Annular lupus vulgaris: an unusual case undiagnosed for five years

Tuberculosis is still a serious problem in both developing and developed countries. It is often confused with various cutaneous disorders both clinically and histopathologically.A 46-year-old woman attended our clinic with progressive, asymptomatic, annular skin lesions on her right upper extremity for 5 years. She had received many different therapies for these lesions at other institutions previously but these medications were not effective and the lesions deteriorated. On dermatological examination, well-demarcated, irregular bordered, violaceous colored, elevated and crusted annular lesions on her right hand dorsum and forearm were observed. She was diagnosed as having lupus vulgaris clinically and histopathologically. Antituberculosis therapy was administered and regression of the lesions started in the second week of medication.We report a case of long-standing, undiagnosed and uncommon, annular form of lupus vulgaris. We want to stress that clinical and histopathological findings are still important for the diagnosis of cutaneous tuberculosis.     

Neonatal Lupus Erythematosus in an Infant with Turner Syndrome

Abstract: Neonatal lupus erythematosus (NLE) is characterized by transient, annular cutaneous lesions, congenital heart block, and a variety of systemic or hematologic abnormalities. We describe a white infant girt with onset of skin lesions on the face and scalp at 4 days of age. At age 4 weeks she had generalized, erythematous, scaly, annular skin lesions that underwent spontaneous regression at age 5 months. Her mother had no cutaneous or other lesions, but complement examinations revealed the presence of anti-Ro(SSA) and anti-La(SSB) antibodies, and absence of anti-Sm and anti-RNP antibodies. Karyotyping revealed Turner syndrome (TS) with 45,XO sex chromosome constitution. Ro(SSA) and La(SSB) antibodies were found, and direct immunofluorescence testing on healthy skin was positive. At age 5 months, follow-up immunologic examination of the infant had normal results but the mother still had anti-Ro(SSA) and anti-La(SSB) antibodies. We believe that this is the first reported case of NLE in association with TS.     

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.     

Clinical course and prognosis of cutaneous lupus erythematosus

Classical variants of specific cutaneous LE lesions are chronic discoid LE (CDLE) and subacute cutaneous LE (SCLE). CDLE and SCLE may appear at any age; however, the most common age of onset is between 20 and 40 years, with a female predominance of 3:1 in CDLE and 3-6:1 in SCLE. Nonspecific LE skin lesions such as generalized or acrolocalized vasculitis (4-30%), livedo reticularis (22-35%), and alopecia (38-78%) are frequently seen in patients with cutaneous LE. Other typical cutaneous LE subsets such as LE profundus/panniculitis, LE tumidus, urticaria vasculitis, hypertrophic LE, and bullous LE are rather rare variants. Butterfly rash and/or macular exanthema are characteristic skin lesions of systemic lupus erythematosus (SLE) rarely found in patients with cutaneous LE.     

Skin lesions associated with circulating lupus anticoagulant

This retrospective study of 295 patients extends the earlier findings of an association between lupus anticoagulation and thrombosis by demonstrating the occurrence of cutaneous lesions related to the coagulation defect in 70 patients. The most frequent cutaneous associations were thrombophlebitis, skin ulcer, gangrene, haemorrhage, and cutaneous necrosis. Of the patients with skin lesions, 41% had the skin lesion as the first sign of the disease. It is important to recognize this association because nearly 40% of the patients with skin lesions have multisystem thrombotic phenomena in the course of the disease.     

Neonatal lupus erythematosus

We present a case of neonatal lupus erythematosus (NLE) in a black infant presenting with symmetrical depigmented macules on the face resembling vitiligo. NLE is a rare condition affecting newborn infants of mothers who have connective tissue disease, with or without autoantibodies to extractable nuclear antigens Ro (SS-A), La (SS-B) or ribonucleoproteins. Infants present with cutaneous lesions or congenital heart block or both. The skin lesions are usually annular and erythematous and transient and resemble those of subacute cutaneous lupus erythematosus. The presentation of this patient was therefore striking.     

Subacute cutaneous lupus erythematosus

A large part of James N. Gilliam's abbreviated investigative career was devoted to testing a hypothesis that strong relationships do exist between the cutaneous and systemic manifestations of lupus erythematosus (LE). As a result of clinical observations made during his early studies designed to test this hypothesis, he introduced the term “subacute cutaneous lupus erythematosus” (SCLE) to designate a clinically distinctive nonscarring type of histologically confirmed cutaneous LE that he felt might represent a cutaneous marker for a discrete subset of LE patients.¹ A series of studies carried out in our and other laboratories have since confirmed that patients who develop SCLE skin lesions do indeed share other clinical, pathologic, serologic, and immunogenetic features. Dr. Gilliam died on June 6, 1984, before the full impact of his initial clinical observations had been fully recognized. I would, therefore, like to dedicate the following discussion of the clinical and laboratory features of patients with SCLE skin lesions to his memory.     

Ulerythema ophryogenes in Cornelia de Lange syndrome

A 17-year-old woman with Cornelia de Lange syndrome had asymptomatic skin lesions since the age of 4 years. These were multiple, follicular, horny papules, present on both cheeks, and surrounded by erythematous skin. Similar lesions were present on the external aspect of the arms, but amidst skin of normal coloration. Keratosis pilaris atrophicans faciei (ulerythema ophryogenes) has been described as a cutaneous marker for several congenital syndromes. To our knowledge, its possible association with Cornelia de Lange syndrome has never been reported.     

Partial lipodystrophy in a patient with systemic lupus erythematosus

A 54-year-old woman developed partial lipodystrophy on the left side of her face. She had been suffering from systemic lupus erythematosus (SLE) since 1985 when she was 45 years old, and she had been treated with 30 mg/day of oral prednisolone as an initial dose. Partial lipodystrophy appeared on her left lower jaw in 1994 when the SLE was inactive, and the dose of prednisolone was reduced to 5 mg/ 3 days. Gradually, the lipodystrophy spread toward her left cheek and her left forehead without any preceding skin symptoms. Histological examination showed a loss of fat tissue and mild lymphocytic infiltrations mainly around cutaneous appendages and vessels in the dermis and subcutaneous tissue. The dose of prednisolone was increased to 10 mg/day and the lesions stopped spreading. Such partial lipodystrophy is distinct from lipoatrophy of lupus profundus.     

Subacute cutaneous lupus erythematosus on the lines of Blaschko

We describe a 42-year-old woman who had erythemateous plaques with sharply demarcated margins and fine scaling on her right trunk and leg. The lesions had a linear distribution following the lines of Blaschko. Histological findings supported the diagnosis of subacute cutaneous lupus erythematosus (SCLE) on the lines of Blaschko. Treatment with systemic corticosteroids and anti-malarial agents resulted in remission. Up to now, eighteen cases of linear lupus erythematosus have been described. Linear lupus erythematosus mostly affects children presenting with lesions on the scalp that were classified as discoid lupus erythematosus. To our knowledge, this is the first case of subacute cutaneous lupus erythematosus on the lines of Blaschko.     

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.     

Cutaneous neonatal lupus erythematosus with unusual features

A three month-old boy was brought by his mother with complaints of multiple reddish lesions on his trunk and face since birth. The patient had erythematous annular plaques with scaling on his extremities, palms and soles with periorbital erythema and edema giving the characteristic "eye mask" or "owl's eye" appearance. His mother did not have history of any illness. Hemogram, liver and renal function tests were within normal limits. A skin biopsy was suggestive of subacute cutaneous lupus erythematosus. Immunological work-up was positive for antinuclear antibodies (ANA) (1:40) with anti-Ro titers of 3.4 and 3.47 (>1.1 = clinically significant titre) in the mother and child respectively, although negative for anti-La antibodies. The child's electrocardiogram and 2D echocardiography were normal. We are presenting a case of anti-Ro-positive cutaneous lupus erythematosus with an uncommon skin manifestation.     

Anetodermic lupus panniculitis and antiphospholipid antibodies: report of three cases

Anetoderma is a rare cutaneous disease characterized by a loss of normal elastic tissue that is presented clinically as localized areas of wrinkled or flaccid skin. This form may be associated with several immunological abnormalities, most notably lupus erythematosus and antiphospholipid antibodies with or without clinical manifestations of the antiphospholipid syndrome. A retrospective study was conducted with the aim of summarizing the clinical characteristics, course and laboratory findings in three women with anetoderma-associated lupus erythematosus panniculitis, an unusual variant of cutaneous lupus erythematosus. The 3 patients (of the 12 patients with lupus erythematosus panniculitis seen by us since 1990) were all at a young age at onset of panniculitis (median, 22 years). None of the patients developed severe systemic involvement up to 9 years (median, 5 years) from onset of the disease. The most noteworthy laboratory finding was the presence of antiphospholipid antibodies. Anetodermic lupus erythematosus panniculitis may be regarded as an uncommon variant of cutaneous lupus erythematosus mainly affecting young females and showing a favourable clinical course, although the patients should be followed and screened for the emergence of antiphospholipid syndrome. Antiphospholipid antibodies could play a role in the elastolytic process, leading to anetoderma.     

Necrobiosis-lipoidica-like skin manifestation in lymphomatoid granulomatosis (Liebow)

A 58-year-old man developed 3 indurative erythematous lesions like necrobiosis lipoidica on the right lower leg. He had had similar cutaneous lesions 1.5 years previously. These had been surgically excised, and a histologic diagnosis of necrobiosis lipoidica was made at another hospital. He was diagnosed as having lymphomatoid granulomatosis by lung biopsy in our hospital. Nasal involvement was confirmed in later examinations and the skin lesions were also considered to be the cutaneous manifestation of lymphomatoid granulomatosis. This outlines that cutaneous manifestations may allow early diagnosis of lymphomatoid granulomatosis.     

Subacute cutaneous lupus erythematosus: report of a patient who subsequently developed a meningioma and whose skin lesions were treated with isotretinoin

Cancer has been reported in patients with systemic lupus erythematosus (SLE). A possible association of the development of hematologic malignancies in patients with SLE has been suggested. In some patients, subacute cutaneous lupus erythematosus, a distinct subset of lupus erythematosus, has appeared, resolved, or both as a solid tumor-related paraneoplastic syndrome. A woman in whom a meningioma was diagnosed 44 years following the onset of subacute cutaneous lupus erythematosus is described; her skin lesions improved after starting isotretinoin therapy. The relationship between lupus erythematosus and neoplasia is summarized and the management of subacute cutaneous lupus erythematosus with retinoids is reviewed.     

Papulo-erythematous eruption of neonatal lupus

INTRODUCTION: Neonatal lupus is rare and cutaneous lesions are usually suggestive of the diagnosis. We describe the case an infant with atypical clinical and histological aspects. CASE REPORT: A female newborn, 6 weeks of life, presented since 3 weeks a papulo-erythematous eruption involving the trunk and the 4 limbs. Cutaneous biopsy showed a dermal infiltrate of mononuclear cells, which corresponded morphologically to lymphocytes. But myelo-monocytic origin was proved by immunostaining. This result could be seen in hematodermia and macrophagic activation syndrome, but we had no clinical sign for these diagnosis. Later, she presented an erythema of the eyelids and erythematous papules of the face leading to suspicion of neonatal lupus. Questioning the mother revealed that she had Raynaud's syndrome since 1995. Antinuclear factors were positive in the patient and her mother, corresponding to antiSSA and antiSSB antibodies. She exhibited cytolytic hepatitis but no congenital heart block. Direct immunofluorescence was negative. The clinical evolution was good with complete clearing of the eruption at the age of 3 months. At 10 months, clinical and biological examinations were normal, with no arguments for haematological disease. DISCUSSION: This observation is original because of the initial papulo-erythematous eruption which is rare in neonatal lupus. Diagnosis was confirmed when specific secondary cutaneous lesions appeared. The biopsy of the first eruption showed a myelo-monocytic infiltrate which has never been described in neonatal lupus. However cutaneous biopsies are rarely performed in neonatal lupus and immunostaining is lacking in the literature.     

Evaluation of the Japanese-Chinese Herbal Medicine,Kampo, for the Treatment of Lupus Dermatoses in Autoimmune Prone MRL/Mp-lpr/lpr Mice

Kampo, a Japanese-Chinese traditional herbal medicine, has been used for the treatment of various diseases for about 3,000 years in China. Among herbal medicines, Sairei-to is well known for improving the symptoms of rheumatoid arthritis (RA) and other collagen diseases. However, its immunosuppressive effects on autoimmune cutaneous phenomena are not completely understood. We investigated the effects of Sairei-to on the development of lupus dermatoses in autoimmune-prone MRL/Mp-lpr/lpr (MRL/lpr) mice, an animal model which spontaneously develops skin lesions similar to those seen in human lupus erythematosus. Virgin female MRL/lpr mice at 1 month of age, which were treated orally with Sairei-to, had reduced amounts of IgG deposition at the dermoepidermal junction, titers of anti-DNA antibodies and rheumatoid factor, and lymphoproliferation. These results support the use of traditional herbal medicines in patients with human RA and systemic lupus erythematosus.     

Linear cutaneous lupus erythematosus following the lines of Blaschko

We describe two Japanese girls with discoid lupus erythematosus (DLE) in whom the condition showed a linear configuration following the lines of Blaschko. The clinical appearance was unusual but histological examination established the diagnosis. After reviewing the previous reports, we found that in six of eight patients with linear 'discoid' lesions, the age at onset was under 14 years; no patient has progressed to systemic lupus erythematosus. Patients with 'linear' DLE may compose a certain clinical subset. We propose the term 'linear cutaneous lupus erythematosus' which may be more suitable for the linear lesions of DLE.     

Lupus erythematodes

The typical clinical forms of cutaneous lupus erythematosus (LE) are the butterfly rash, acute, subacute and chronic cutaneous lupus, intermediate lupus (lupus tumidus), chilblain- and bullous lupus, lupus profundus, and ulcerating lesions on the mucous membrane. Besides the typical lupus forms, nonspecific skin lesions are also observed such as dermal mucinosis, acneiform skin lesions, different variants of livedo, necrotizing vasculitis with ulcers, purpura, urticaria vasculitis, neutrophilic dermatosis, hyperpigmentation, hair and nail changes as well as overlap syndromes with erythema multiforme, scleroderma, Sjögren syndrome, Raynaud phenomenon, lichen planus, bullous pemphigoid und psoriasis. There are lupus imitators which create differential diagnostic challenges, such as infections with atypical mycobacteria or subcutaneous T-cell lymphoma both of which are similar to lupus profundus. All these skin lesions can present as maximal pathological findings seen in lupus or be caused by a variety of pathological laboratory findings such as the anti-phospholipid antibodies or a deficiency of complement factors. In the latter situation severe lupus often with complications can be expected.     

Tumid lupus erythematosus

Tumid lupus erythematosus (TLE) is a variant of cutaneous lupus erythematosus. Most patients who present with these skin lesions are young women. The condition clinically resembles polymorphous light eruption, systemic lupus erythematosus (SLE), reticulated erythematous mucinosis, or gyrate erythema. Histopathologically, the lesions resemble classic lupus erythematosus because of their superficial and deep lymphohistiocytic inflammatory infiltrates and dermal mucin. However, unlike classic lupus erythematosus, there is little or no epidermal or dermo-epidermal involvement. Antinuclear antibody test results are usually negative. We describe 4 cases of TLE and discuss the differential diagnosis.