Lupus erythematosus panniculitis

Introduction

This is an unusual clinical variant of lupus erythematosus characterised by appearance of erythematous, firm, well defind indurated nodules on face, breast, upper portion of arms, thighs and buttocks. Typical lesions of DLE and ulceration may be present on the skin overlying these nodules, but sometimes the nodules may lie beneath normal skin. The histology is that of lobular panniculitis and direct immunofluorescent test shows linear pattern at dermoepidermal junction. Overlying epidermis may or may not show evidence of changes typical of LE. The condition responds well to chloroquine therapy. We describe here 2 cases of LE panniculitis with no evidence of systemic involvement.

Case Report

Case 1: A 22-year-old female was seen in late 1994 for a firm induration on her right cheek which started 4 months before, as a pea sized swelling and gradually increased in size. The lesion was painless and nontender, and unassociated with any other complaints like fever, arthralgia or photosensitivity. Past history and family history were unremarkable.

Examination revealed a well circumscribed, irregularly bordered, nontender subcutaneous induration on the right cheek, 3cm x 4cm in dimension. The surface was normal looking. No other cutaneous or systemic abnormality was detected clinically.

Routine laboratory tests and a chest X-ray were noncontributory. Antinuclear antibody test gave negative results. Histopathology showed features of a nonspecific panniculitis comprising of a collection of lymphocytes, histiocytes and occasional plasma cells. Fibrinoid deposit was observed in the fat lobules.

The patient was put on oral chloroquine (150 mg base/day) and the lesions disappeared after 2 months of therapy, leaving an unsighty depression of the cheek. Two months after stoppage of therapy, a similar subcutaneous swelling appeared on her left upper arm which showed similar good response and no recurrence was obseerved on 8 months of follow up.

Case 2: A 40-year-old female presented with a firm erythematous plaque on her right cheek which started 10 months earlier as a small pea sized swelling and has gradually increased in size. The lesion was painless and nontender at the begining but for last few months, the swelling became painful. She had no other complaints like fever, arthralgia or photosensitivity. She was treated with a local physician as a case of Hansen′s disease, but on starting antileprotic drugs, the lesion flared up. There was no significant past history, family history and personal history.

Examination revealed a well circumscribed, erythematous, tender, indurated subcutaneous swelling on the right cheek just below right orbit, extending over nose. The skin over the swelling was erythematous, slightly scaly with atrophy at the centre. Right parotid gland was enlarged and tender. Another pea sized swelling was found on her left arm. No other cutaneous or systemic abnormality was detected clinically.

Routine laboratory tests and a chest X-ray was within normal limits. Antinuclear factor was positive but LE cell test was negative. Histopathology showed features of a typical lobular panniculitis [Figure - 1]. Epidermis was more or less normal and typical changes of discoid lupus erythematosus was not seen.

The patient was put on oral chloroquin (150 mg base/day). The lesions started regressing at the end of the month and the treatment is still continuing.

Discussion

This entity was first described by Kaposi in 1883. The term LE panniculitis was coined by Irgang in the year 1940. Lupus erythematosus profunds or panniculitis is an unusual clinical variant of lupus erythematosus in which cutaneous infiltrate occurs primarily in the deeper portion of corium giving rise to firm sharply defind nodules of varying size, lying beneath normal skin. This was typically found in first case and the histopathology was sufficiently characteristic to make a diagnosis in absence of other cutaneous or systemic lesions of lupus erythematosus. In both the cases, immunoflourescent test was not done due to lack of facility.

In the second case, due to presence of erythema, tenderness and scaling, diagnosis of borderline tuberculoid leprosy with Type I reaction came in differential diagnosis and she was advised antileprotic treatment from another hospital. Biopsy was confirmatory and she is responding very well to chloroquin.

So far we could find only one case of lupus panniculitis reported in Indian litrature. 1[1]