Hypertrophic lupus erythematosus: a clinicopathological study of 14 cases

BACKGROUND: Hypertrophic lupus erythematosus (HLE) is a distinct and rare subset of lupus erythematosus (LE). It is characterized by verrucous lesions, chronic in its course, and resistant to treatment. The purpose of this study was to identify clinical and histological characteristics of HLE. METHODS: We review our experience with 14 cases of HLE identified in a group of 220 patients with different forms of LE, at the UNICAMP Hospital, between 1976 and 2002. RESULTS: All patients presented verrucous plaques concomitantly with discoid lesions. The most common sites of involvement were the face and the arms. Histology of HLE lesions revealed pseudoepitheliomatous hyperplasia engulfing elastotic material. Elastic fibers were seen in migration throughout the epidermis. Classic features of LE were noted in all cases. Three of the patients developed hyperkeratotic papules with central keratinous plug on their arms at the previous LE sites. These lesions resemble clinically and histopathologically keratoacanthomas. In one patient, HLE lesion progressed to squamous cell carcinoma (SCC), 26 years after the onset of the disease. CONCLUSIONS: Transepithelial elimination of the elastotic material may be a feature of HLE. Some HLE lesions may present as keratoacanthoma, but classical features of LE aid the correct diagnosis. SCC may arise on a long-standing HLE lesion; therefore HLE requires clinical and histopathological follow up.     

Hypertrophic discoid lupus erythematosus

Hypertrophic discoid lupus erythematosus is a distinct form of chronic cutaneous (discoid) lupus, which is characterized by hyperkeratotic plaques that typically are observed over the face, arms, and upper trunk. We present the case of a 43-year-old man with verrucous plaques that were distributed symmetrically over the face, who initially was treated with oral antibiotics and topical glucocorticoids for acne vulgaris. A biopsy specimen confirmed the diagnosis of hypertrophic discoid lupus erythematosus. The clinical and histopathologic features of this clinical variant are reviewed.     

Verrucous lupus erythematosus: Ultrastructural studies on a distinct variant of chronic discoid lupus erythematosus

Ten patients with classic discoid lupus erythematosus of the face associated with verrucous, papulonodular lesions on the arms and hands were studied by electron microscopy. The ultrastructural findings on the verrucous lesions included apoptotic keratinocytes, intraepidermal lymphocytes, and gapping, detachment, and reduplication of the basal lamina. Also, tubuloreticular inclusion bodies were present in the endothelial cells. These observations, together with the clinical, histopathologic, and immunofluorescence findings, suggest that the verrucous lesions represent a rare, but distinct, variant of chronic discoid lupus erythematosus.     

HYPERTROPHIC LUPUS ERYTHEMATOSUS: DIFFERENTIATION FROM SQUAMOUS CELL CARCINOMA

Hypertrophic lupus erythematosus (HLE) may be more common as a variant of discoid lupus erythematosus (DLE) than is reported in the literature. Differentiation from neoplastic transformation of a lesion of DLE to squamous cell carcinoma (SCC) is crucial because of the metastatic potential of this type of SCC. A man is described in whom hypertrophic lesions of LE accompanying lesions of classic DLE were interpreted histopathologically as SCC. The problem of differentiating true HLE from SCC arising within DLE is discussed with a review of the relevant literature.     

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.     

Chronic lupus erythematosus presenting as acneiform lesions

BACKGROUND: Cutaneous manifestations of lupus erythematosus are numerous but usually permit easy diagnosis. However, there are atypical lesions that can mimic benign dermatologic disorders. We report on a patient with lesions of acne leading to the diagnosis of chronic lupus erythematosus, and who subsequently developed systemic lupus erythematosus. CASE REPORT: A 30-year-old woman presented with inflammatory lesions and comedos on the face. The eruption started after her last pregnancy and was refractory to local and general treatment. She also complained of arthralgia, Raynaud's phenomenon and diffuse alopecia. Cutaneous biopsy was characteristic of chronic lupus erythematosus. Immunofluorescence microscopy of lesional skin showed a lupus band deposit. Antinuclear antibodies were highly positive. The patient was successfully treated with chloroquine. Three years later, the patient presented with photodistributed eruption. Antinuclear antibodies were still positive and in addition anticardiolipin antibodies were found. Final diagnosis was systemic lupus erythematosus. DISCUSSION: Acneiform lesions are rarely reported in lupus erythematosus. Only three similar cases were reported in literature. Atypical and treatment-resistant eruptions should attract attention. Furthermore, the occurrence of systemic lupus in chronic lupus erythematosus is not an unfrequent phenomenon and the oestrogen-dependance of chronic lupus lesions may predict this association.     

疣状盘状红斑狼疮1例

患者女，６２岁，病期２６年。躯干上部、面及上肢疣状盘状斑块、刺状角栓，组织病理象为表皮呈疣状增生及胶样小体。病程慢性，无特效疗法。     

Amyloid deposition is frequently observed in skin lesions of hypertrophic lupus erythematosus

Four cases of Hypertrophic Lupus Erythematosus (HLE) were reported. The lesions of HLE were observed on the forearms, face and hands in all four cases. Clinically, the lesions were erythematous, hyperkeratotic plaques. The clinical course was marked by chronicity and progression of the lesion. Histologically, marked hyperkeratosis, parakeratosis, acanthosis, degenerative changes of basal cells in H/E stain, and thickened, multilayered basement membrane in PAS stain, were observed. The observations of Dylon stain revealed that localized amyloid deposition was observed in all four cases of HLE lesions, as fluorescent-orange colored amyloid deposits in the papillary dermis and subepidermal areas at near orjust below the dermo-epidermal junction appeared under fluorescent microscope. On the basis of clinical and histological observations, we suggest that chronic irritation, such as sunlight exposure over a long-duration, might have caused the characteristic abnormalities at the dermo-epidermal junction and also initiated the frequency of amyloid deposits locally secondary to the diseases. We compared our HLE cases to other types of lupus erythematosus (LE) skin lesions, as to whether deposition of amyloid materials were frequently observed or not. Amyloid deposition was observed in one case of DLE and none of the SLE cases. Localized amyloid deposition was more frequently observed in skin lesions, secondary to HLE disease, as compared to other types of LE.     

Spinocellular cancer at the site of chronic discoid lupus erythematosus

A case of squamous cell carcinoma is presented that developed within a tense, fibrotic scar caused by chronic discoid lupus erythematosus. The carcinoma appeared in a woman of 60 years of age who has had chronic discoid lupus erythematosus on the scalp for 30 years. According to the literature, the frequency of this type of malignant transformation is estimated to be within the same range as the incidence of squamous cell carcinomas arising within tense, fibrotic scars, that is ranging between 0.1% and 2.6%. Therefore, no additional carcinoma-promoting factors of different origin specific for chronic discoid lupus erythematosus could be found.     

Lupus erythematosus with perforation of the pinna

Cutaneous inflammation occurred in a 56-year-old woman who had chronic discoid lupus erythematosus. Lesions on the pinna resulted in perforation. This finding has not been reported previously in association with lupus erythematosus.     

Lupus erythematosus panniculitis presenting as palpebral edema and parotiditis

Lupus erythematosus panniculitis or lupus erythematosus profundus is characterized by inflammation of the deep dermis and subcutaneous tissue. It can occur in isolation or associated with chronic systemic or discoid lupus erythematosus. It usually consists of nodules and hardened subcutaneous plaques on the forehead, cheeks, proximal extremities, and buttocks. Periorbital and parotid involvement are rare and can lead to misdiagnosis. We present the case of a patient with lupus erythematosus panniculitis who presented with palpebral edema and involvement of the periocular fat and parotid gland.     

Mixed lichen planus-lupus erythematosus disease: a distinct entity? Clinical,histopathological and immunopathological studies in six patients

This report describes six patients with Features of both chronic discoid lupus erythematosus (DIE) and lichen planus (LP). All patients showed chronic, livid, partly atrophic LE-like lesions, preferentially located on the head, the face and the upper trunk. Reticular, whitish patches were found in the oral mucosa of five patients. In addition, papulo-nodular, verrucous lesions and lichenoid papules were present in some of these cases. The histopathological and immunopathological findings in these lesions are discussed in detail. It is concluded that some of our patients have a coexistence of both diseases while others suffer from an unusual variant of CDLE. We do not support the suggestion that this ‘mixed LP-LE disease’ should be considered as a distinct entity.     

Discoid lupus erythematosus in the Nigerians

Results of a detailed clinical and laboratory study of 37 Nigerian patients with chronic discoid lupus erythematosus are presented. Patients with chronic discoid lupus erythematosus constituted 0.46% of all out-patients seen in the skin clinic between May 1974 and December 1977. A preponderance of females was noticed (female/male ratio of 5:1), while the age distribution of African patients corresponded to values characteristic for the condition seen in other geographical regions. Several morphological types of the condition have been seen. The vitiligoid variant of chronic discoid lupus erythematosus seems to be common in West Africans. Sixteen out of 37 patients presented laboratory abnormalities considered as markers of the association between chronic and systemic lupus erythematosus. Their significance, however is, uncertain as it has been demonstrated on several occasions that in a tropical milieu heavy parasitic infections produce marked immunological disturbances. The problem of the relationship between chronic and systemic lupus erythematosus is discussed and the literature on the incidence of chronic discoid lupus erythematosus in various African countries is reviewed.     

The contribution of direct immunofluorescence to the diagnosis of lupus erythematosus

The contributions of direct immunofluorescence, light microscopy and serology to the investigation of 52 patients with possible lupus erythematosus were studied. Direct immunofluorescence was found to be most valuable in the investigation of possible systemic lupus erythematosus whilst only of supportive value in the investigation of possible chronic discoid lupus erythematosus. The importance of performing light microscopy before direct immunofluorescence in cases of possible chronic discoid lupus erythematosus is shown.     

皮肤型红斑狼疮的治疗现状

皮肤型红斑狼疮是红斑狼疮病谱中相对较轻的一型,治疗上有别于系统性红斑狼疮,目前尚无固定的治疗模式。局部外用糖皮质激素是广泛采用的治疗手段之一,对各种皮肤型红斑狼疮均有效。较新型的外用制剂如他克莫司及吡美莫司,主要用于治疗对糖皮质激素和常规药物无效的皮肤型红斑狼疮。对外用药物治疗无效的皮损,可选择皮损内注射糖皮质激素,以快速发挥抗炎和免疫抑制作用。细胞毒药物和沙利度胺主要治疗复发或难治性皮肤型红斑狼疮。激光及光疗也能有效改善皮肤型红斑狼疮的皮损。     

Lupus erythematosus in children: a report of six cases

The clinical features of childhood discoid lupus erythematosus (DLE) are similar to those of adult DLE in presentation and chronic course. However, children have a particularly high levels of transition to systemic disease. Systemic lupus erythematosus (SLE) is the most common rheumatic disease associated with significant morbidity and mortality in children. This is a retrospective study reporting all cases of childhood lupus erythematosus observed in the dermatology department of Habib Thameur Hospital over a 14-year period. From 1989 to 2003, six cases of childhood lupus erythematosus are included, three patients with discoid lupus erythematosus (2 girls, 1 boy), and three patients with systemic lupus erythematosus (2 boys, 1 girl). The mean age of onset was 12 years (range 10-16 years). Skin manifestations were localized in sun exposed areas in both discoid and systemic lupus erythematosus. Photosensitivity was noted in all cases. The diagnosis was confirmed by histopathologic examination, direct immunofluorescence, and immunologic findings. Treatment included sun avoidance, oral hydroxychloroquine, and topical and systemic steroids. An average follow-up time was 18.1 months (1-96 months). The severity of onset of SLE is usually greater in children than adults. We note that lupus erythematosus is not a static disease and progression from DLE to SLE is possible.     

Detection of anti-type VII collagen antibody in Sjögren's syndrome/lupus erythematosus overlap syndrome with transient bullous systemic lupus erythematosus

Bullous systemic lupus erythematosus (SLE) is a chronic, widespread, non-scarring, subepidermal blistering eruption associated with autoimmunity to type VII collagen. We describe a patient with Sjögren's syndrome/lupus erythematosus overlap syndrome who showed transient blistering eruptions over limited skin surface and in oral mucosa. At the time of aggravation, the patient's serum contained IgG autoantibodies that bound to the dermal side of 1 mol/L NaCl-split normal skin, as determined by an indirect immunofluorescence test, and that reacted to type VII collagen, as determined by immunoblotting on dermal extract. Our observations suggest that a chronic, widespread, blistering eruption is not a prerequisite for the diagnosis of bullous SLE, and a mild, transient, blistering eruption could be a manifestation of type I bullous SLE.     

Epidermolysis bullosa acquisita preceding the development of systemic lupus erythematosus

In most cases of epidermolysis bullosa acquisita that occur in patients with systemic lupus erythematosus, the diagnosis of systemic lupus erythematosus is made before the development of blistering. We observed three patients with well-documented epidermolysis bullosa acquisita that developed several years before the onset of systemic lupus erythematosus. One patient was producing anti-U1RNP autoantibodies at the time epidermolysis bullosa acquisita was diagnosed, and all five produced this antibody during the systemic lupus erythematosus phase of their illness. In addition, in all five cases of epidermolysis bullosa acquisita with systemic lupus erythematosus antibodies to double-stranded DNA ultimately developed, and severe systemic lupus erythematosus and lupus nephritis developed in four patients. Sera from 15 other patients with epidermolysis bullosa acquisita without overt systemic lupus erythematosus were analyzed for systemic lupus erythematosus-related autoantibodies. Four patients were found to have at least one such autoantibody. These findings further document an association between epidermolysis bullosa acquisita and systemic lupus erythematosus and suggest that patients with systemic lupus erythematosus who present with epidermolysis bullosa acquisita may represent a subset of lupus erythematosus that puts the patient at increased risk for the development of more severe systemic illness. Patients presenting with epidermolysis bullosa acquisita, especially those who are black or Hispanic, should be monitored for the development of potentially life-threatening systemic lupus erythematosus.     

Clinical significance and correlation of antinuclear antibodies and anti-DNA antibodies.

The clinical significance and correlation of antinuclear antibodies (ANA) and anti-DNA antibodies was studied using 142 ANA positive sera from different patients having various diseases. High titers of ANA were found briefly in systemic lupus erythematosus and sometimes in scleroderma or mixed connective tissue disease. The peripheral pattern of ANA was seen exclusively in systemic lupus erythematosus and occasionally in mixed connective tissue disease. Anti-DNA antibodies could be found in systemic lupus erythematosus, discoid lupus erythematosus, scleroderma, chronic active hepatitis, but a high titer of anti-DNA (over 60 unit/ml) was present only in patients with systemic lupus erythematosus, especially those having lupus nephritis. There was little correlation between ANA and anti-DNA antibodies.     

Lupus erythematosus panniculitis in a patient with autoimmune hepatitis

Lupus erythematosus panniculitis (profundus), a rare variant of chronic panniculitis, sometimes develops during the course of discoid lupus erythematosus or systemic lupus erythematosus. A 61-year-old woman had suffered from autoimmune hepatitis type I for 5 years. Prednisolone had been administered as maintenance therapy and her hepatitis had been well controlled. However, asymptomatic erythematous indurated nodules developed symmetrically in both pre-auricular regions, and skin biopsy revealed lupus erythematosus panniculitis (profundus). Increase in dosage of prednisolone resolved the skin lesion, leaving depressed atrophic scars. This is the first report of lupus erythematosus panniculitis complicating autoimmune hepatitis.