similarly worsened, as intrapulmonary shunting increased from 40.5% to 55.6%. Daratumumab was therefore discontinued. Regrettably, the patient died of complications of TEMPI syndrome within a year.Discussion | TEMPI syndrome is a multisystem disease with early cutaneous findings and potentially life-threatening pulmonary compromise. 1 Progression to intrapulmonary shunting and hypoxemia can often be avoided with proper treatment, which may result in a more indolent course. 2 Therefore, early recognition of symptoms and establishing the diagnosis is key. 2 Dermatologists can play an important role in diagnosis by recognizing cutaneous findings, which are often an initial presenting symptom. 1 Telangiectasias were reported in 12 of 15 published cases according to a 2018 review and usually affect the face, chest, and back. 1,3 In this patient, they were most notable on the chest and back, along with the lips, where their atypical presentation resembled angiomas or venous lakes (Figure). Relatively similar mucocutaneous findings, although less pronounced, were observed by Schroyens et al. 4 While this patient's lip lesions were not biopsied, we believe they represent telangiectasias given their established association with the disease. 1 Because of the prevalence of telangiectasias in TEMPI syndrome, the disease has been recognized as a monoclonal gammopathy of cutaneous significance, and telangiectasias have been proposed as a major diagnostic criterion. 1,5 Their presence, when concurrent with other TEMPI findings, should raise clinical suspicion for this novel disorder and prompt appropriate referral.The pathogenesis of telangiectasias in TEMPI syndrome remains poorly understood, 5 although a few hypotheses exist. One suggests that the same angiogenic process drives the cutaneous findings and intrapulmonary shunting, as evidenced by the resolution of both symptoms in the same time frame with treatment. 1 Another speculates that hypoxia leads to telangiectasias by causing dilation of superficial capillaries or through induction of hypoxia-inducible factor 1α, a transcription factor that regulates angiogenesis. 3,6 While the exact mechanism remains unclear, this patient's clinical course with concurrent worsening telangiectasias and pulmonary status supports the hypothesis that the 2 share a pathophysiologic association.We present a case of TEMPI syndrome with classic and atypical cutaneous findings, which dermatologists should be familiar with for timely diagnosis. To our knowledge, this case represents the first that was refractory to daratumumab and highlights the need for additional therapies.