Unusual severe radiation-induced toxicity in a patient with discoid lupus erythematosus: A case report and critical review of the literature.

Data on the incidence and severity of radiation-induced toxicity in patients with systemic and/or cutaneous lupus erythematosus (SLE/CLE) are very limited. After reporting the case of a patient who experienced major toxicity and CLE flare in the irradiated area following breast irradiation, we conducted a comprehensive literature review of available data in this setting. The few retrospectives studies which have evaluated both the risk of toxicity in SLE/CLE patients and/or the potential induction or reactivation of SLE/CLE with radiotherapy have not shown differences between SLE/CLE patients and controls. Several other factors such as concurrent chemotherapy, a particular genetic background, or lupus treatments (essentially hydroxychloroquine) can explain severe radiation-induced toxicity. Therefore, patients with SLE/CLE should be irradiated like patients without SLE/CLE, with close monitoring during radiotherapy if other risk factors exist. Further studies examining a larger number of patients would probably allow a better understanding of the radiosensitivity of these patients.

Recognition and Management of Cutaneous Connective Tissue Diseases.

Connective tissue diseases (CTDs) encompass a broad spectrum of clinical presentations that involve multidisciplinary management. Cutaneous findings are common in CTD and careful examination of these features aids in appropriate diagnosis and subsequent evaluation. Thorough work-up of CTD is crucial to properly identify disease subtypes and systemic involvement. Management plans can be developed based on diagnosis and systemic manifestations of disease. Disease management often requires treatment with pharmacotherapies with potential for toxicities, further underscoring the importance of diagnostic accuracy in this patient population. Evolving research strives to better elucidate the pathogenic mechanisms of CTDs allowing for more targeted treatment modalities.

The Koebner phenomenon on tattoos and piercings in a patient with cutaneous lupus: a case report and review of the literature.

The Koebner phenomenon is associated with cutaneous lupus erythematosus (CLE). A 20-year-old woman with a 10-year history of systemic lupus, treated with hydroxychloroquine and methotrexate, presented with features of chronic discoid lupus erythematosus (DLE) on the scalp, at the site of ear piercings, and on the temporal bone at the site of trauma from her jewelry. She also had subacute CLE (SCLE) lesions on old black tattoos. Histology and direct immunofluorescence confirmed CLE. We reviewed 13 cases of Koebner phenomenon on tattoos in patients with CLE (seven men, median age: 31.5 years) and none after piercings. Lesions developed within 1 week to 16 years after tattooing. Lesions may be isolated, precede, or be associated with other CLE lesions. They can appear secondarily on the tattoo. There is no specific color affinity, but cases have shifted from red to black, possibly when mercury was withdrawn from red inks. CLE on tattoos is a rare phenomenon that more often presents with DLE features than SCLE. Patients should be warned of the potential risk of developing lesions on tattoos. Immunosuppressive treatment needs to be taken into account if a patient wishes to get a tattoo. However, tattooing is not associated with severe complications.

Cutaneous lupus erythematosus: factors related to cutaneous activity and damage in a cohort of 260 patients from A Coruña, Spain.

BACKGROUND: Cutaneous lupus erythematosus is a chronic autoimmune disease that can leave important sequelae. OBJECTIVE: To determine the factors that predict the activity and damage of the skin disease, and the impact of tobacco on the efficacy of antimalarials using the Cutaneous Lupus Erythematosus Disease Area and Severity Index. MATERIALS AND METHODS: A consecutive case series was performed on 260 patients with cutaneous lupus erythematosus (α = 0.05; precision ± 6.5%). We carried out a descriptive analysis of the variables included, with a multivariate analysis to measure the association of variables with the Cutaneous Lupus Erythematosus Disease Area and Severity Index activity and damage (p value < 0.05). RESULTS: The Cutaneous Lupus Erythematosus Disease Area and Severity Index activity was greater in smokers than non-smokers (4.0 ±5.3 vs 1.2 ±3.4, p = 0.006). No significant differences were observed in the Cutaneous Lupus Erythematosus Disease Area and Severity Index activity when the efficacy of antimalarials was analyzed between smokers and non-smokers. Cutaneous Lupus Erythematosus Disease Area and Severity Index damage was higher in smokers than in non-smokers (2.0 ± 3.6 vs 1.2 ± 2.6, p = 0.029). Cutaneous Lupus Erythematosus Disease Area and Severity Index activity was associated with: (a) being an active smoker (odds ratio 3.04, 95% confidence interval 1.68-5.51, p < 0.001; regression coefficient 2.05, 95% confidence interval 0.69-3.42, p = 0.003); (b) the chronic cutaneous lupus erythematosus subtype (odds ratio 1.98, 95% confidence interval 1.02-3.84, p = 0.044); and (c) C-reactive protein increase (≥0.5 mg/dL) (regression coefficient 2.56, 95% confidence interval 0.40-4.71, p = 0.020). Cutaneous Lupus Erythematosus Disease Area and Severity Index damage was associated with: (a) the activity (regression coefficient 0.11, 95% confidence interval 0.01-0.20, p = 0.024); (b) the chronic cutaneous lupus erythematosus subtype (regression coefficient 2.46, 95% confidence interval 1.37-3.56, p < 0.001); (c) the use of topical treatment (regression coefficient 1.31, 95% confidence interval 0.01-2.61, p = 0.049); and (d) systemic treatment (regression coefficient 1.44, 95% confidence interval 0.35-2.53, p < 0.010). CONCLUSION: Smoking is related to an increase risk and a greater activity of cutaneous lupus erythematosus. The chronic cutaneous lupus erythematosus subtype and an increased C-reactive protein level were also associated with a higher disease activity. The sequelae were related to the activity, the chronic cutaneous lupus erythematosus subtype, and the use of topical and systemic treatment. The impact of tobacco on the efficacy of antimalarials may be caused by an increase in the severity of the disease more than by resistance in smokers.

Isoradiotopic response of discoid lupus after radiotherapy: A case report and review of the literature.

Radiotherapy is frequently associated with a great number of collateral effects, which can affect the skin and its appendages. In addition to more common side effects, like radiodermatitis, other cutaneous conditions are less known and often they are underdiagnosed. Among these, isoradiotopic response is one of the rare radiotherapy-associated phenomena. This term refers to the appearance of a secondary dermatosis in a previously irradiated district. The term was used for the first time by Shurman et al. to describe a case of lichen ruber planus arising in the genital area after radiotherapy for squamous cell carcinoma. The pathologic mechanism is not completely clear, but a few hypotheses have been proposed. Alterations in the local lymphatic drainage, in the nervous system and the immune microenvironment have all been called into play (the immunocompromised district theory). We present the case of a male patient that developed discoid lupus on a previously irradiated cutaneous area and review the literature, highlighting the numerous possible manifestations of this phenomenon.

Koebner phenomenon of discoid lupus erythematosus on old tattoos.

The Koebner phenomenon is the development of lesions in traumatized skin, often with spontaneously occurring lesions elsewhere. We report a case of a woman in her 20 s presenting with discoid lesions superimposed on tattoos that she obtained many years ago. Although a few case reports have been published describing development of lesions on red tattoos attributed to photosensitivity, or koebnerization occurring less than a month following tattoo placement, our patient demonstrates a unique delayed development of discoid lesions years after obtaining her tattoos in non-sun exposed skin. Patients with conditions associated with the Koebner phenomenon must be counseled regarding forms of preventable trauma such as tattoos, and should be made aware that the Koebner phenomenon may not only manifest immediately, but can also present many years later.

Generalized Discoid Lupus Erythematosus as the Presenting Sign of Small Cell Lung Carcinoma.

A 46-year-old woman with a 30 pack-year smoking history presented with a worsening eruption on the left cheek that failed to improve with metronidazole gel. The cutaneous eruption spread to most of her face and did not respond to a brief tapering course of prednisone. During the initial evaluation at our institution, approximately 6 weeks after the onset of the cutaneous eruption, the patient had erythematous, crusted plaques on her face and scalp (Figure 1A); they were also present on the V-area of the anterior aspect of the neck and upper region of the chest, the shoulders, and the arms, with isolated lesions on the trunk and legs. Her oral mucosa had erythematous erosions on the hard palate and gingivae. A review of systems revealed pain and burning of her skin lesions, but no muscle weakness or other systemic clinical manifestations. The differential diagnosis included autoimmune connective tissue disease, pemphigus foliaceous, sarcoidosis, lichen planus, phototoxic drug eruption, and eczema herpeticum.

Anti-SSA antibodies are present in immunoglobulin preparations.

BACKGROUND: Anti-SSA autoantibodies are among the most frequently detected autoantibodies and have traditionally been associated with Sjögren's syndrome (SjS) and systemic lupus erythematosus. The unexpected finding of anti-SSA antibodies in a patient with common variable immunodeficiency disorder (CVID) treated with intravenous immunoglobulin (IVIG), who developed discoid lupus erythematosus, prompted us to investigate the presence of anti-SSA antibodies in IVIG preparations. Since anti-SSA antibodies may be present in apparently healthy individuals without overt autoimmune features, IVIG preparations may also contain anti-SSA antibodies. STUDY DESIGN AND METHODS: IVIG consists of polyclonal immunoglobulin G isolated from the plasma of more than 1000 blood donors. Several IVIG batches from different suppliers and serum samples of patients receiving these IVIG products were tested for the presence of anti-nuclear antibodies (ANAs) and extractable nuclear antibodies (ENAs). In addition, we tested several plasma pools for the presence of anti-SSA and subsequent serum samples of individual donors. RESULTS: Several CVID-patients receiving IVIG tested positive for ANA and anti-SSA. The IVIG products administered also contained clearly detectable concentrations of these antibodies. The frequency of apparently healthy blood donors with anti-SSA positivity was 0.69% and one of 1894 donors (0.05%) showed a very high titer of anti-SSA of more than 10,000 U/mL. CONCLUSION: Anti-SSA is present in IVIG products and in blood donors without clinical symptoms. IVIG replacement can interfere with ANA and ENA serology by passive transfer of autoantibodies. We hypothesize that such autoantibodies may be causally related to disease manifestations in some recipients.

Discoid lupus erythematosus-related squamous cell carcinoma of the lip in an HIV-seropositive black male.

Discoid lupus erythematosus (DLE) is an autoimmune disease commonly affecting sun-exposed areas of the skin. Subjects with DLE have high-levels of plasmacytoid dendritic cells -derived interferon-α, which mediates both loss of immune tolerance to self-antigens and exaggerated inflammatory state, and supports proliferation and differentiation of hyperactive B-cells. In a few cases, DLE of the lips, scalp, ears or nose may eventually progress to squamous cell carcinoma (SCC). Photosensitivity and the long-standing immune-mediated chronic inflammation and dysregulated healing characterized by atrophy, hypopigmentation or scarring inherent to DLE are risk factors for progression to SCC. We review some aspects of the pathogenesis of DLE and the possible roles of inflammation and photosensitivity in the carcinomatous transformation of DLE keratinocytes, and present an illustrative case of DLE of the lower lip in an HIV-tuberculosis co-infected black person, that progressed to SCC.

Drug-induced lupus erythematosus.

Drug-induced lupus erythematosus (DI-LE) is defined as an entity characterized by clinical manifestations and immunopathological serum findings similar to those of idiopathic lupus but which is temporally related to drug exposure and resolves after withdrawal of the implicated drug. Similarly to idiopathic lupus, DI-LE can be divided into systemic LE, subacute cutaneous LE (SCLE), chronic cutaneous LE (CCLE) and cutaneous LE tumidus. DI-SCLE is the most frequent variant of drug-induced cutaneous LE and presents mainly with annular-polycyclic lesions; the clinical picture is often widespread, with involvement of the lower legs that are usually spared in idiopathic SCLE. ANA and anti-Ro/SSA antibodies are typically present, whereas antihistone antibodies are uncommonly found. We have recently addressed the question whether DI-SCLE differs significantly from its idiopathic counterpart by virtue of clinical features and, based on our findings, we have suggested that the frequent occurrence of malar rash and bullous, erythema multiforme-like and vasculitic manifestations can be regarded as the hallmark of DI-SCLE. In contrast, the histology is not a useful diagnostic criterion for DI-SCLE, considering that the typical pattern of lichenoid interface dermatitis is seen only in the early stage of disease and tissue eosinophilia does not represent a differentiating histopathological feature. DI-CCLE and DI-LE tumidus, albeit possibly misdiagnosed, are rarely observed and are characterized by classic discoid lesions and erythematous-oedematous plaques on sun exposed areas, respectively. Management of DI-LE is based on the discontinuation of the offending drug; topical and/or systemic corticosteroids and other immunomodulating/immunosuppressive agents should be reserved for resistant cases.

Genital discoid lupus: a rare manifestation of cutaneous lupus erythematosus.

Cutaneous lupus erythematosus classically presents as sharply demarcated plaques with pigmentary changes and varying degrees of scarring typically affecting the face and scalp. Genital manifestations of lupus erythematosus have rarely been described in literature. Here we report two cases of discoid lupus erythematosus affecting the genital area. Both patients were equally affected by widespread discoid lesions on the head. Although rare, genital involvement of lupus erythematosus must be considered in the differential diagnosis of genital lesions.

Discoid lupus erythematosus at a site of previous injury.

A 71-year-old man with three patches of discoid lupus erythematosus (DLE) confined to the right preauricular region drew our attention because of the unusual linear arrangement of the lesions. Twenty-five years previously, the patient had suffered a trauma in the same area from falling off his motorcycle. We believe that, despite the great lapse in time, this injury may have facilitated the onset of DLE in the very same area, through long-term destabilization of the local neuroimmune network. The case fits the recently coined concept of the immunocompromised district, a cutaneous region with altered immune control, more susceptible to harbouring opportunistic infections, tumours, and immune disorders.

The triggering role of allergic contact dermatitis in discoid lupus erythematosus.

BACKGROUND: The onset and exacerbations of discoid lupus erythematosus (DLE) can be precipitated by several factors like needling, scratches, trauma, X-rays, heat, cold, pressure, tattooing, scars, allergic and irritant dermatitis and inflammatory dermatoses. OBJECTIVE: To investigate the role of allergic contact dermatitis (ACD) in devolopment and triggering of the lesions of DLE. MATERIALS AND METHODS: This study was performed on 30 patients with DLE. European baseline series and cosmetic patch test series were used. At least 1+ reaction was accepted as meaningful. RESULTS: Twenty-three (76.7%) of 30 DLE patients and 16 (40%) of 40 control group patients were allergic to at least one allergen on standard patch test series. The difference between the groups were found statistically significant. Seventeen (56.7%) of 30 DLE patients and 6 (15%) of 40 control group patients were allergic to at least one allergen on cosmetic patch test series. The difference between the groups were statistically significant. The most sensitized allergens in both the groups were nickel sulphate, paraphenylen diamine, potassium dichromate from standard patch test series; quaternium 15, cocamidopropyl betain from cosmetic patch test series, in order. CONCLUSION: This study is distinctive since it is the first study to determine the eliciting role of ACD on DLE by imposing standard and cosmetic patch test series on DLE and control group patients. Worldwide, there is no study based on this subject. In the DLE group, the results of sensitization on standard and cosmetic patch test series were higher and statistically significant. Larger studies are required to reveal the exact role.

Koebner's phenomenon in systemic lupus erythematosus.

Koebner phenomenon is defined as a nonspecific skin stimulus eliciting a disease skin reaction. The nature of the skin trauma varies greatly and includes areas of thermal injury, excoriations, surgical incisions, and scars. We report a patient with recent onset of systemic lupus erythematosus who developed Herpes zoster on immunosuppressant medication. Two weeks after resolution of the vesicles, the patient presented with new ulcerative reddish lesions over the herpes zoster scare and worsening of her malar rash without evidence of worsening of any other organ. Koebner phenomenon was suspected. We review the literature on Koebner phenomenon in SLE.

Lúpus eritematoso crônico: uma abordagem clínica, epidemiológica, laboratorial e terapêutica / Chronic lupus erythematosus: a clinical approach, epidemiological, laboratory and therapeutic

O lúpus eritematoso cutâneo crônico, caracterizado, sobretudo, pelo lúpus discoide, é uma entidade clínica incomum, porém de elevada prevalência em mulheres em idade fértil. A sua etiologia é desconhecida, mas fatores genéticos, autoimunes, hormonais e ambientais compõem o processo fisiopatológico da doença. Os meios diagnósticos utilizados para que se possa iniciar o tratamento específico, composto de protetores solares, corticosteroides tópicos e, se preciso, medicações sistêmicas, principalmente os antimaláricos, são o exame clínico, a imunofluorescência direta e o estudo histopatológico. Este artigo descreve de maneira sucinta os principais aspectos epidemiológicos, clínicos, diagnósticos e terapêuticos do lúpus eritematoso cutâneo crônico, conforme revisão de literatura.

Ethnic and geographical differences in systemic lupus erythematosus: an overview.

Systemic lupus erythematosus (SLE) is one of the most heterogeneous autoimmune disorders known. There is production of a variety of autoantibodies and patients present with a wide range of symptoms due to multiple organ involvement by the disease process. The underlying cause is not fully understood but it may involve genetic and environmental factors. It is interesting to note that while SLE is found worldwide, it is more commonly found in some countries, and within a country certain ethnic groups appear to be more susceptible to develop this condition than others. Additionally, the presentation and course of SLE appear highly variable between patients of different ethnic origins. For example, African-Americans and Orientals are believed to have a more severe disease than Caucasian whites. But are these ethnic and geographical differences real? If yes, they may provide investigators insight into the underlying pathoaetiology of this condition and pave the way to future research directions in lupus.