A case of extensive acquired progressive lymphangioma.

Acquired progressive lymphangioma (benign lymphangioendothelioma) is a rare lymphatic anomaly of unclear pathogenesis. Excision is generally advised for local disease, although other therapies have been tried. This report describes a unique case of extensive acquired progressive lymphangioma involving the abdomen, genitalia, and lower extremity of a 1-year-old boy. Rapid progression and multisite involvement required exploration of nonsurgical options for management.

Lichen planopilaris in women: A survey-based study examining baseline hair characteristics and product use in 129 patients seen at Mayo Clinic.

BACKGROUND: Cicatricial alopecia is a type of permanent hair loss in which the hair follicle is replaced by scar tissue. Given its relatively low incidence, limited information is available regarding baseline hair characteristics, onset, and other disease-associated factors. OBJECTIVE: We sought to further elucidate this information in our lichen planopilaris (LPP) patients. METHODS: Between 1993 and 2016, 505 patients were diagnosed with biopsy-proven LPP. Of these patients, we mailed a 20-question survey to 420 patients with updated mailing address and contact information. Responses were received from 129 of 420 patients for a response rate of 30.7%. A total of 108 patients completed the survey and 21 patients declined participation. Descriptive analysis was performed using JMP software. RESULTS: Median age at time of pathologic diagnosis was 58 years, with self-reported average age of onset accordingly from 50 to 60 years. Approximately 15% of patients were diagnosed with frontal fibrosing alopecia (FFA), a subtype of LPP. Median follow-up from date of pathologic diagnosis was 72 months. At baseline, 46 out of 108 patients (42.6%) endorsed having thicker hair than peers in childhood. No trend was identified in the rate of traction hairstyle use in childhood, current use of cosmetics, sunscreen, or facial cleanser. CONCLUSIONS: Within our LPP cohort, FFA subtype pathology comprised approximately 15% of cases. There was no trend toward sunscreen use and FFA subtype. Our findings support previously published studies in terms of average age at disease onset, location of hair loss, and median follow-up at 72 months (range: 29-273) from date of pathologic diagnosis.

Lichen Planopilaris in Women: A Retrospective Review of 232 Women Seen at Mayo Clinic From 1992 to 2016.

OBJECTIVE: To characterize the clinicopathologic findings, comorbidities, and treatment outcomes of women with lichen planopilaris (LPP). METHOD: In this retrospective review of women with LPP at Mayo Clinic from 1992 to 2016, we searched for scarring alopecia in all female patients aged 1 to 100 years from January 1, 1992, through December 31, 2016. Men were excluded from this study to more accurately determine the association of hormonal factors in LPP pathogenesis. Two hundred thirty-two patients were included as they met diagnostic criteria for LPP based on clinicopathologic correlation, with 217 having confirmatory biopsies. RESULTS: We identified 232 women with LPP (mean age, 59.8 years). Of those, 92.7% (215) presented with hair loss; 23.7% (55) had preceding inflammation; 30.6% (71) had thyroid disease, including hypothyroidism (23.2%; 54); and 9.4% (22) had vitamin D deficiency. Incidence of depression and anxiety was 45.7% (106) and 41.8% (97), respectively. History of total abdominal hysterectomy/bilateral salpingo-oophorectomies and hormone replacement therapy was found in 16.8% (39) and 16.4% (38), respectively. Lichen planus at other body sites occurred in 16.4% (38) of patients; and 53.2% (123) had slowing of disease progression or disease stabilization, often requiring combination therapies. In those who achieved slowing or stabilization of disease, mean time to recurrence was 1.8 year. The mean time to remission was 1.1 year. CONCLUSION: The typical LPP patient is a 60-year-old female with vertex scarring alopecia who presents with burning, erythema, inflammation, and scale. Almost half of patients will have comorbid autoimmunity. As previously reported, LPP is associated with thyroid disease. We also found higher rates of depression, anxiety, nutritional deficiencies, and skin cancer than reported in the general population.

Frontal Fibrosing Alopecia in Women: The Mayo Clinic Experience With 148 Patients, 1992-2016.

OBJECTIVE: To characterize the clinicopathologic findings, comorbidities, and treatment outcomes of women with frontal fibrosing alopecia. PATIENTS AND METHODS: Retrospective review of women with frontal fibrosing alopecia at Mayo Clinic from January 1, 1992, to February 22, 2016. The terms "scarring alopecia," "lichen," "planopilaris," "fibrosing," and "alopecia" were used for the search of female patients aged 1 to 100 years. A total of 686 patients were reviewed to confirm the diagnosis of frontal fibrosing alopecia. Patients were included if they met diagnostic criteria. RESULTS: A total of 148 women with frontal fibrosing alopecia were identified, with a mean age of 62 years; 60.1% presented with eyebrow loss; 67.6% and 27.7% described preceding or concurrent pruritus and trichodynia, respectively; 44.6% had a history of hypothyroidism; 13% had a history of surgical menopause; and 63.3% had a history of hormone replacement therapy. A total of 18.2% had lichen planus at other body sites, and 26.3% achieved disease stabilization, often requiring combination therapies. The mean time to remission was 1.8 years. CONCLUSION: Patients with frontal fibrosing alopecia typically present with frontotemporal and eyebrow alopecia with preceding symptoms. Hypothyroidism and a history of hysterectomy may be more common than previously reported. Time to presentation, diagnosis, and stabilization is often months to years. Patients who lack treatment response may present with eyebrow loss, eyelash loss, and facial papules. Combination therapy is helpful in achieving slowing of disease progression or disease stabilization, although recurrence is common. Additional studies on treatment and efficacy are needed. Limitations to this study include the retrospective design and varied follow-up.

Tablet-Based Application for Objective Measurement of Motor Fluctuations in Parkinson Disease.

BACKGROUND: The motor subscale of the Movement Disorder Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS-III) has limited applicability for the assessment of motor fluctuations in the home setting. METHODS: To assess whether a self-administered, tablet-based application can reliably quantify differences in motor performance using two-target finger tapping and forearm pronation-supination tasks in the ON (maximal dopaminergic medication efficacy) and OFF (reemergence of parkinsonian deficits) medication states, we recruited 11 Parkinson disease (PD) patients (age, 60.6 ± 9.0 years; disease duration, 12.8 ± 4.1 years) and 11 healthy age-matched controls (age, 62.5 ± 10.5 years). The total number of taps, tap interval, tap duration, and tap accuracy were algorithmically calculated by the application, using the more affected side in patients and the dominant hand in healthy controls. RESULTS: Compared to the OFF state, PD patients showed a higher number of taps (84.2 ± 20.3 vs. 54.9 ± 26.9 taps; p = 0.0036) and a shorter tap interval (375.3 ± 97.2 vs. 708.2 ± 412.8 ms; p = 0.0146) but poorer tap accuracy (2,008.4 ± 995.7 vs. 1,111.8 ± 901.3 pixels; p = 0.0055) for the two-target task in the ON state, unaffected by the magnitude of coexistent dyskinesia. Overall, test-retest reliability was high (r >0.75) and the discriminatory ability between OFF and ON states was good (0.60 ≤ AUC ≤ 0.82). The correlations between tapping data and MDS-UPDRS-III scores were only moderate (-0.55 to 0.55). CONCLUSIONS: A self-administered, tablet-based application can reliably distinguish between OFF and ON states in fluctuating PD patients and may be sensitive to additional motor phenomena, such as accuracy, not captured by the MDS-UPDRS-III.

Orthostatic hypotension in Parkinson's disease: Does it matter if asymptomatic?

INTRODUCTION: Orthostatic hypotension (OH) may frequently be asymptomatic in patients with Parkinson's disease (PD). However, the relationship between symptomatic/asymptomatic status and functional disability remains unclear. METHODS: Using orthostatic blood pressure (BP) measurements and the Orthostatic Hypotension Symptom Assessment (OHSA) questionnaire, 121 consecutive PD patients without history of chronic hypertension and not taking alpha-adrenergic antagonists for bladder disorders were classified according to (1) OH symptomatic status, based on presence/absence of orthostatic symptoms (symptomatic OH: OHSA item 1 ≥ 1), and (2) OH severity, based on the magnitude of BP fall on the lying-to-standing test: OH- (<20/10 mmHg); moderate OH+ (≥20/10 mmHg but < 30/15 mmHg); and severe OH+ (≥30/15 mmHg). The primary endpoints were the activities of daily living/instrumental activities of daily living (ADL/iADL) and the Ambulatory Capacity Measure (ACM). Secondary endpoints included PD quality of life (PDQ-8) and prevalence of falls. RESULTS: The overall prevalence of OH+ was 30.6% (37/121 patients), with 62.2% symptomatic (23/37) and 37.8% asymptomatic (14/37). Symptomatic and asymptomatic OH + patients had similar impairments in ADL/iADL and ACM, significantly worse than OH- (p ≤ 0.035). There was a trend for worse ADL/iADL and ACM scores in severe OH + compared to moderate OH+, but both were worse than OH- (p ≤ 0.048). Symptomatic and asymptomatic OH + showed similar impairment in PDQ-8 and higher prevalence of falls compared to OH-. CONCLUSIONS: Asymptomatic OH+ was associated with similar impairments in ADL/iADL and ACM than symptomatic OH+. These findings support screening for OH in PD patients regardless of postural lightheadedness.