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Antimalarials are usually recommended for the first-line systemic treatment of cutaneous lupus erythematosus. Alopecia in patients with discoid lupus erythematosus (DLE) is sometimes a refractory condition in spite of topical therapies. We herein described a case of DLE on the scalp with a pathological change of a xanthomatous reaction, which was successfully treated with hydroxychloroquine (HCQ). A 34-year-old woman presented with hair loss to the parietal region. She had been diagnosed with systemic lupus erythematosus (SLE) four years previously. Treatment with 30 mg/day of prednisolone (PSL) had been initiated, and the dose was gradually reduced. At 10 mg/day of PSL, she had noticed her hair loss. Physical examination revealed some small erythematous lesions to the parietal region with accompanying hair loss. Pathological findings of the erythematous lesion on her head revealed thickening of the basement membrane zone, the interface dermatitis with vacuolar degeneration, and both superficial perivascular and perifollicular infiltration of inflammatory cells in the dermis. In addition, there was an infiltrate of xanthomatous cells detected in the papillary dermis, which were positive for CD68 and CD163. The patient started treatment with HCQ at a dose of 200 mg/day. The skin lesions completely resolved within five months after initiation of HCQ without increase in the dose of PSL. Xanthomatous reactions are rarely recognized in lupus erythematosus. The chronic epithelial injury in DLE could be implicated in triggering the secondary reactive process of a xanthomatous reaction. We believe that the reaction seen in our patient was a secondary change to pathological alteration due to SLE. However, as yet unrecognized factors may play a role in the development of a xanthomatous reaction in DLE. [ABSTRACT FROM AUTHOR]

Summary: Background: Myositis‐specific autoantibodies (MSAs) are associated with unique clinical subsets in polymyositis/dermatomyositis (PM/DM). Autoantibodies against transcriptional intermediary factor (TIF)‐1γ and TIF‐1α are known to be MSAs. Previously, we reported that TIF‐1β is also targeted in patients with DM with or without concomitant anti‐TIF‐1α/γ antibodies. Objectives: To evaluate the clinical features of seven cases with anti‐TIF‐1β antibodies alone. Methods: Serum autoantibody profiles were determined, and protein and RNA immunoprecipitation studies were conducted. Western blotting was performed to confirm autoantibody reactivity against TIF‐1β. Results: Anti‐TIF‐1β antibody was identified by immunoprecipitation assay in 24 cases. Among them, seven patients were positive for anti‐TIF‐1β antibody alone. Six of the seven patients were classified as having DM. Among the six cases of DM, two patients had no muscle weakness and normal creatine kinase (CK) levels, and were classified as having clinically amyopathic DM. Four patients had muscle weakness, but three of them had normal serum CK levels that responded well to systemic steroids. Characteristic features of DM included skin rashes, such as Gottron sign, periungual erythema, punctate haemorrhage on the perionychium and facial erythema including heliotrope, which were observed in 86%, 57%, 86% and 71% of our cases, respectively. One of the seven patients had appendiceal cancer. None of the patients had interstitial lung disease. Conclusions: Seven patients were confirmed to have anti‐TIF‐1β antibody without any other MSAs, including TIF‐1α/γ antibodies, and six of them were diagnosed with DM. We suggest that anti‐TIF‐1β antibody is an MSA, and that it is associated with clinically amyopathic DM or DM with mild myopathy. What's already known about this topic? Previously we reported that transcriptional intermediary factor (TIF)‐1β is also targeted in patients with dermatomyositis with or without concomitant anti‐TIF‐1α/γ antibodies.Anti‐TIF‐1β antibody could be a myositis‐specific autoantibody. What does this study add? We describe seven patients with anti‐TIF‐1β antibody but without anti‐TIF‐1α/γ reactivity.Anti‐TIF‐1β antibody is possibly associated with clinically amyopathic dermatomyositis or dermatomyositis with mild myopathy. Linked Comment:Fiorentino. Br J Dermatol 2019; 180:709–710. Respond to this article Plain language summary available online [ABSTRACT FROM AUTHOR]

Background Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a commonly occurring condition related to systemic autoimmune disease. It is characterized histopathologically by a distinct pattern of granulomatous inflammation in the presence or absence of leukocytoclastic vasculitis. The properties of granulomatous cells in PNGD are still uncertain. Objective We sought further investigation on the phenotype of the infiltrated cells in PNGD from two patients with systemic lupus erythematosus (SLE) and reviewed the previous published reports in order to provide a comprehensive summary on the clinical features of PNGD in SLE. Methods The immunohistochemical features of granulomatous cells in PNGD associated with SLE were analyzed. Immunohistochemical studies were performed on sections from our two cases using antibodies against CD68, CD163, CD15, Factor XIIIa, myeloperoxidase and neutrophil elastase. The clinical characteristics of the SLE patients who developed PNGD were also evaluated. We included all cases retrieved through a PubMed search with the key words PNGD and SLE. Results Cutaneous lesions consisted of erythematous plaques distributed on the face and upper limbs in both cases. The infiltrated cells were mainly positive for CD68 and CD163, a phenotype suggestive of M2 macrophages. Some mature neutrophils and lymphocytes were also present. A review of the literature of PNGD associated with SLE revealed a predominance in females, high prevalence of lupus nephritis and a good response to systemic steroids, with frequent skin lesions relapses during tapering of the treatment. Limitations This study examined only two cases; the pathogenesis of the disease remains to be clarified. Conclusion PNGD lesions were abundantly infiltrated by M2 macrophages, suggesting that they may have a role in this condition. SLE accompanied by PNGD might be associated with lupus nephritis and frequent relapses of skin lesions. [ABSTRACT FROM AUTHOR]

The article presents case studies of a 56-year-old man, 62-year-old man, and 36-year-old man with skin diseases. Topics discussed include the presence of anti-Mi-2 antibody in patients depicting as a marker for dermatomyositis (DM), need of repeated testing for monitoring the disease, and disease severity.

Linked Article: Ueda‐Hayakawa et al. Br J Dermatol 2019; 180:881–887 [ABSTRACT FROM AUTHOR]

Summary: Dermatomyositis is a disease where skin and muscles become inflamed. Sometimes it affects just muscles, sometimes just the skin. Most patients fall within a spectrum where both are inflamed. Elevations of the enzyme creatinine kinase (CK) are found in the bloodstream if muscles are inflamed, and tests such as an electromyogram may also be abnormal. Dermatomyositis is an autoimmune condition, with antibodies directed against the patient's own tissues (autoantibodies). Different patterns of such autoantibodies are associated with different subsets of the condition. There is also an underlying cancer in a minority of adults with dermatomyositis. One group of autoantibodies is specifically associated with muscle inflammation. Within it is a family called anti‐TIF (transcriptional intermediary factor) antibodies, including anti‐TIF‐1α and ‐1γ antibodies. Anti‐TIF‐1α antibodies always appear with anti‐TIF‐1γ. In this paper from Japan, the authors investigate which conditions result when patients have a third autoantibody, anti‐TIF‐1β, on its own, without either of the other two. From 3442 stored serum (blood) samples from patients with connective tissue diseases, they found seven such patients. Six had dermatomyositis, but in two, muscles were either not involved (one patient) or involved but producing no symptoms (one patient); the other four patients had classic dermatomyositis but muscle involvement was mild and only one of the four had an elevated CK. One of these four patients did, however, have an underlying cancer. The seventh patient had 'undifferentiated connective tissue disease'. The authors conclude that anti‐TIF‐1β antibodies are associated with dermatomyositis where muscle inflammation is absent or only mild. Linked Article: Ueda‐Hayakawa et al. Br J Dermatol 2019; 180:881–887 [ABSTRACT FROM AUTHOR]