Atypical and Rare Forms of Cutaneous Lupus Erythematosus: The Importance of the Diagnosis for the Best Management of Patients

Lupus erythematosus (LE) is an autoimmune disease with a wide range of clinical and cutaneous manifestations. Along with the well-known typical cutaneous manifestations of LE, some cutaneous manifestations are rarer, but still characteristic, enabling the dermatologist and the general practitioner who know them to suspect cutaneous LE (CLE) and investigate a possible underlying systemic involvement. Indeed, not infrequently a skin manifestation is the first presentation of systemic LE (SLE), and >75% of SLE patients show signs of skin disease during the course of the illness. Especially, SLE involvement occurs in cases of acute CLE, while it is uncommon in subacute CLE and rare in chronic CLE. This review aims to concentrate especially on atypical cutaneous manifestations of LE to enable the clinician to diagnose even the rarest forms of CLE.

Automated summary

Lupus erythematosus (LE) is an autoimmune disease with a wide range of clinical and cutaneous manifestations. Some cutaneous manifestations are rarer, but still characteristic, enabling dermatologists and general practitioners to suspect cutaneous LE (CLE) and investigate possible systemic involvement. Skin manifestations can often be the initial presentation of systemic LE (SLE), with over 75% of SLE patients showing signs of skin disease. Acute CLE cases commonly involve SLE, while it is less common in subacute CLE and rare in chronic CLE. This review focuses on atypical cutaneous manifestations of LE to aid clinicians in diagnosing even the rarest forms of CLE.