The red face revisited: connective tissue disorders

Red face is not a rare finding in patients with connective tissue disorders. The malar eruption is the most frequent cutaneous manifestation of systemic lupus erythematosus (LE). This condition is more apparent among fair-skinned individuals, and it usually appears after sun exposure. A very important clinical sign is that nasolabial folds remain free of any erythematous or other changes. With subacute cutaneous LE, sun exposure can provoke a red face that resembles the malar eruption of systemic LE. The typical clinical findings of chronic cutaneous LE are the discoid lesions. There is a clinical form of chronic cutaneous LE called erythema perstans faciei. This form is purely erythematous, and it usually appears on the face. Other rare "red face" forms of chronic cutaneous LE are LE tumidus and LE telangiectaticus. Red face is not typical of systemic sclerosis, but facial telangiectasias are frequent, especially with CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. The differential diagnoses of other red face manifestations are easy due to the additional findings. Telangiectasias are accompanied by calcinosis, sclerodactyly, digital ischemia, and Raynaud disease. Many studies mention telangiectasias as markers of the severity of the systemic sclerosis, the disease duration, any pulmonary arterial hypertension, and any esophageal involvement. Purple- or violet-colored upper eyelids are the hallmark and one of the first clinical signs that is helpful for the diagnosis of dermatomyositis. This violaceous to dusky erythema can extend over the whole face and the upper aspects of the trunk. Erythematous changes on the face that are different from those of the heliotrope sign which occurs with dermatomyositis may be observed in both sun-exposed skin and non-sun-exposed skin. Malar and facial erythema, linear extensor erythema, V-sign or shawl sign, and other photodistributed eruptions can also appear.