Lupus erythematosus profundus

Kamal Aggarwal, VK Jain, Surbhi Dayal

 Department of Skin, V. D. and Leprosy, Pt. BDS Postgraduate Institute of Medical Sciences, Rohtak- 124001 (Haryana), India

Correspondence Address:
V K Jain
11 /9J, Medical Enclave, Pt, BDS PGIMS, Rohtak-124 001 (Haryana)
India

Introduction

Lupus erythematosus profundus or panniculitis (LEP) is an unusual but distinct clinical variety of lupus erythematosus. The inflammatory reaction in LEP takes place primarily in the deep corium and the subcutaneous tissues leading to deep indurated nodules or sharply defined plaques. The overlying skin usually appears normal but there may be erythema, atrophy, ulceration or poikilodermatous or hyperkeratotic changes.[1],[2],[3],[4] The lesions are most frequent on cheeks but other sites of predilection are face, upper arms, hands, chest, buttocks and thighs.[5] LEP may develop in association with discoid lupus erythematosus (DLE)[6] or systemic lupus erythematosus (SLE)[5] or may occur as an isolated phenomenon. The differential diagnoses of LEP include panniculitis due to other connective tissue disorders like dermatomyositis or scleredema and Weber Christian panniculitis or Jessner′s lymphocytic infiltration, lyrnphocytoma cults and sarcoidosis. [6]

Case Report

A 41 -year-old man presented with his-tory of development of a single, asymptomatic, erythematous plaque in the left lumbar region since last 2 months. The lesion, initially, was about 2x2 cms in size, oval in shape and firm in consis-tency. It gradually increased in size to about 6x6 cms, with well-defined margins and acquired hard consistency. About 2 weeks, thereafter, the pa-tient developed multiple asymptomatic, ill-to well-defined, erythematous, subcutaneous nodules involving the left nipple and left flank. There was no history of trauma pre-ceding the le-sions and no history of pho-tosensitivity, oral ulcer-ation, arthral-gia or neurop-sychiatric manifesta-tions. Family history was negative.

Examination revealed multiple, erythema-tous indurated plaques and subcutaneous nodules on the left lumbar region [Figure - 1], left flank and left mammary region. The lesions were of vari-able size, ranging from 2x2 cms to 6x6 cms, oval to round in shape, with ill-to well-defined mar-gins, sharply demarcated from the surrounding normal skin, firm to hard in consistency, non-ten-der and bound down to the underlying tissues. No associated lesions of DLE were found. There was left sided inguinal lymphadenopathy with the lymph nodes being firm, discrete and non-tender.

The following laboratory tests were done which showed normal results:- complete blood cell count, differential cell count, ESR, total serum proteins with A:G ratio, blood sugar, blood urea creatinine, alkaline phosphatase, serum electrolytes, liver function tests and urinalysis. X-ray chest was normal. Rheumatoid factor was negative. Blood for LE cells and antinuclear factor was also negative.

Skin biopsy was taken which showed slight hyperkeratosis and acanthosis with liquefactive degeneration of the basal layer at some places. The dermis showed dense lymphocytic infiltrate around the skin adnexal structures, perivascularly and in between the collagen bundles. Similar infiltrate was present in the subcutaneous tissues, especially, around the blood vessels.

We could not perform immunofluorescent studies because of non-availability.

No evidence of SLE was found on clinical examination and laboratory tests.

The patient was given 40mg of prednisolone daily, orally, which led to significant improvement. After 3 weeks, the dose was reduced and gradually tapered and the lesions healed with atrophy.

Discussion

In 1883, Kaposi, [7] first, described the association of subcutaneous nodules with lupus erythematosus. This finding has subsequently being recognized as LE profundus, more accurately, LE panniculitis (LEP). In 1956, Arnold[8] reviewed the literature and helped substantiate the existence of this entity. Recently, Ackerman defined the histological features of LEP.[9] The typical clinical presentation is that of multiple indurated nodules or plaques involving the arms, face, buttocks, legs, chest and less frequently the abdomen, back and neck. The lesions may heal spontaneously leaving depressed atrophic scars. LEP may occur alone prior to the onset or after years of either type of LE. Histopathological studies in previously reported cases have showed changes of DLE in 20% of the cases and that of poikilodermaa in 25% cases.[10] Liquefactive degeneration of the basal layer with lymphocytic infiltration of upper dermis and focal lymphocytic panniculitis have been reported.[11] Lymphoid nodules with germinal centers have been reported in subcutaneous fat or dermis in 55% of the patients.[12] Secondary hyaline degeneration of basement membrane, blood vessels and adipose tissue has been considered characteristic.[12] In our case, there was liquefactive degeneration of basal layer at places and lymphocytic infiltration in dermis and subcutaneous tissue. Immunofluorescence studies have shown deposits of IgM at the basement membrane in most of the reported cases and C3 in some of the patients.[10] We could not do immunofluorescence as we lacked this facility.

Our case did not have any cutaneous lesions of IDLE or SLE but there was an association of mastitis which may account for 10% of all patients with lupus panniculitis.[13] The mastitis cleared completely after treatment with oral corticosteriods. However, as LEP tends to have a chronic course with re-occurrence of nodules and plaques and the subsequent development of SLE in a small percentage of patients, the patient is being re-evaluated periodically.

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