorso y zonas de exposición sola. Presentaban un borde eritematovesiculoso bien delimitado. Otras lesiones adoptaron la forma de reloj de arena. Estas manifestaciones fueron recurrentes con períodos de actividad y calma. También fueron relevantes la fotosensibilidad, la hipopigmentación y, en algunos casos, discreta atrofia. Histopatológicamente lo más destacable fue la degeneración vacuolar de la capa basal de queratinocitos y la ausencia de hiperqueratosis folicular, diferenciándose del lupus eritematoso discoide crónico (LEDC). Las restantes lesiones se parecían a las del lupus eritematosos sistémico (LES), pero con menor intensidad. Hubo poco engrosamiento de la menbrana basal PAS positiva, y el infiltrado linfocitario subpapilar y perivascular fue escaso. En algunos casos se demostró edema dérmico alcian blue positivo, poniendo de manifiesto la presencia de mucina. A diferencia de lo relatado por otros autores, encontramos discreta atrofia en 4 pacientes. Lo más significativo de los exámenes inmunológicos fue la presencia del Ac anti-Ro en el 100% de los enfermos y el anti-La solo en el 17,6%. Fue criterio de inclusión para esta serie tener anti-Ro positivo con anti-ADN y anti-Sm negativos. El FAN fue positivo en el 70%. Con respecto a las manifestaciones clínicas generales, se observaron artritis/artralgias en el 100% de los casos. Las lesiones fueron simétricas, no erosivas ni deformantes. Un solo enfermo tuvo pleuresia y glomerulonefritis crónica difusa...

Clinical, histopathologic, and immunological findings in 17 patients with subacute cutaneous lupus erythematosus (SCLE) and Roantibodies are described. Skin manifestations consisted of recurrent, polycyclical, circumscribed papulosquamous, psoriasiform, or hourglass-like lesions with vesiculoerythematous borders, mainly on the upper limbs, back, and sun-exposed areas. Photosensitivity, hypopigmentation, and occasionally mild atrophy were also noted. Histopathologic features included vacuolar degenerative changes involving keratinocyte basement membrane. Unlike chronic discoid lupus erythematosus lesions, follicular hyperkeratosis was absent. Although other characteristics were similar to those seen in systemic lupus erythematosus patients, specimens from SCLE showed less basement membrane thickening and inflammatory cell infiltrates. A few samples revealed positive alcian blue staining for dermal mucin. Only four patients had moderateatrophy. Since only Ro-positive, and DNA- and Sm-negative patients were assessed, immunological studies showed Ro antibodies in 100%of cases and La antibodies in 17.6%. FAN measurements using rat liver and Hep-2 cells were positive in 70% of patients. SCLE was associated with symmetrical, non-erosive, and non-deforming arthritis/arthralgia in all patients, vasculitis in three (17.8%), Raynaud’s syndrome in two (11.8%), pleuritis and chronic glomerulonephritis in one, and panniculitis in one. Ro antibody screening tests were reviewed.

A Case of Subacute Cutaneous Lupus Erythematosus / 대한피부과학회지

We present a case of subacute cutaneous lupus erythematosus which fulfilled the criteria of systemic lupus erythematosus in a 52-year-old woman with annular and polycyclic erythematous patches on the neck and upper part of the anterior chest. She complained malar of resh, arthralgia, general weakness, and severe headache. Laboratory findings showed hematologic, serologic, central nervous system, and nephrologic abnormalities. Serologic abnormalities of this patient were positive anti-nuclear antibody, anti-La antibody, rhematoid factor, anti-RNP antibody and anti-platelet antibody, but anti-dsDNA and anti-Ro antibody test were negative. She was treated with oral prednisolone and intravenous cyclophosphamide. In the course of treatment, sepsis developed and she died from multi-organ system failure.

A Clinical Study of Localized Scleroderma in Childhood / 대한피부과학회지

BACKGROUND: Localized scleroderma, a rare autoimmune disease, can be classified into three subtypes.linear scleroderma, morphea and generalized morphea. Localized scleroderma is usually limited to the skin and subcutaneous tissue but may result in permanent functional disability. Because of its rarity, there are few large scale clinical studies of pediatric patients with localized scleroderma in Korea. OBJECTIVE: In this study we have examined 11 Korean pediatric patients with localized scleroderma registered at Hanyang university hospital. A review of the clinical presentation, laboratory data, therapy and complcations was carried out in detail. METHODS: The medical records of 11 pediatric patients with localized scleroderma seen at the department at Hanyang University Hospital, between 1990 and 1996, were reviewed. The diagnoses were classified into one of the above three localized scleroderma subtypes based on the results of the physical examination, and all patients were examined periodically. The clinical features of the disease, laboratory results, treatment modalities, and complications were summerized from the review of the records. RESULTS: Among the 11 patients, 8 were female and 3 were male; 7 had morphea and 4 had linear scleroderma. The average age at onset was 8.6 years. Antinuclear antibodies above the titer of 1:40 were present in 36%. One of 3 patients with linear scleroderma which involved the upper extremities had moderate degrees of joint contracture. Ten of 11 patients were treated with intralesional injection of corticosteroid and oral medications of hydroxychloroquine, prednisolone and/or nosteraidal anti-inflammatory drugs. CONCLUSION: Localized scleroderma has no recognized internal organ involvements and is not thought to progress to systemic sclerosis. Since localized scleroderma may cause complications from local atrophy to joint contractures, patients will need to take adequate treatment. and follow-up examinations.