Cutaneous lupus erythematosus: a personal approach to management

SUMMARY Skin disease in patients with lupus erythematosus may be subdivided into two broad categories - those lesions that when biopsied demonstrate interface dermatitis and those that do not demonstrate interface dermatitis. The skin lesions that are represented by the interface dermatitis include discoid lupus erythematosus, subacute cutaneous lupus erythematosus and acute cutaneous lupus erythematosus. Patients with these 'specific' manifestations have varying degrees of systemic involvement from rare systemic disease in patients with localized discoid lupus erythematosus to common and often severe involvement in patients with acute cutaneous lupus erythematosus. Patients who do not demonstrate interface dermatitis also may have systemic disease and in some instances the skin manifestations are linked to some of the more severe systemic manifestations. Many patients with cutaneous lesions characterized by the interface dermatitis can be controlled with 'standard' therapies including sunscreens, protective clothing and behavioural alteration, and topical corticosteroids with or without an oral antimalarial agent. This review presents a brief summary of each common cutaneous manifestation of lupus erythematosus, its relationship to systemic involvement and treatment issues to effectively deal with the lupus erythematosus patient who has skin disease.     

Cicatricial alopecia: discoid lupus erythematosus

ABSTRACT:   Chronic cutaneous lupus erythematosus consists of three major dermatologic diseases: discoid lupus erythematosus (DLE), lupus panniculitis/lupus profundus, and lupus tumidus ( Table 1 ). DLE is estimated to be responsible for 50–85% of patients with chronic cutaneous lupus erythematosus. Scalp involvement is most often the presenting symptom. The clinical features and diagnosis of DLE, its pathophysiology and treatment, are reviewed.  

  Table 1.  Chronic cutaneous lupus erythematosus     

3.

Vitiligo associated with malignant melanoma and lupus erythematosus     

Gül U  Kiliç A  Tulunay O  Kaygusuz G 《The Journal of dermatology》2007,34(2):142-145

There exists several reports where malignant melanoma is associated with vitiligo, vitiligo with discoid lupus erythematosus and lupus erythematosus with urticaria. However, there are no reports in which vitiligo, malignant melanoma, lupus erythematosus and urticaria coexist in the same case. Herein, we report a case of a patient who developed lupus erythematosus, malignant melanoma, vitiligo and urticaria simultaneously.     

4.

系统性红斑狼疮的治疗进展   总被引：5，自引：1，他引：4  

周培媚  路永红 《中国皮肤性病学杂志》2009,23(6)

系统性红斑狼疮的治疗以激素和免疫抑制剂为基础。近年来,随着各相关学科的发展,新型免疫抑制剂、生物制剂、干细胞移植等相继用于治疗系统性红斑狼疮,并取得了显著疗效。此外,近年来的研究,对抗疟药、维生素D、他克莫司等在系统性红斑狼疮中的治疗作用也有了更深入的认识。本文就该方面的进展作一综述。     

5.

Cyclosporin combined with methotrexate in two patients with recalcitrant subacute cutaneous lupus erythematosus     

Klein A  Vogt T  Wenzel SM  Fleck M  Landthaler M 《The Australasian journal of dermatology》2011,52(1):43-47

The treatment of subacute cutaneous lupus erythematosus is a therapeutic challenge. Frequently, patients are resistant to or intolerant towards single selected immunosuppressants. In combination, lower doses of methotrexate and cyclosporin A might be used to minimize toxicity. This is the first report of a successful combination of these drugs in two patients suffering from subacute cutaneous lupus erythematosus refractory to standard treatment.     

6.

Blaschkoid lichen planus occurring in childhood systemic lupus erythematosus     

Abheek Sil MBBS  Sayantani Chakraborty MBBS  MD  Avik Panigrahi MBBS  Satarupa Mondal MBBS 《Pediatric dermatology》2020,37(3):579-581

Lupus erythematosus-lichen planus (LE-LP) overlap syndrome represents a rare disorder with clinical and histopathological features of both lichen planus and lupus erythematosus presenting in the same lesions. However, lichen planus and lupus erythematosus can also coexist in the same patient. Blaschkoid lichen planus in the setting of concurrent childhood-onset systemic lupus erythematosus has not been previously described.     

7.

Dramatic effect of hydroxychloroquine on lupus alopecia          下载免费PDF全文

Shohei Igari  Toshiyuki Yamamoto 《The Journal of dermatology》2018,45(2):194-197

Lupus alopecia is usually difficult to treat. We report a case of a 40‐year‐old woman with Sjögren's syndrome and atopic dermatitis who presented with discoid lupus erythematosus on the forearms and lupus erythematosus profundus with alopecia involving the scalp. A biopsy specimen taken from the discoid lupus erythematosus lesion on the forearm further exhibited a xanthomatous reaction, which however was not detected in another specimen from the lupus erythematosus profundus on the scalp. Treatment with oral hydroxychloroquine showed dramatic effects and complete hair regrowth was obtained 3 months later.     

8.

Lupus erythematosus profundus--report of a case treated with dapsone     

Y Yamada  S Dekio  J Jidoi  S Ozasa 《The Journal of dermatology》1989,16(5):379-382

A male adult patient with discoid lupus erythematosus (DLE) developed lupus erythematosus profundus. We decided to treat him with oral administration of dapsone, which proved to be very effective.     

9.

Intravenous immunoglobulin G use in patients with chronic inflammatory demyelinating polyneuropathy: An uncommon cause of drug-induced discoid lupus erythematosus     

Tara Jennings  Sarah Ahmed  Abhishek Aphale  Jason Lee  Rodrigo Valdes-Rodriguez 《The Australasian journal of dermatology》2019,60(3):e208-e210

Drug-induced chronic cutaneous lupus erythematosus, or drug-induced discoid lupus erythematosus, is a rare cutaneous phenomenon. Various medications have been associated with drug-induced discoid lupus erythematosus including fluorouracile agents, especially tegafur and uraciltegafur, and TNF-α antagonists such as infliximab or etanercept. Recent literature has described a case series of six patients receiving IgG immunoglobulin for chronic inflammatory demyelinating polyneuropathy with subsequent presentations of discoid lupus erythematosus. We present a patient with chronic inflammatory demyelinating polyneuropathy who developed discoid lupus erythematosus secondary to IgG immunoglobulin.     

10.

Lupus erythematosus vegetans     

Khorshid SM  Beynon HL  Rustin MH 《The British journal of dermatology》1999,141(5):893-896

A 29-year-old woman with known systemic lupus erythematosus presented with exudative and vegetative plaques bilaterally on her groins. The clinical and histological findings indicated a diagnosis of pyodermatitis vegetans. Direct immunofluorescence studies revealed the presence of a definite basement membrane zone band for IgG, IgM and C3, favouring lupus erythematosus. We propose the term lupus erythematosus vegetans for this combination of clinicopathological and immunohistological findings.     

11.

Lupus panniculitis clinically simulating alopecia areata     

Kossard S 《The Australasian journal of dermatology》2002,43(3):221-223

A 27-year-old woman with a known history of lupus erythematosus presented with two circumscribed patches of non-scarring alopecia closely resembling alopecia areata. Scalp biopsy showed a predominantly subcutaneous and deep dermal lymphocytic infiltrate that surrounded the deep follicular segments and hair bulbs, as well as the eccrine glands. There was associated hyaline fat sclerosis. The epidermis, infundibular and isthmus segments of follicles were relatively spared and lacked the lichenoid inflammation and fibrosis seen with lupus erythematosus. The biopsy findings illustrate that the deep variant of lupus panniculitis may be concentrated around the hair bulbs and deep temporary segments of hair follicles and spare the permanent stem cell-rich follicular segments. This pattern is capable of producing a temporary hair-loss, clinically simulating alopecia areata. The clinical history, presence of subtle erythema and scalp tenderness on physical examination, as well as the biopsy findings, were important clues in distinguishing our case from a true combination of alopecia areata and lupus erythematosus.     

12.

线状皮肤型红斑狼疮   总被引：2，自引：0，他引：2  

廖文俊  樊平申  付萌  王雷  高天文 《临床皮肤科杂志》2010,39(2)

目的:分析线状皮肤型红斑狼疮的临床表现、组织病理及免疫组化组织病理特点.方法:采用回顾性分析方法对7例线状皮肤型红斑狼绝患者的临床表现、组织病理改变、免疫荧光及实验窀检查资料进行分析.结果:7例患者中男2例,女5例;平均年龄23岁(4～40岁);平均发病年龄19.43岁(3～37岁),其中2例发病年龄<6岁.皮损部位:1例位于左下肢,其余6例均位于头面部.皮损均呈线状分布,其走向与Blasehko线一致;所有患者均无系统受累的证据.结合组织病理和免疫荧光检查,6例诊断为线状盘状红斑狼疮,1例为线状深在性红斑狼疮.结论:线状皮肤型红斑狼疮是红斑狼疮的罕见变异型,不引起系统受累.临床诊断有一定困难,需借助于组织病理和免疫荧光检查.     

13.

Photosensitivity in lupus erythematosus     

Scheinfeld N  Deleo VA 《Photodermatology, photoimmunology & photomedicine》2004,20(5):272-279

BACKGROUND: Lupus erythematosus is a systemic disease process that may manifest with a variety of internal and cutaneous findings. Photosensitivity is one the most common manifestations of lupus erythematosus. In patients with lupus erythematosus, there is a relationship between exposure to ultraviolet light, autoantibodies, genetics and other factors in the development of photosensitivity. METHODS: Literature was reviewed on the topics of lupus erythematosus and photosensitivity discussed together and separately. The suggested mechanisms for their relationship were reviewed and analyzed. RESULTS: Photosensitivity's relationship to and influence on the systemic manifestations of lupus remain to be defined. Mechanisms for photosensitivity might include: modulation of autoantibody location, cytotoxic effects, apoptosis induction with autoantigens in apoptotic blebs, upregulation of adhesion molecules and cytokines, induction of nitric oxide synthase expression and ultraviolet-generated antigenic DNA. Tumor necrosis factor alpha also seems to play a role in the development of photosensitivity. CONCLUSION: The basis for photosensitivity in lupus has yet to be fully defined. It is more commonly associated with subacute and tumid lupus erythematosus than with other variants. Anti-Ro antibodies appear to relate to photosensitivity. Tumor necrosis factor alpha polymorphisms appear to be important in some variants of lupus with photosensitivity. There is no sine qua non antibody or mutation of photosensitivity in lupus. In patients with lupus, more work needs to be done to define the mechanisms of photosensitivity.     

14.

Facets of lupus erythematosus: panniculitis responding to thalidomide     

Simone Wienert  Stephan Gadola  Thomas Hunziker 《Journal der Deutschen Dermatologischen Gesellschaft》2008,6(3):214-216

Lupus erythematosus profundus or lupus panniculitis is a rare clinical variant of lupus erythematosus, which involves the deep dermis and subcutaneous fat. Diagnosis may be difficult in cases with isolated involvement. Further manifestations of lupus erythematosus may thus be essential for diagnosis, which depends on the clinical picture, histopathology and a positive lesional lupus band test. We report a severe, mutilating case of lupus panniculitis, which responded well to thalidomide.     

15.

Topical niacinamide (Nicotinamide) treatment for discoid lupus erythematosus (DLE): A prospective pilot study     

Ahmed H. Nouh MD  Ahmed R. Elshahid MD  Ahmed S. Kadah MD  Youssef A. Zeyada MbBch 《Journal of Cosmetic Dermatology》2023,22(5):1647-1657

Background

Cutaneous lupus erythematosus is an umbrella term for a group of autoimmune connective tissue disorders affecting the skin. Discoid lupus erythematosus (DLE) is the chronic condition and most common form of cutaneous lupus erythematosus.

Aims

Current therapies of DLE are challenging and not completely satisfactory, highly expensive, off-label, or poorly available (like antimalarials due to COVID-19 outbreaks). Nicotinamide, also called niacinamide, is a water-soluble form of vitamin B3 (niacin). Its multiple effects let us think that nicotinamide could be a therapy for lupus-associated skin lesions.

Methods

We performed a prospective randomized double-blind clinical trial on 60 subjects diagnosed with Discoid lupus erythematosus using topical Nicotinamide 2% and 4% preparations in form of cream and gel on skin and scalp lesions. Control group was included using only cream/gel base as placebo control.

Results

Obtained data showed that topical Nicotinamide can be used for the treatment of DLE as adjuvant to other treatment regimens with good cosmetic results and minimal side effects. Topical 4% Nicotinamide is superior to 2% preparation in response but associated with a higher incidence of irritation.

Conclusion

Topical Nicotinamide can be used for the treatment of DLE as an adjuvant to other treatment regimens with good cosmetic results and minimal side effects. Further trials with long-term therapy, follow-up period, and bigger sample sizes are required.     

16.

Hypertrophic lupus erythematosus treated successfully with acitretin as monotherapy   总被引：1，自引：0，他引：1  

Al-Mutairi N  Rijhwani M  Nour-Eldin O 《The Journal of dermatology》2005,32(6):482-486

Hypertrophic lupus erythematosus (HLE) is a distinct and rare subset of chronic cutaneous lupus erythematosus characterized by verrucous lesions which are chronic in course and resistant to treatment (1). We describe the successful use of acitretin in a patient with HLE who had multiple hyperkeratotic verrucous plaques over the dorsa of his hands, feet, and legs and who failed to respond to local steroids and antimalarials.     

17.

Cutaneous lupus erythematosus and its association with systemic lupus erythematosus: A nationwide population-based cohort study in Korea     

Yoo Sang Baek  Se Ha Park  Jinok Baek  Joo Young Roh  Hee Joo Kim 《The Journal of dermatology》2020,47(2):163-165

Although lupus erythematosus is known to be more common among women of color, the study populations in previous reports were predominantly Caucasian and there is scarce information on Asian patients. Therefore, we performed a retrospective study using a nationwide population-based cohort in South Korea. The average annual incidence of cutaneous lupus was 4.36/100 000. Among 634 patients with cutaneous lupus, 20.8% had systemic disease: cutaneous lupus was diagnosed before systemic lupus in 4.26% and after systemic lupus in 8.52%. More female patients than male patients developed systemic lupus erythematosus. The average time to progression to systemic lupus was 1.53 ± 1.46 years.     

18.

Experience with the use of hydroxychloroquine for the treatment of lupus erythematosus     

Yoko Momose  Satoru Arai  Hikaru Eto  Mitsumasa Kishimoto  Masato Okada 《The Journal of dermatology》2013,40(2):94-97

Hydroxychloroquine (HCQ) is generally used to treat systemic lupus erythematosus (SLE) in Western countries. However, chloroquine retinopathy became a problem in Japan, and chloroquine has never been used since then. Even now HCQ remains non‐approved. Therefore, the Japanese Hydroxychloroquine Study Group has been organized, and activities have started to have HCQ approved within Japan. In the present study, we investigated the effectiveness of HCQ against the skin manifestations of lupus erythematosus. There were seven patients, all female, and they consisted of four patients with SLE (skin lesion type: discoid lupus erythematosus [DLE] in three, subacute cutaneous lupus erythematosus in one and lupus erythematosus profundus in one), two patients with cutaneous lupus erythematosus (both DLE), and one patient with a combination of SLE and dermatomyositis. HCQ was effective in three patients and ineffective in the two patients. We could not judge the efficacy of HCQ in the other two patients. There were no adverse effects in any of the patients. Efficacy was exhibited against telangiectasia and erythema. HCQ is also an effective and safe treatment for Japanese patients, and it is hoped that it will be approved for use in Japan very soon.     

19.

Recent advances in cytokines in cutaneous and systemic lupus erythematosus     

Naoya MIKITA  Takaharu IKEDA  Mariko ISHIGURO  Fukumi FURUKAWA 《The Journal of dermatology》2011,38(9):839-849

Lupus erythematosus (LE) includes a broad spectrum of diseases from a cutaneous‐limited type to a systemic type. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which affects multiple organs. Cutaneous lupus erythematosus (CLE) includes skin symptoms seen in SLE and cutaneous‐limited LE. Although immune abnormalities, as well as heritable, hormonal and environmental factors, are involved in the pathology of LE, the actual pathogenesis is still unclear. Recently, the involvement of various cytokines has been shown in the pathogenesis of LE. Moreover, some trials with biological agents targeted specific cytokines are also ongoing for SLE. In this article, we review the contributions of major cytokines such as interferon, tumor necrosis factor‐α and interleukin‐18 to LE, especially SLE and CLE.     

20.

A case of edematous striae distensae in lupus nephritis     

Lee JH  Lee EK  Kim CW  Kim TY 《The Journal of dermatology》1999,26(2):122-124

A 17-year-old girl with systemic lupus erythematosus presented with painful edematous abdominal striae. She had been treated with systemic steroid for the systemic lupus erythematosus. At the time of presentation, she had abruptly gained 10 kg due to combined lupus nephritis. The histopathologic finding of the edematous striae distensae included dermal edema with separation of collagen fibers and small fragmented elastic fibers. Edematous striae distensae are uncommon but can develop from the combined effects of glucocorticoid and generalized edema.     

Vitiligo associated with malignant melanoma and lupus erythematosus

There exists several reports where malignant melanoma is associated with vitiligo, vitiligo with discoid lupus erythematosus and lupus erythematosus with urticaria. However, there are no reports in which vitiligo, malignant melanoma, lupus erythematosus and urticaria coexist in the same case. Herein, we report a case of a patient who developed lupus erythematosus, malignant melanoma, vitiligo and urticaria simultaneously.     

系统性红斑狼疮的治疗进展

系统性红斑狼疮的治疗以激素和免疫抑制剂为基础。近年来,随着各相关学科的发展,新型免疫抑制剂、生物制剂、干细胞移植等相继用于治疗系统性红斑狼疮,并取得了显著疗效。此外,近年来的研究,对抗疟药、维生素D、他克莫司等在系统性红斑狼疮中的治疗作用也有了更深入的认识。本文就该方面的进展作一综述。     

Cyclosporin combined with methotrexate in two patients with recalcitrant subacute cutaneous lupus erythematosus

The treatment of subacute cutaneous lupus erythematosus is a therapeutic challenge. Frequently, patients are resistant to or intolerant towards single selected immunosuppressants. In combination, lower doses of methotrexate and cyclosporin A might be used to minimize toxicity. This is the first report of a successful combination of these drugs in two patients suffering from subacute cutaneous lupus erythematosus refractory to standard treatment.     

Blaschkoid lichen planus occurring in childhood systemic lupus erythematosus

Lupus erythematosus-lichen planus (LE-LP) overlap syndrome represents a rare disorder with clinical and histopathological features of both lichen planus and lupus erythematosus presenting in the same lesions. However, lichen planus and lupus erythematosus can also coexist in the same patient. Blaschkoid lichen planus in the setting of concurrent childhood-onset systemic lupus erythematosus has not been previously described.     

Dramatic effect of hydroxychloroquine on lupus alopecia

Lupus alopecia is usually difficult to treat. We report a case of a 40‐year‐old woman with Sjögren's syndrome and atopic dermatitis who presented with discoid lupus erythematosus on the forearms and lupus erythematosus profundus with alopecia involving the scalp. A biopsy specimen taken from the discoid lupus erythematosus lesion on the forearm further exhibited a xanthomatous reaction, which however was not detected in another specimen from the lupus erythematosus profundus on the scalp. Treatment with oral hydroxychloroquine showed dramatic effects and complete hair regrowth was obtained 3 months later.     

Lupus erythematosus profundus--report of a case treated with dapsone

A male adult patient with discoid lupus erythematosus (DLE) developed lupus erythematosus profundus. We decided to treat him with oral administration of dapsone, which proved to be very effective.     

Intravenous immunoglobulin G use in patients with chronic inflammatory demyelinating polyneuropathy: An uncommon cause of drug-induced discoid lupus erythematosus

Drug-induced chronic cutaneous lupus erythematosus, or drug-induced discoid lupus erythematosus, is a rare cutaneous phenomenon. Various medications have been associated with drug-induced discoid lupus erythematosus including fluorouracile agents, especially tegafur and uraciltegafur, and TNF-α antagonists such as infliximab or etanercept. Recent literature has described a case series of six patients receiving IgG immunoglobulin for chronic inflammatory demyelinating polyneuropathy with subsequent presentations of discoid lupus erythematosus. We present a patient with chronic inflammatory demyelinating polyneuropathy who developed discoid lupus erythematosus secondary to IgG immunoglobulin.     

Lupus erythematosus vegetans

A 29-year-old woman with known systemic lupus erythematosus presented with exudative and vegetative plaques bilaterally on her groins. The clinical and histological findings indicated a diagnosis of pyodermatitis vegetans. Direct immunofluorescence studies revealed the presence of a definite basement membrane zone band for IgG, IgM and C3, favouring lupus erythematosus. We propose the term lupus erythematosus vegetans for this combination of clinicopathological and immunohistological findings.     

Lupus panniculitis clinically simulating alopecia areata

A 27-year-old woman with a known history of lupus erythematosus presented with two circumscribed patches of non-scarring alopecia closely resembling alopecia areata. Scalp biopsy showed a predominantly subcutaneous and deep dermal lymphocytic infiltrate that surrounded the deep follicular segments and hair bulbs, as well as the eccrine glands. There was associated hyaline fat sclerosis. The epidermis, infundibular and isthmus segments of follicles were relatively spared and lacked the lichenoid inflammation and fibrosis seen with lupus erythematosus. The biopsy findings illustrate that the deep variant of lupus panniculitis may be concentrated around the hair bulbs and deep temporary segments of hair follicles and spare the permanent stem cell-rich follicular segments. This pattern is capable of producing a temporary hair-loss, clinically simulating alopecia areata. The clinical history, presence of subtle erythema and scalp tenderness on physical examination, as well as the biopsy findings, were important clues in distinguishing our case from a true combination of alopecia areata and lupus erythematosus.     

线状皮肤型红斑狼疮

目的:分析线状皮肤型红斑狼疮的临床表现、组织病理及免疫组化组织病理特点.方法:采用回顾性分析方法对7例线状皮肤型红斑狼绝患者的临床表现、组织病理改变、免疫荧光及实验窀检查资料进行分析.结果:7例患者中男2例,女5例;平均年龄23岁(4～40岁);平均发病年龄19.43岁(3～37岁),其中2例发病年龄<6岁.皮损部位:1例位于左下肢,其余6例均位于头面部.皮损均呈线状分布,其走向与Blasehko线一致;所有患者均无系统受累的证据.结合组织病理和免疫荧光检查,6例诊断为线状盘状红斑狼疮,1例为线状深在性红斑狼疮.结论:线状皮肤型红斑狼疮是红斑狼疮的罕见变异型,不引起系统受累.临床诊断有一定困难,需借助于组织病理和免疫荧光检查.     

Photosensitivity in lupus erythematosus

BACKGROUND: Lupus erythematosus is a systemic disease process that may manifest with a variety of internal and cutaneous findings. Photosensitivity is one the most common manifestations of lupus erythematosus. In patients with lupus erythematosus, there is a relationship between exposure to ultraviolet light, autoantibodies, genetics and other factors in the development of photosensitivity. METHODS: Literature was reviewed on the topics of lupus erythematosus and photosensitivity discussed together and separately. The suggested mechanisms for their relationship were reviewed and analyzed. RESULTS: Photosensitivity's relationship to and influence on the systemic manifestations of lupus remain to be defined. Mechanisms for photosensitivity might include: modulation of autoantibody location, cytotoxic effects, apoptosis induction with autoantigens in apoptotic blebs, upregulation of adhesion molecules and cytokines, induction of nitric oxide synthase expression and ultraviolet-generated antigenic DNA. Tumor necrosis factor alpha also seems to play a role in the development of photosensitivity. CONCLUSION: The basis for photosensitivity in lupus has yet to be fully defined. It is more commonly associated with subacute and tumid lupus erythematosus than with other variants. Anti-Ro antibodies appear to relate to photosensitivity. Tumor necrosis factor alpha polymorphisms appear to be important in some variants of lupus with photosensitivity. There is no sine qua non antibody or mutation of photosensitivity in lupus. In patients with lupus, more work needs to be done to define the mechanisms of photosensitivity.     

Facets of lupus erythematosus: panniculitis responding to thalidomide

Lupus erythematosus profundus or lupus panniculitis is a rare clinical variant of lupus erythematosus, which involves the deep dermis and subcutaneous fat. Diagnosis may be difficult in cases with isolated involvement. Further manifestations of lupus erythematosus may thus be essential for diagnosis, which depends on the clinical picture, histopathology and a positive lesional lupus band test. We report a severe, mutilating case of lupus panniculitis, which responded well to thalidomide.     

Topical niacinamide (Nicotinamide) treatment for discoid lupus erythematosus (DLE): A prospective pilot study

Background

Cutaneous lupus erythematosus is an umbrella term for a group of autoimmune connective tissue disorders affecting the skin. Discoid lupus erythematosus (DLE) is the chronic condition and most common form of cutaneous lupus erythematosus.

Aims

Current therapies of DLE are challenging and not completely satisfactory, highly expensive, off-label, or poorly available (like antimalarials due to COVID-19 outbreaks). Nicotinamide, also called niacinamide, is a water-soluble form of vitamin B3 (niacin). Its multiple effects let us think that nicotinamide could be a therapy for lupus-associated skin lesions.

Methods

We performed a prospective randomized double-blind clinical trial on 60 subjects diagnosed with Discoid lupus erythematosus using topical Nicotinamide 2% and 4% preparations in form of cream and gel on skin and scalp lesions. Control group was included using only cream/gel base as placebo control.

Results

Obtained data showed that topical Nicotinamide can be used for the treatment of DLE as adjuvant to other treatment regimens with good cosmetic results and minimal side effects. Topical 4% Nicotinamide is superior to 2% preparation in response but associated with a higher incidence of irritation.

Conclusion

Topical Nicotinamide can be used for the treatment of DLE as an adjuvant to other treatment regimens with good cosmetic results and minimal side effects. Further trials with long-term therapy, follow-up period, and bigger sample sizes are required.     

Hypertrophic lupus erythematosus treated successfully with acitretin as monotherapy

Hypertrophic lupus erythematosus (HLE) is a distinct and rare subset of chronic cutaneous lupus erythematosus characterized by verrucous lesions which are chronic in course and resistant to treatment (1). We describe the successful use of acitretin in a patient with HLE who had multiple hyperkeratotic verrucous plaques over the dorsa of his hands, feet, and legs and who failed to respond to local steroids and antimalarials.     

Cutaneous lupus erythematosus and its association with systemic lupus erythematosus: A nationwide population-based cohort study in Korea

Although lupus erythematosus is known to be more common among women of color, the study populations in previous reports were predominantly Caucasian and there is scarce information on Asian patients. Therefore, we performed a retrospective study using a nationwide population-based cohort in South Korea. The average annual incidence of cutaneous lupus was 4.36/100 000. Among 634 patients with cutaneous lupus, 20.8% had systemic disease: cutaneous lupus was diagnosed before systemic lupus in 4.26% and after systemic lupus in 8.52%. More female patients than male patients developed systemic lupus erythematosus. The average time to progression to systemic lupus was 1.53 ± 1.46 years.     

Experience with the use of hydroxychloroquine for the treatment of lupus erythematosus

Hydroxychloroquine (HCQ) is generally used to treat systemic lupus erythematosus (SLE) in Western countries. However, chloroquine retinopathy became a problem in Japan, and chloroquine has never been used since then. Even now HCQ remains non‐approved. Therefore, the Japanese Hydroxychloroquine Study Group has been organized, and activities have started to have HCQ approved within Japan. In the present study, we investigated the effectiveness of HCQ against the skin manifestations of lupus erythematosus. There were seven patients, all female, and they consisted of four patients with SLE (skin lesion type: discoid lupus erythematosus [DLE] in three, subacute cutaneous lupus erythematosus in one and lupus erythematosus profundus in one), two patients with cutaneous lupus erythematosus (both DLE), and one patient with a combination of SLE and dermatomyositis. HCQ was effective in three patients and ineffective in the two patients. We could not judge the efficacy of HCQ in the other two patients. There were no adverse effects in any of the patients. Efficacy was exhibited against telangiectasia and erythema. HCQ is also an effective and safe treatment for Japanese patients, and it is hoped that it will be approved for use in Japan very soon.     

Recent advances in cytokines in cutaneous and systemic lupus erythematosus

Lupus erythematosus (LE) includes a broad spectrum of diseases from a cutaneous‐limited type to a systemic type. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which affects multiple organs. Cutaneous lupus erythematosus (CLE) includes skin symptoms seen in SLE and cutaneous‐limited LE. Although immune abnormalities, as well as heritable, hormonal and environmental factors, are involved in the pathology of LE, the actual pathogenesis is still unclear. Recently, the involvement of various cytokines has been shown in the pathogenesis of LE. Moreover, some trials with biological agents targeted specific cytokines are also ongoing for SLE. In this article, we review the contributions of major cytokines such as interferon, tumor necrosis factor‐α and interleukin‐18 to LE, especially SLE and CLE.     

A case of edematous striae distensae in lupus nephritis

A 17-year-old girl with systemic lupus erythematosus presented with painful edematous abdominal striae. She had been treated with systemic steroid for the systemic lupus erythematosus. At the time of presentation, she had abruptly gained 10 kg due to combined lupus nephritis. The histopathologic finding of the edematous striae distensae included dermal edema with separation of collagen fibers and small fragmented elastic fibers. Edematous striae distensae are uncommon but can develop from the combined effects of glucocorticoid and generalized edema.