The association between lupus erythematosus profundus and systemic lupus erythematosus: a single center study and literature review

( Do Ik Kwon ),( Jang Hwan Jung ),( Seol Hwa Seong ),( Kang Hoon Lee ),( Jong Bin Park ),( Kee Suck Suh ),( Min Soo Jang ) 대한피부과학회 2020 대한피부과학회 학술발표대회집 Vol.72 No.1

Background: Lupus erythematosus profundus (LEP) is a variant of chronic cutaneous lupus erythematosus and association between LEP and systemic lupus erythematosus (SLE) has been described as from 10% to 50% in the previous studies. Objectives: To investigate the association of SLE in LEP patients. Methods: This study was conducted on 32 patients diagnosed as LEP in the authors’ hospital and we reviewed previous studies using PubMed searches (Search terms included LEP, lupus erythematosus profundus, lupus erythematosus panniculitis, lupus profundus, lupus panniculitis. Studies describing 3 or more patients with LEP were included). Results: The mean age was 40.8 years (range, 17-73) and the mean follow-up duration was 72.4 months (range, 0- 162). Three patients (9.3%) were diagnosed concurrently SLE with LEP and two patients (6.3%) developed into SLE after 6 to 7 years. In the review of previous studies, 189 patients were histologically confirmed as LEP including 51 patients (26.9%) accompanied by SLE. Of them, 30 patients (15.8%) had been diagnosed as SLE before LEP, and 15 patients (7.9%) were simultaneously diagnosed as SLE by LEP. Only 5 patients (2.6%) developed into SLE during the follow-up period of LEP. Conclusion: The proportion of LEP patients associated with SLE in our center (16.7%) was lower than that of literature review (26.9%). And LEP seems to rarely progresses into the SLE.

류마티스 관절염 환자에서 Adalimumab 사용 후 발생한 심부홍반루푸스

한길 ( Gil Han ),김성애 ( Sung-ae Kim ),조재위 ( Jae-we Cho ),이규석 ( Kyu-suk Lee ) 대한피부과학회 2015 대한피부과학회지 Vol.53 No.4

The clinical manifestations and immunohistologic findings of drug-induced lupus erythematosus (DILE) are similar to those of idiopathic lupus. However, DILE is different from idiopathic lupus because it is induced after continuous drug exposure and resolves after discontinuation of the causative drug. DILE can be divided into systemic lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus (CCLE). Lupus erythematosus profundus is a subtype of CCLE, and drug-induced CCLE is very rarely reported in the literature. Herein, we report a rare case of adalimumab-induced lupus erythematosus profundus developed in a rheumatoid arthritis patient. The patient is a 43-year-old Korean woman who had multiple tender nodules and plaques on her face, trunk, and both extremities after using adalimumab for rheumatoid arthritis. She was diagnosed with adalimumab-induced lupus erythematosus profundus, and her condition improved after discontinuation of adalimumab. (Korean J Dermatol 2015;53(4):319∼323)

Vesiculobullous eruption, the first manifestation of lupus erythematosus

( Su Hyun Park ),( Ki Hwa Choi ),( Ji Yeoun Lee ),( Tae Young Yoon ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Bullous systemic lupus erythematosus is an autoantibodymediated subepidermal blistering disease that occurs in patients with systemic lupus erythematosus. In cases from the literature, the vast majority had a previous history of lupus erythematosus before making the diagnosis of bullous systemic lupus erythematosus. A 46-year-old woman presented with an extensive vesiculobullous eruption on the face, trunk, and both upper extremities for 1 month. She had no other systemic symptoms. Histologic findings showed hydropic degeneration of the basal layer, a subepidermal blister and a predominantly neutrophilic dermal infiltrate. Direct immunofluorescence microscopy for IgG revealed granular bandlike deposition along the basement membrane zone. The patient was also found to have mouth sores, anemia, significant thrombocytopenia, positive antinuclear antibody test, and positive anti-Smith antibody test, confirming the diagnosis of bullous systemic lupus erythematosus. The skin lesions resolved after administrating oral corticosteroids (methylprednisolone 60mg/day) for 2 weeks. Here, we present a case of a 46-year-old woman with bullous systemic lupus erythematosus who presented with vesiculobullous lesions as an initial manifestation of systemic lupus erythematosus.

Lupus cystitis in Korean patients with systemic lupus erythematosus: risk factors and clinical outcomes

Koh, J H,Lee, J,Jung, S M,Ju, J H,Park, S-H,Kim, H-Y,Kwok, S-K SAGE Publications 2015 Lupus Vol.24 No.12

<P>This study was performed to investigate the clinical characteristics of lupus cystitis and determine the risk factors and clinical outcomes of lupus cystitis in patients with systemic lupus erythematosus (SLE). We retrospectively reviewed 1064 patients at Seoul St. Mary’s Hospital in Seoul, Korea, from 1998 to 2013. Twenty-four patients had lupus cystitis. Lupus cystitis was defined as unexplained ureteritis and/or cystitis as detected by imaging studies, cystoscopy, or bladder histopathology without urinary microorganisms or stones. Three-fourths of patients with lupus cystitis had concurrent lupus mesenteric vasculitis (LMV). The initial symptoms were gastrointestinal in nature for most patients (79.2%). High-dose methylprednisolone was initially administered to most patients (91.7%) with lupus cystitis. Two patients (8.3%) died of urinary tract infections. Sixty-five age- and sex-matched patients with SLE who were admitted with other manifestations were included as the control group. Patients with lupus cystitis showed a lower C3 level (<I>p</I> = 0.031), higher SLE Disease Activity Index score (<I>p</I> = 0.006), and higher ESR (<I>p</I> = 0.05) upon admission; more frequently had a history of LMV prior to admission (<I>p</I> < 0.001); and less frequently had a history of neuropsychiatric lupus (<I>p</I> = 0.031) than did patients with SLE but without lupus cystitis. The occurrence of lupus cystitis was associated with a history of LMV (OR, 21.794; 95% CI, 4.061–116.963). The median follow-up period was 3.4 years, and the cumulative one-year mortality rate was 20%. Complications developed in 33.3% of patients with lupus cystitis and were related to survival (log-rank <I>p</I> = 0.021). Our results suggest that the possibility of lupus cystitis should be considered when a patient with SLE and history of LMV presents with gastrointestinal symptoms or lower urinary tract symptoms. Development of complications in patients with lupus cystitis can be fatal. Thus, intensive treatment and follow-up are needed, especially in the presence of complications.</P>

A case of lupus erythematosus panniculitis

( Hae Jeong Youn ),( Byung Gon Choi ),( Song Hee Han ),( Min Jung Kim ),( Soo Young Kim ),( Nam Kyung Roh ),( Ho Jung Jung ),( Yu Ri Kim ),( Yang Won Lee ),( Yong Beom Choe ),( Kyu Joong Ahn ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Lupus erythematosus panniculitis(LEP) is a rare variant of lupus erythematosus, which primarily affects the subcutaneous adipose tissue. LEP may appear as the sole manifestation of lupus erythematosus or may occur prior to or after the onset of discoid lupus erythematosus or systemic lupus erythematosus(SLE). The incidence of SLE in patients with LEP has been reported to range from 10% to 41%. The incidence of LEP occurs in only 1-5% of patients with SLE. Why LEP develops is still lacking. We report a case of LEP in 25 year old women who presented depressed lipoatrophic areas withoutinflammatory skin changes on the both upper arm for a year ago. Histopathology showed lobular panniculitis without vasculitis. The patient was weak positive for antinuclear antibodies, borderline positive for anti-double stranded DNA and low C4 level. Herein we report a rare case of LEP with a systemic lupus erythematosus.

개에 있어서 전신성홍반성루프스 1예와 전신성혼반성루프스 의증 1예 및 자가면역성혈소판감소성출혈성자반병 1예

이창우,나기정,임정식,서정욱 한국임상수의학회 1996 한국임상수의학회지 Vol.13 No.1

Systemic lupus erythematosus in a dog, suspected systemic lupus erythematosus in a dog, and autoimmune thrombocytopenic purpura hemmorrhagica in a dog are reported. A fice-year old, female Chihuahua (Case 1) showed initially hemorrhagic diathesis and purpura hemorrhagica. Afterward, it showed polymyositis and polyarthritis. LE-cell was demonstrated on LE-cell preparation trom blood. Systemic lupus erythematosus was diagnosed. This reponded well to the immunosuppressants, but developed iatrogenic Cushing syndrome and steroid hepatopathy. A two-and-half-year old, male toy poodle (Case 2) had chief complaint of red urine. Occult blood test for the urine sediment. This did not respond at all to antibiotics and carbazochrome, which is one of systemic coagulants. LE-cell was demonstrated on LE-cell preparation from blood. This responded relatively well to immunosupressants such as prenisolone, azathioprine and cyclophosphamide. systemic lupus erythematosus is suspected. A nine-year-and-three-month old, female Maltese (Case 3), which had history of congestive heart failure and ovariohysterectomy showed purpura hemorrhagica in the skin of chest. This had severe thrombocytopenia and leukocytosis. As prednisolone was administered before immunological examination or demonstration of LE-cell, it was impossible to diagnose whether purpura hemorrhagica developed as a member of systemic lupus erythematosus or independent of systemic lupus erythematosus. This responded well to prednisolone, and so autoimmune thrombocytopenic purpura hemorrhagica was diagnosed.

[P129] Discoid lupus erythematosus on the scalp : An unusual presentation

( Su Hyun Park ),( Myeong Heon Chae ),( Ji Yeoun Lee ),( Tae Young Yoon ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Discoid lupus erythematosus is a subtype of chronic cutaneous lupus erythematosus. Clinically, lesions of discoid lupus erythematosus consist of well-demarcated, erythematous, discoid plaques that have prominent adherent thick scale and follicular plugging that often results in scarring and atrophy. A diagnosis of discoid lupus erythematosus is possible on the basis of a combination of histologic findings. A 69-year-old woman presented with alopecic patches on the scalp for 7 months. On physical examination, there were diffuse erythematous scaly alopecic patches without atrophy on the scalp. Histologic findings revealed hyperkeratosis, irregular acanthosis, and focal mild hydropic degeneration in the epidermis. There were dense lymphohistiocytic infiltrate around hair follicles and eccrine glands, and severe hydropic degeneration along the follicular-dermal junction in the superficial and deep dermis. Based on these findings, a diagnosis of discoid lupus erythematosus was made. Herein, we report a case of discoid lupus erythematosus that shows diffuse erythematous scaly alopecic patches without atrophy on the scalp.

Clinical characteristics of pediatric cutaneous lupus erythematosus: experience from a tertiary referral center in Korea

( Shinyoung Song ),( Seha Park ),( Seulki Lee ),( Jin Ok Baek ),( Joo Young Roh ),( Hyangjoon Park ),( Hee Joo Kim ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.2

Background: The clinical characteristics of cutaneous lupus erythematosus (CLE) are well delineated in adults, but pediatric data, particularly in Asian populations, are limited. Objectives: Therefore, we evaluated the characteristics of pediatric cases by retrospectively reviewing the medical records of children with CLE during a 15-year period in a tertiary care dermatology clinic in South Korea. Methods: The study included 21 children (8 males and 13 females), 4 of whom had neonatal lupus erythematosus (NLE). Results: Among 17 patients with CLE, discoid lupus erythematosus (DLE) was most common (47.1%), followed by acute CLE (ACLE, 35.3%). All ACLE cases had systemic lupus erythematosus (SLE). Female predominance was conspicuous in ACLE/SLE (6/11 females versus 0/6 males), as was older age, whereas DLE and NLE showed near equal sex distributions. The median age at the diagnosis of CLE was significantly higher in females than in males (15 years versus 4.5 years, p=0.02). All patients with ACLE/ SLE simultaneously showed skin and systemic symptoms from onset. The kidney was the most commonly involved organ. Conclusion: This study revealed unique characteristics of pediatric CLE, further warranting a comprehensive review among various ethnicities to understand the wide spectrum of CLE in the pediatric population.

A case of tumid lupus erythematous following Kikuchi`s disease

( Joongoon Kim ),( Moonhyung You ),( Yeonwoong Kim ),( Byeongsu Kim ),( Donghoon Shin ),( Jongsoo Choi ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Tumid lupus erythematosus is a rare subtype of chronic cutaneous lupus erythematosus. Clinical lesions are characterized by raised erythematous plaques on sun-exposed areas such as the face, chest, arms and shoulders. Kikuchi’s disease is commonly associated with systemic lupus erythematosus, but rarely associated with tumid lupus erythematosus. A 36-year-old female patient presented with multiple erythematous plaques on both cheeks and back. She was diagnosed as Kikuchi’s disease by lymph node biopsy 3 month ago. ANA was negative, and histopathologic examination showed little vacuolar alteration, periadnexal, perivascular lymphocytic infiltration at dermo-epidermal junction. Mucin deposition in the reticular dermis was identified by alcian blue stain, so we diagnosed as tumid lupus erythematosus. After treatment with oral hydrochloroquine 100mg daily, the skin lesion was improved significantly. Herein, when skin lesion developed in patient with Kikuchi’s disease, we recommend skin biopsy to identify whether the skin lesion is from Kikuchi’s disease or other associated diseases.

전신홍반루푸스 치료 중 발생한 루푸스 장염

정병수 ( Byoung Soo Chung ),김민성 ( Min Sung Kim ),나찬호 ( Chan Ho Na ),신봉석 ( Bong Seok Shin ) 대한피부과학회 2009 대한피부과학회지 Vol.47 No.8

Systemic lupus erythematosus is a multisystemic autoimmune inflammatory disease with a variety of presenting features and manifestations. Gastrointestinal complications are common in patients with systemic lupus erythematosus, and lupus enteritis is a major cause of acute abdominal pain in the patients suffering with systemic lupus erythematosus. We report here on a 41-year-old-woman with systemic lupus erythematosus and who had developed an acute abdomen during treatment with systemic glucocorticoid and antimalarial drugs. Lupus enteritis was strongly suspected according to the clinical manifestations and the findings of the abdominal computerized tomography scan. She recovered after being treated with intravenous high dose glucocorticoid and conservative therapy. (Korean J Dermatol 2009;47(8):956~959)