Risk, II.

We describe a family who presented with several scattered, vascular, cutaneous lesions and was found to have a novel mutation in RASA1, diagnostic of capillary malformation-arteriovenous malformation syndrome. Our patient was initially given a presumptive clinical diagnosis of hereditary hemorrhagic telangiectasia. Capillary malformation-arteriovenous malformation syndrome shares several features with hereditary hemorrhagic telangiectasia and hereditary benign telangiectasia, but it can be distinguished clinically according to its morphologic appearance and distribution of cutaneous vascular lesions, the presence of internal fast-flow lesions, and genetic analysis. On physical examination, there were multiple telangiectatic, red macules of variable size surrounded by pale halos on the hands, upper vermillion lip, and face ( Figure 1). These lesions raised concern for HHT and the patient was referred to pediatric dermatology and genetics for further consultation. During examination in our pediatric dermatology clinic, the patient was also found to have blanchable, thumbprint-like lesions with background hyperpigmentation and a pale halo on the chest and legs (Figure 2A). Similar thumbprint-type lesions were observed on the patient's sisters and father.Informed consent and release was obtained from the parents of the patient for photographs included in the manuscript.

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BACKGROUND Staging systems for cutaneous squamous cell carcinoma (CSCC) include Brigham and Women's Hospital (BWH) and American Joint Committee on Cancer staging system, eighth edition (AJCC-8). OBJECTIVE To evaluate and compare AJCC-8 and BWH staging systems for CSCC in immunosuppressed patients. MATERIALS AND METHODS A retrospective cohort study of immunosuppressed patients diagnosed with primary CSCC from 2012 to 2016. The main end point was any poor outcome (PO), which included local recurrence, nodal metastasis, and disease-specific death. RESULTS Fifty-eight immunosuppressed patients had 263 CSCCs. Fifty percent of tumors were AJCC-8 T1, 44.7% T2, and 4.8% T3. Fifty percent of tumors were BWH T1, 48.5% T2a, 1.3% T2b, and 0.4% T3. Risk of PO for AJCC-8 was 1.7%, 8.8%, and 36.4% for T1, T2, and T3, respectively (p < .01). Risk of PO for BWH was 1.8%, 9.9%, 33.3%, and 100.0% for T1, T2a, T2b, and T3, respectively (p < .01). Thirty-six percent of AJCC-8 T3/T4 tumors had POs compared with 5.1% in low T1/T2 stages (p = .002). Fifty percent of BWH T2b/T3 tumors had POs compared with 5.3% in low T1/T2a stages (p = .01). CONCLUSION AJCC-8 and BWH staging systems stratify CSCC with similar distinctiveness, homogeneity, and monotonicity for immunosuppressed patients.

37; serving as a speaker for AbbVie; working as an investigator for Biogen IDEC and Pfizer; receiving grant support from Biogen IDEC; and having a licensed outcome measure for AbbVie. No other disclosures were reported.

Preoperative lesion circumference is directly proportional to primary closure length and is a better indicator of closure length than preoperative area and short axis for MMS of NMSCs. Closure lengths located on the nasal tip, supratip, or periocular areas are most sensitive to differences in NMSC size. These data might aid Mohs surgeons with preoperative planning for wound reconstruction and patient counseling.

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