Late onset systemic lupus erythematosus with lichen planus-like eruption and cardiac tamponade

ARTICLE

Systemic lupus erythematosus (SLE) rarely occurs after 60 years of age. Patients with late onset SLE show unusual clinical and laboratory features and should be regarded as a special subgroup with an insidious onset and a benign course compared to the earlier onset group [1-3]. These manifestations often lead to a delay in making a correct diagnosis in elderly patients with late onset SLE. We report an elderly woman with a lichen planus (LP)-like eruption as the initial symptoms and during the follow-up, late onset SLE was finally diagnosed after the development of life-threatening pericarditis with cardiac tamponade. Our case suggests that LP-like eruptions may be considered as one of the unusual variations of skin eruptions in late onset SLE.

Case report

In April 1999, a 71-year-old Japanese woman presented with a 2-year history of asymptomatic scaly erythema on her trunk and extremities. No fatigue, malaise or fever were observed. She denied having experienced oral lesions, Raynaud's phenomenon, arthralgia or photosensitivity. She was not taking any medication. Cutaneous examination revealed scaly erythematous plaques on her arms, forearms, the dorsum of both hands, as well as on her back, and chest (Fig. 1). The larger lesions were raised more at the periphery than in the centers. The scales were often thick, but no follicular plugging was seen. No lymphadenopathy was noted. Laboratory tests revealed the following values: white blood cell count, 6,600/mm³, including 60% neutrophils and 32% lymphocytes; red blood cell count, 426 x 10⁴/mm³; platelets, 22.3 x 10⁴/mm³; serum C-reactive protein 0.2 mg/dl; GOT, 34 IU/l (normal < 33); GPT, 20 IU/l; LDH, 520 IU/l (normal 260-485); ALP 375 IU/l (normal 115-360); total protein, 7.8 g/dl including gamma-globulin 34.3% (normal 10-20); antinuclear antibody, 1:640 with a speckled pattern; anti-double stranded (ds) DNA antibody, 19.4 IU/ml; C3, 18 mg/dl (normal 40-100); C4, below 6 mg/dl (normal 10-40); CH50, below 10 IU/ml (normal 30-44); rheumatoid factor, 21 IU/l (normal > 20); LE test, negative; anti-RNP antibody,1:16; anti-Sm, anti-SS-A and SS-B antibodies, negative; urine, normal. Histological examination of biopsy specimens from scaly erythematous lesions on the dorsum of the hand and back revealed a mixture of orthokeratosis and parakeratosis, focal hypergranulosis, acanthosis with elongation of round-tipped rete ridges, vacuolar basal cell degeneration with a small number of eosinophilic bodies, and bandlike infiltrate in the dermis (Fig.2). Direct immunofluorescence study demonstrated no deposits of IgG, IgM, IgA, C3 or C4. The histological appearance was similar to, but different from the pattern observed in typical LP. The eruptions gradually subsided leaving a depigmented scar after the local application of corticosteroid, however recurrence after discontinuation of therapy was observed during the follow-up.

In August 1999, she was admitted with a similar but much more severe eruption accompanied by fever, edema and shortness of breath. The chest X-ray findings revealed enlargement of the cardiac silhouette and an echocardiogram showed a large degree of pericardial effusion with right atrial and right ventricular collapse consistent with the diagnosis of cardiac tamponade. The laboratory findings were as follows: WBC, 6,100/mm³, including 81% neutrophils and 13% lymphocytes (793/mm³); antinuclear antibody, 1:320 with a speckled pattern; anti-ds DNA antibody, 52.2 IU/ml; C3, 12 mg/dl; C4, below 6 mg/dl; CH50, below 10 U/ml. In addition to the development of skin lesions, our patient also met at least four of the criteria for SLE; pericarditis, lymphopenia, anti-DNA antibody and antinuclear antibody on repeated occasions. Because of the rapid deterioration of pericardial effusion and congestive cardiac failure, she was started on a regime of steroid pulse therapy (1,000 mg of methylprednisolone intravenously daily for three days) followed by oral predonisone (1 mg/kg daily). The treatment successfully reversed most of the clinical (skin lesions and pericardial effusion) and serological signs (C3 and anti-ds DNA antibody) of SLE activity.

Discussion

SLE is not usually thought to be a disease which may begin in the elderly. The clinical presentation and course of SLE may be influenced by the age at disease onset. Patients with late onset SLE constitute a distinct subset of the general SLE population and these patients tend to show a relatively mild severity of disease and a good prognosis [1-3]. Pericarditis or pleuritis are the most common manifestations presenting in late onset patients. Although the most common cardiac manifestation of SLE is pericarditis, the occurrence of subsequent pericardiac tamponade is rare in SLE [4, 5]. In our patient, life-threatening pericardial tamponade developed which resolved after the administration of intravenous corticosteroids.

The initial clinical presentation in late onset SLE has been reported to vary from study to study. As an initial symptom, our patient had cutaneous lesions clinically and histologically resembling LP, and then the passage of time allowed for the development of additional criteria (lymphopenia, pericarditis) until the diagnosis of SLE could be made. In elderly patients, this condition is often not correctly diagnosed until other features such as pleuritis and/or pericarditis develop [1].

The classical cutaneous lesions such as malar erythema or discoid lesions, occur less frequently in late onset SLE [1]. The unusual clinical and histological features of skin eruptions [6] often do not clearly indicate SLE in the elderly. Our patient presented with a LP-like eruption as the initial symptom. The histological appearance of our patient's skin lesions was similar to, but different from the pattern observed in typical LP. To date, several cases of late onset SLE have been reported to have LP-like eruptions [7-9]. Such LP-like eruptions may be one of the unusual variations of late onset SLE. Both LP and SLE are thought to result from the destruction of basal cells by activated T lymphocytes. As a result, the lichenoid phase of SLE and LP may be so similar that a clear distinction may be difficult [10, 11] although histological differentiation is indicated [12]. Therefore, LP/SLE overlapping cases may either have both diseases or are unusual variants of SLE [9].

In our case, direct immunofluorescence study demonstrated no deposits of IgG, IgM, IgA, C3 or C4 . The deposits of IgG and IgM together with C3 can be demonstrated at the dermoepidermal junction in more than 80% of skin lesions of SLE, although in early and late stages the test may be negative. In LP-like eruptions from late onset SLE, deposits of immunoglobulins or complements may not be observed.

Skin eruptions resembling LP are commonly encountered in dermatological clinics. As a result, elderly patients with LP-like eruptions should be carefully followed until a final diagnosis can be clearly made. Even though SLE only rarely occurs in the elderly, it remains a disorder that should not be forgotten.

Article accepted on 18/7/00

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