The initial appearance of subacute cutaneous lupus erythematosus (SCLE) skin lesions in conjunction with Ro/SS-A autoantibodies occurring as an adverse reaction to hydrochlorothiazide [i.e. drug-induced SCLE (DI-SCLE)] was first reported in 1985. Over the past decade an increasing number of drugs in different classes has been implicated as triggers for DI-SCLE. The management of DI-SCLE can be especially challenging in patients taking multiple medications capable of triggering DI-SCLE. Our objectives were to review the published English language literature on DI-SCLE and use the resulting summary data pool to address questions surrounding drug-induced SCLE and to develop guidelines that might be of value to clinicians in the diagnosis and management of DI-SCLE. A systematic review of the Medline/PubMed-cited literature on DI-SCLE up to August 2009 was performed. Our data collection and analysis strategies were prospectively designed to answer a series of questions related to the clinical, prognostic and pathogenetic significance of DI-SCLE. One hundred and seventeen cases of DI-SCLE were identified and reviewed. White women made up the large majority of cases, and the mean overall age was 58·0 years. Triggering drugs fell into a number of different classes, highlighted by antihypertensives and antifungals. Time intervals ('incubation period') between drug exposure and appearance of DI-SCLE varied greatly and were drug class dependent. Most cases of DI-SCLE spontaneously resolved within weeks of drug withdrawal. Ro/SS-A autoantibodies were present in 80% of the cases in which such data were reported and most remained positive after resolution of SCLE skin disease activity. No significant differences in the clinical, histopathological or immunopathological features between DI-SCLE and idiopathic SCLE were detected. There is now adequate published experience to suggest that DI-SCLE does not differ clinically, histopathologically or immunologically from idiopathic SCLE. It should be recognized as a distinct clinical constellation differing clinically and immunologically from the classical form of drug-induced systemic lupus erythematosus.

The skin is the second most frequently affected organ system in lupus erythematosus. Although only very rarely life threatening--an example is lupus erythematosus-associated toxic epidermal necrolysis--skin disease contributes disproportionally to disease burden in terms of personal and psychosocial wellbeing, vocational disability, and hence in medical and social costs. Since several manifestations are closely associated with the presence and activity of systemic lupus erythematosus, prompt and accurate diagnosis of cutaneous lupus erythematosus is essential. This review aims to cover common, rare, and atypical manifestations of lupus erythematosus-associated skin disease with a detailed discussion of histopathological correlates. Cutaneous lupus erythematosus covers a wide morphological spectrum well beyond acute, subacute and chronic cutaneous lupus erythematosus, which are commonly classified as lupus-specific skin disease. Other uncommon or less well-known manifestations include lupus erythematosus tumidus, lupus profundus, chilblain lupus, mucosal lupus erythematosus, and bullous lupus erythematosus. Vascular manifestations include leukocytoclastic and urticarial vasculitis, livedoid vasculopathy and livedo reticularis/ racemosa. Finally, we discuss rare presentations such as lupus erythematosus-related erythema exsudativum multiforme (Rowell syndrome), Kikuchi-Fujimoto disease, extravascular necrotizing palisaded granulomatous dermatitis (Winkelmann granuloma), and neutrophilic urticarial dermatosis.

Background-Reliable and validated measures of skin disease severity are needed for cutaneous dermatomyositis (DM). CDASI (Cutaneous Dermatomyositis Disease Area and Severity Index), DSSI (Dermatomyositis Skin Severity Index), and CAT (Cutaneous Assessment Tool) skin indices have been developed as outcome instruments.

The Lupus Foundation of America (LFA) convened an international working group to obtain a consensus definition of disease flare in lupus. With help from the Paediatric Rheumatology International Trials Organization (PRINTO), two web-based Delphi surveys of physicians were conducted. Subsequently, the LFA held a second consensus conference followed by a third Delphi survey to reach a community-wide agreement for flare definition. Sixty-nine of the 120 (57.5%) polled physicians responded to the first survey. Fifty-nine of the responses were available to draft 12 preliminary statements, which were circulated in the second survey. Eighty-seven of 118 (74%) physicians completed the second survey, with an agreement of 70% for 9/12 (75%) statements. During the second conference, three alternative flare definitions were consolidated and sent back to the international community. One hundred and sixteen of 146 (79.5%) responded, with agreement by 71/116 (61%) for the following definition: "A flare is a measurable increase in disease activity in one or more organ systems involving new or worse clinical signs and symptoms and/or laboratory measurements. It must be considered clinically significant by the assessor and usually there would be at least consideration of a change or an increase in treatment." The LFA proposes this definition for lupus flare on the basis of its high face validity.