Lupus Erythematosus Profundus: A Case Series and Review of Literature

Abstract

Objective: Lupus Erythematosus Profundus (LEP) is a rare subset of Lupus erythematosus and clinically presents as indurated subcutaneous painful nodules and plaques. The objective of the study was to evaluate clinical, histopathological and supplementary laboratory parameters to diagnose Lupus erythematous profundus.Methods and materials: This was a retrospective study, a clinical series of histo-pathologically proven cases of 11 patients. Data were collected from clinic records from 1996 to 2010. A detailed history including onset of disease, age, sex, residence, hospital ID number and clinical examination, association with Discoid lupus erythematosus (DLE) or Systemic lupus erythematosus (SLE), laboratory workup, histopathological evaluation and Direct Immunofluorescence data was taken from the records.Result: There were total of 11 patients, with mean years of presentation of 26 years. Male to female ratio was 3:8. Face was the commonest site of distribution. Exclusively LEP presentation was seen in 83% and in 18% associated with DLE and 9% with SLE. Clinically lesions varied from nodules, indurated plaques, atrophy and ulcer. In laboratory work up ANA was positive in 9% of the cases, others baseline investigations were within normal limits. On histopathological evaluation, lobular panniculitis and hyaline fat necrosis was seen in all patients. The infiltrates were predominantly lymphohistiocytic. Direct immunofluorescence (DIF) could be done in 3 out of 11 cases. Lupus band at basement membrane zone was seen in all the 3 cases. Deposition of predominantly IgG followed by IgM, IgA and C3 were seen. Two of the three cases with positive DIF findings had no interface pathology.Conclusion: Diagnosis of LEP is based on clinico-pathological correlation. An early diagnosis and prompt treatment may help prevent disfigurement.