Pediatric Cutaneous Lupus Erythematosus Treated with Pulsed Dye Laser

Pulsed dye laser (PDL) has been used in adults to treat refractory cutaneous lupus erythematosus (CLE). We report the first case of CLE in a child successfully treated with PDL.     

Subacute Cutaneous Lupus Erythematosus in Iceland

Identification of patients with subacute cutaneous lupus erythematosus (SCLE) in Iceland was performed by means of a survey based on the histopathologic or immunofluorescent diagnosis of lupus erythematosus. Respondents underwent serologic studies, gave histories, and, dependent upon the latter, were examined. The resultant proportional rate of patients with SCLE approached 10% of all lupus erythematosus cases.     

Cutaneous Mucinosis in a Child with Systemic Lupus Erythematosus

Cutaneous mucinosis is rarely associated with systemic lupus erythematosus. A 13-year-old girl had a two-month history of numerous cutaneous mucinous papules and the new onset of systemic lupus erythematosus. The differential diagnosis and pathogenesis of this variant of cutaneous mucinosis are discussed. This is the first report of a child with this unusual entity.     

A Statistical Analysis of a Pediatric Dermatology Clinic

Statistical analysis was performed of the diseases seen and therapies utilized during the first two years of the Pediatric Dermatology Clinic at the University of Miami School of Medicine, Miami, Florida. This survey includes 2,821 patient visits made by 1,578 patients. This analysis classifies patients by diagnosis, sex, age, race, and treatment. Diagnostic and treatment frequencies, sex and race ratios, and trends in the patient's age at presentation are also noted. This survey reveals a wide spectrum of pediatric dermatologic disease with 154 different diagnoses made. A review of the 86 different therapeutic modalities utilized is also presented.     

Pediatric Hospital Dermatology: Experience with Inpatient and Consult Services at the Mayo Clinic

Data describing the management of pediatric patients admitted to a hospital under the care of a dermatologist and dermatology hospital consults for pediatric inpatients are limited. We aim to describe the role of an inpatient hospital service jointly run by dermatology and pediatrics and the activities of a pediatric dermatology hospital consult service. We retrospectively identified pediatric (age < 18 yrs) dermatology inpatients and hospital consult patients from January 1, 2009, through December 31, 2010. We examined patient demographics, indications for admission, length of stay, treatment provided, consult‐requesting service, and consult diagnosis. One hundred eight admissions were by a dermatologist. The mean age was 5.8 years; the median length of stay was 3 days. Indications for admission included atopic dermatitis (86.1%), psoriasis (3.7%), and eczema herpeticum (2.8%). The main treatment provided was wet dressings (97.2%). Eighty‐three dermatology hospital consults were requested. The mean age was 7.4 years. The main indications for dermatology consultation included drug rash (12.1%), cutaneous infections (12.1%), contact dermatitis (9.6%), psoriasis (8.4%), atopic dermatitis (6.0%), and hemangiomas (6.0%). This study describes the utility of the hospital pediatric dermatology inpatient and consult services in treating patients with severe skin disease.     

Linear Cutaneous Lupus Erythematosus in the Lines of Blaschko

The lines of Blaschko describe distribution patterns which may represent embryologic developmental pathways. This distribution may occasionally be followed by some congenital and acquired skin disorders. We describe a child with linear cutaneous lupus erythematosus (LCLE) following Blaschko lines on the face.     

Optimal Use of Antimalarials in Treating Cutaneous Lupus Erythematosus

Antimalarials have been used to treat cutaneous and systemic lupus erythematosus (LE) for decades. Although controlled studies comparing the efficacy of antimalarials versus placebo and other treatments are generally lacking, many case reports and series support the therapeutic efficacy of these agents in treating both LE-specific and -nonspecific skin lesions. Currently, the two most frequently used antimalarial agents are chloroquine and hydroxychloroquine. There may be a delay of weeks to months in the onset of therapeutic effects of antimalarials when treating LE. Smoking appears to inhibit the therapeutic efficacy of antimalarials when treating cutaneous LE. Antimalarials have been associated with a number of potentially serious adverse effects, including irreversible loss of vision. The aim of this review is to discuss the many facets of antimalarials that will help clinicians optimally utilize these agents when treating cutaneous LE.     

Chronic Cutaneous Lupus Erythematosus: Depression Burden and Associated Factors

American Journal of Clinical Dermatology - Depression may occur in up to 30% of individuals with cutaneous lupus erythematosus (CLE), many of whom may also have systemic manifestations. Chronic...     

Studies on Criteria of the European Academy of Dermatology and Venerology for the Classification of Cutaneous Lupus Erythematosus

A group of 140 cases of various forms of lupus erythematosus (LE) were examined for 24 variables, including the 11 criteria of the American Rheumatism Association (ARA) for the classification of systemic lupus erythematosus (SLE), and 13 additional criteria suggested by the European Academy of Dermatology and Venerology (EADV) for studies of cutaneous LE with or without systemic involvement. The EADV study factors included skin histopathology and immunopathology, complement and IgG levels, and other laboratory tests, as well as selected clinical findings, most notably the papulosquamous and/or annular lesions that characterize subacute cutaneous LE (SCLE). The patients examined included 50 SLE, 35 SCLE, 30 discoid LE (DLE), 25 disseminated DLE (DDLE), and 17 polymorphous light eruption (PMLE) cases. Preliminary analyses of the data reveal the following: (1) The SCLE cases differed significantly from SLE, DLE, and DDLE in 10 of 11 ARA criteria (all but photosensitivity). (2) The frequencies of positive findings in SCLE also differed significantly for 11 of 13 EADV study factors. (3) While no significant differences appeared in the frequency of photosensitivity between the five study groups, photo-testing revealed significant increases in the frequency of persistence of the photo reactions for 10 days and their Koebnerization in the SCLE cases. (4) The presence of SS-A (Ro)/SS-B (La) antibodies had some predictive value for the appearance of systemic involvement in SCLE, as seen by the increased frequencies of five or more ARA criteria, although highly significant differences from SLE occurred in the absence of renal involvement and lower frequency of ANA and LE band test.(ABSTRACT TRUNCATED AT 250 WORDS)     

亚急性皮肤型红斑狼疮误诊为银屑病1例

患者女,72岁。颜面、躯干及四肢出现泛发性红斑鳞屑3月,初诊银屑病。背部皮损组织病理检查示:表皮角化过度、棘层肥厚与萎缩交替,基底层液化变性,真皮浅层淋巴细胞、噬色素细胞呈带状浸润,免疫学检查示:ANA1:320斑点型,抗Ro/SSA抗体2+,抗La/SSB抗体2+。诊断:亚急性皮肤型红斑狼疮。     

Cutaneous Lupus Erythematosus: An Update on Pathogenesis,Diagnosis and Treatment

Cutaneous lupus erythematosus (CLE) includes a broad range of dermatologic manifestations, which may or may not be associated with systemic disease. Recent studies in this area continue to shape our understanding of this disease and treatment options. Epidemiologic studies have found an incidence of CLE of 4.30 per 100,000, which approaches similar analysis for systemic lupus erythematosus (SLE). Although there have been extensive efforts to define SLE, the classification of CLE and its subgroups remains a challenge. Currently, diagnosis relies on clinical and laboratory findings as well as skin histology. The Cutaneous Lupus Area and Severity Index? (CLASI?) is a validated measure of disease activity and damage. CLE pathogenesis is multifactorial and includes genetic contributions as well as effects of ultraviolet (UV) light. Immune dysregulation and aberrant cell signaling pathways through cytokine cascades are also implicated. Patient education and avoidance of triggers are key to disease prevention. Antimalarials and topical steroids continue to be the standard of care; however, immunosuppressants, thalidomide analogs and monoclonal antibodies are possible systemic therapies for the treatment of recalcitrant disease.     

Membranous and Soluble Forms of Fas Antigen in Cutaneous Lupus Erythematosus

The role of Fas-mediated apoptosis in cutaneous lupus erythematosus (LE) is still unclear, although the Fas/FasL system has been investigated in autoimmune diseases in relation to impaired apoptosis. In order to elucidate the connections between acute cutaneous LE (ACLE) and chronic cutaneous LE (CCLE), we determined the expression of membranous Fas antigen (mFas) on peripheral blood mononuclear cells (PBMC) by flow cytometry and the levels of the soluble form of the Fas antigen (sFas) in sera. The ratio and the mean fluorescence intensity of mFas were much higher in ACLE patients than in others, including patients with CCLE and atopic dermatitis and normal healthy controls. The levels of sFas in ACLE and CCLE patients were also elevated, and there was a significant increase in sFas levels in ACLE patients over that in CCLE patients. Immunohistochemical studies revealed that Fas antigen was predominantly expressed on infiltrating cells around blood vessels and appendages in ACLE and CCLE patients. Based on these findings, it is suggested that the expression of Fas antigen is closely associated with the activation of circulating lymphocytes, especially in ACLE patients, but is not directly associated with keratinocyte damage.     

Histopathology and Immunofluorescence of Discoid Lupus Erythematosus in Children

Abstract: The histopathology and immunofluorescence of discoid lupus erythematosus (DLE) are well documented In adults, but in children they have not been studied systematically. We reviewed nine skin biopsy specimens from six children with DLE. Only three specimens showed the well-developed epidermal changes seen in lesions of adults with DLE, and in the other six, these changes were either mild or absent. All nine specimens showed some degree of vacuolar alteration of the basal layer, ranging from focal and mild to severe. They all had a moderate to heavy lymphocytic dermal infiltrate in a perivascular and periadnexal distribution. Direct immunofluorescence performed on five specimens of lesional skin was weakly positive for IgM or IgG in three cases, negative in one, and strongly positive for IgM, IgG, IgA, and C3 in one. This study indicates that the range of histologic and immunofluorescence features in children with DLE is similar to that in adults. The histologic findings are distinctive enough to be useful in establishing the diagnosis, although clinico-pathologic correlation is required.     

Conversion of Antinuclear Antibody Specificity as a Marker of Deterioration of Cutaneous Lupus Erythematosus into Lupus Nephritis

A 34-year-old male systemic lupus erythematosus patient (SLE) with cutaneous vasculitis developed renal failure after switching anti-nuclear antibody (ANA) specificity. He developed cutaneous lupus erythematosus with homogeneous and speckled type ANA and a high titer of anti-DNA antibody without renal involvement at 21 years of age. After developing lupus nephritis at the age of 27, the original ANA disappeared gradually. Two years later, a discrete speckled type ANA titer elevated abruptly to as high as 1:640 with low complementemia and without DNA antibody. Within five years, he suffered renal failure. This case of SLE suggests a direct correlation with ANA pattern and organ involvement.     

Erythema Gyratum Atrophicans Transiens Neonatale: A Variant of Cutaneous Neonatal Lupus Erythematosus

We cared for a patient with neonatal lupus erythematosus with cutaneous and hepatic involvement that was clinically and histologically indistinguishable from erythema gyratum atrophicans transiens neonatale, an entity described by Gianotti and Ermacora in 1975, which we consider to be a subtype of cutaneous neonatal lupus erythematosus. The differential diagnosis of any annular erythema in the newborn requires careful clinicopathologic evaluation, with direct immunofluorescence studies and determination of anti-Ro/SS-A, anti-La/SS-B, and anti-nRNP antibodies in both the baby and the mother.