Background  Telangiectasia macularis multiplex acquisita (TMMA) is a rarely documented entity with distinctive clinical manifestation. Most patients are middle-aged adults. It may be associated with other diseases such as hepatitis, diabetes, or cardiovascular diseases.

Objectives  To assess the clinical presentation of TMMA and its association to other systemic diseases, especially the correlation to liver disease, in Taiwan.

Methods  We identified and retrospectively analyzed clinical criteria-matched and skin biopsy-proven patients from 2002 to 2010 at a single medical center. The clinical criteria for diagnosis of TMMA included: (i) crops of telangiectasia superimposed on erythematous macules symmetrically on bilateral upper arms, possibly extending to the forearms, shoulders, V-shaped area of the anterior chest, back or thighs; (ii) no mucosal or systemic involvement; (iii) not associated with autoimmune diseases, such as lupus erythematosus, dermatomyositis or systemic scleroderma that may induce cutaneous telangiectasia; and (iv) no ataxia or unsteady gait.

Results  Twenty-five patients were enrolled in this study. The male-to-female ratio was 19:6. The age of onset was variable, mostly between the third to sixth decades. The V-shaped area of the anterior chest was commonly involved (68%), in addition to the arms (100%). Viral hepatitis was diagnosed in 56.5% of the patients (13/23); 38.9% (7/18) and 50% (9/18) of the patients had diabetes and hypertension, respectively. In addition, 64.7% of the patients (11/17) had dyslipidemia. The pathological reports of most cases showed only mild perivascular lymphocytic infiltration with or without telangiectasia.

Conclusions  TMMA should be assigned to a distinct entity with unique clinical manifestation. It has not been well recognized and mostly misdiagnosed as other diseases with telangiectasia. It is important to evaluate the complete liver function tests when TMMA is suspected.