Squamous Cell Carcinoma in Perineal, Perianal, and Gluteal Hidradenitis Suppurativa: Experience in 12 Patients.

BACKGROUND: Few reports describe squamous cell carcinoma (SCC) arising in hidradenitis suppurativa (HS). OBJECTIVE: The 2 objectives were (1) to describe the clinical characteristics, pathologic findings, and postoperative outcomes of SCC in HS and (2) to assess whether human papillomavirus (HPV) is involved in the pathogenesis. MATERIALS AND METHODS: Cases of SCC in HS were identified through institutional medical records (1976-2013) and the Rochester Epidemiology Project. Tumor specimens were assessed for HPV DNA/RNA with in situ hybridization. RESULTS: Twelve patients were identified (11 Caucasians and 9 men). All SCCs involved gluteal, perianal, or perineal skin; 1 patient had, in addition, involvement of the vagina. Surgical excision was performed on all 12 patients, 4 of whom had a colostomy. Mean duration of HS before SCC development was 28.5 years (range, 15-53 years). Mean follow-up was 4.3 years after surgical excision. Seven of 12 patients followed had postoperative SCC recurrence. Squamous cell carcinoma caused death despite wide surgical excision in these 7 patients. Of the remaining 5 patients, 4 are unknown and 1 who did not recur had an in situ SCC (Bowen disease carcinoma). Squamous cell carcinoma was not associated with high-risk or low-risk HPV. CONCLUSION: Invasive SCC arising in HS carries a high risk of death.

Patient Satisfaction and Quality of Life Following Surgery for Hidradenitis Suppurativa.

BACKGROUND: Hidradenitis suppurativa (HS) is a progressive, recurrent inflammatory disorder. OBJECTIVE: To assess long-term satisfaction and postoperative perceptions among patients who underwent surgical management of HS. MATERIALS AND METHODS: A questionnaire was mailed to 499 HS surgical patients to assess surgical outcome, satisfaction, and quality of life. RESULTS: Of the 499 questionnaires mailed, 113 were returned (22.6% response rate) and 2 were excluded for redundancy. Of the 111 respondents, 65 (58.6%) were female, 91 (82.0%) had Hurley Stage III disease, 88 (79.3%) were treated with excision and 23 (20.7%) with unroofing, 45 (40.5%) had perianal or perineal disease, and 41 (36.9%) had axillary disease. Most patients were satisfied or very satisfied with their surgical results (84.7%; 94 of 111), were glad they underwent surgery (96.3%; 105 of 109), and would recommend surgery to a friend or relative (82.6%; 90 of 109). Most patients were satisfied or very satisfied with the appearance of their healed wound (62.4%; 68 of 109). Retrospective mean quality of life increased significantly from 5 preoperatively to 8.4 postoperatively (p < .001). CONCLUSION: Hidradenitis suppurativa surgical management was well regarded by patients and should be considered by future patients to limit the morbidity of HS.

Mucosal Lesions in an Allergy Practice.

The diagnosis and treatment of mucosal disease with an allergic pathogenesis are challenging. Oral allergy is often a hypersensitivity reaction with variable symptoms and physical exam findings. Clinical diagnosis requires a history of prior allergen exposure, a delay from exposure to clinical findings, and improvement following allergen removal. The past decades have seen great contributions to the field of oral allergy. The aim of this review is to provide an approach to the diagnosis and treatment of oral dermatologic disease with a focus on diseases with an investigated allergic pathogenesis.

Surgical Management of Hidradenitis Suppurativa: Outcomes of 590 Consecutive Patients.

BACKGROUND: Hidradenitis suppurativa is a progressive, recurrent inflammatory disease. Surgical management is potentially curative with limited efficacy data. OBJECTIVE: To evaluate hidradenitis surgical patients. METHODS: Retrospective review of outcomes of 590 consecutive surgically treated patients. RESULTS: Most patients were white (91.0% [435/478]), men (337 [57.1%]), smokers (57.7% [297/515]) with Hurley Stage III disease (476 [80.7%]). Procedure types were excision (405 [68.6%]), unroofing (168 [28.5%]), and drainage (17 [2.9%]) treating disease of perianal/perineum (294 [49.8%]), axilla (124 [21.0%]), gluteal cleft (76 [12.9%]), inframammary (12 [2.0%]), and multiple surgical sites (84 [14.2%]). Postoperative complications occurred in 15 patients (2.5%) and one-fourth (144 [24.4%]) suffered postoperative recurrence, which necessitated reoperation in one-tenth (69 [11.7%]) of patients. Recurrence risk was increased by younger age (hazard ratio [HR], 0.8; 95% confidence interval [CI], 0.7-0.9), multiple surgical sites (HR, 1.6; 95% CI, 1.1-2.5), and drainage-type procedures (HR, 3.5; 95% CI, 1.2-10.7). Operative location, disease severity, gender, and operative extent did not influence recurrence rate. CONCLUSION: Excision and unroofing procedures were effective treatments with infrequent complications and low recurrence rates. Well-planned surgical treatment aiming to remove or unroof the area of intractable hidradenitis suppurativa was highly effective in the management of this challenging disease.

Systemic associations of hidradenitis suppurativa.

Hidradenitis suppurativa (HS) is a progressive, inflammatory disease that affects mostly young women and appears to be caused by inflammation of hair follicles in areas of friction in the body (eg, the axillae, groin, perineum, and medial aspects of the thighs). Given this pathophysiology, one might expect comorbidities that contribute to inflammation and friction. Observed comorbidities fall into several categories: obesity and the metabolic syndrome, hormone-related disorders, deleterious health habits and mood, autoimmune disease, inflammatory disease and finally, the risk of skin cancer and sequelae of nonhealing wounds. The available literature on comorbid diseases of HS is limited but rapidly increasing. In this review, we summarize recent and major studies of HS disease association.

Treatment experience in a local population with hidradenitis suppurativa.

BACKGROUND: Treatment of hidradenitis suppurativa has been studied, but treatment strategies and outcomes have not been reported for a large community-based group of patients. OBJECTIVE: We sought to determine the treatments most commonly prescribed and the performance of all systemic and surgical treatments used in hidradenitis suppurativa patients in Olmsted County, Minnesota, treated over a 40-year period. METHODS: A retrospective chart review was performed to evaluate hidradenitis suppurativa treatments in 376 episodes with 115 Olmsted County patients seen by a clinician at Mayo Clinic in Rochester, Minnesota, between 1968 and 2008. Treatment episode outcomes were recorded from clinical notes for the 73 treatment episodes that had a follow-up period of more than 30 days. RESULTS: Systemic antibiotics alone were prescribed most frequently in 70.0% of episodes. Systemic antibiotics alone improved 39 of 49 treatment episodes (79.6%), including 13 episodes (26.5%) when the disease was fully cleared. All 5 of 5 episodes (100%) of surgical treatment alone improved, including 4 (80%) in which the disease was fully cleared. Surgery in combination with systemic antibiotic treatment yielded improvement in 5 episodes (71.4%), with 2 episodes (28.6%) showing complete clearance. CONCLUSION: Systemic antibiotics were the most frequently prescribed treatment type in 115 patients over a 40-year period. Both systemic antibiotic therapy and surgical treatment are effective in disease management.

Long-term risk of death in patients with hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa (HS) is associated with substantial morbidity. Few studies have addressed the natural history and mortality rates associated with HS. A higher risk of cardiovascular death in patients with HS has been reported. We investigated whether patients with HS have an increased overall risk of death compared with age- and gender-matched referents. METHODS: We performed a retrospective cohort study of residents of Olmsted County, Minnesota, with incident HS between January 1, 1980, and December 31, 2008, and age- and gender-matched referents. The main outcomes and measures were the overall and cause-specific risks of death. RESULTS: We identified 226 incident cases of HS and 678 age- and gender-matched referents among Olmsted County residents during the study period. Compared to referents, patients with HS had a significantly higher risk of all-cause death (hazard ratio [HR, 2.48; 95% CI, 1.53-4.03, P < 0.001) and cardiovascular- or cerebrovascular-related death (HR, 2.85; 95% CI, 1.10-7.40, P = 0.03). However, these risks were attenuated by adjusting for smoking history: all-cause HR, 1.65 (95% CI, 0.97-2.82, P = 0.07) and cardiovascular- or cerebrovascular-related HR, 2.03 (95% CI, 0.71-5.81, P = 0.18). The 71% of patients were former or current smokers at the time of HS diagnosis. CONCLUSIONS: Hidradenitis suppurativa patients have a substantially increased risk of death from any cause, including cardiovascular or cerebrovascular causes; the risk is especially dependent on smoking history.

Unmasking Masks.

Masks are coverings of the face that serve a variety of purposes. Throughout history, they have been used for religious ceremonies, theatric entertainment, protection of identity, and as a tool to limit the spread of infectious diseases. The development of masks in the medical setting has evolved along with our understanding of the mode of disease transmission, material science, and population health dynamics. The simple barrier face mask, surgical mask, and filtering facepiece respirator are each appropriate in different settings. Establishing evidence-based guidance behind the use of masks is essential, particularly in public health crises.

A case of streptococcal surgical site infection following Mohs surgery.

Group A ß-hemolytic Streptococcal (GAS) cellulitis is an uncommon surgical site infection that presents with rapid onset of pain and swelling in the first few days after a procedure. Unlike staphylococcal cellulitis, GAS cellulitis lacks purulence and spreading erythema. The absence of these classic signs may delay the diagnosis of GAS cellulitis and lead to severe complications. We present the case of an immunosuppressed 49-year-old patient who developed swelling and severe pain at his incision site two days after undergoing Mohs micrographic surgery on his forehead. He was clinically diagnosed with GAS cellulitis and recovered with intravenous antibiotics. Unfortunately, there is a paucity of information about GAS cellulitis in the dermatologic literature and clinicians need to recognize and aggressively treat this rare but serious complication of Mohs micrographic surgery.

Sudden cardiac arrest in a young patient with hypertrophic cardiomyopathy and zero canonical risk factors: the inherent limitations of risk stratification in hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy is the most common heritable cardiovascular disease and a common cause of sudden cardiac death (SCD) in young adolescents and athletes. Clinical risk stratification for SCD is predicated on the presence of established risk factors; however, this assessment is far from perfect. Herein, we present a 16-year-old male who was resuscitated successfully from his sentinel event of out-of-hospital cardiac arrest. Prior to this event, he was asymptomatic and lacked all traditional SCD-predisposing risk factors for hypertrophic cardiomyopathy.

A population-based study of the incidence of burning mouth syndrome.

OBJECTIVE: To calculate the incidence of burning mouth syndrome (BMS) in Olmsted County, Minnesota, from 2000 through 2010. PATIENTS AND METHODS: By using the medical record linkage system of the Rochester Epidemiology Project, we identified newly diagnosed cases of BMS from January 1, 2000, through December 31, 2010. Diagnoses were confirmed through the presence of burning pain symptoms of the oral mucosa with normal oral examination findings and no associated clinical signs. Incidence was estimated using decennial census data for Olmsted County. RESULTS: In total, 169 incident cases were identified, representing an annual age- and sex-adjusted incidence of BMS of 11.4 per 100,000 person-years. Age-adjusted incidence was significantly higher in women than in men (18.8 [95% CI, 16.4-22.9] per 100,000 person-years vs 3.7 [95% CI, 2.6-5.7] per 100,000 person-years; P<.001). Postmenopausal women aged 50 to 89 years had the highest incidence of the disease, with the maximal rate observed in women aged 70 to 79 years (70.3 per 100,000 person-years). After the age of 50 years, the incidence of BMS in men and women significantly increased across age groups (P=.02). Study participants residing in Olmsted County, Minnesota, were predominantly white, which is a study limitation. In addition, diagnostic criteria for identifying BMS in the present study may not apply for all situations because no diagnostic criteria are universally recognized for identifying BMS. CONCLUSION: To our knowledge, this is the first population-based incidence study of BMS reported to date. The data reveal that BMS is an uncommon disease highly associated with female sex and advancing age.

Early postnatal nutrition determines adult physical activity and energy expenditure in female mice.

Decades of research in rodent models has shown that early postnatal overnutrition induces excess adiposity and other components of metabolic syndrome that persist into adulthood. The specific biologic mechanisms explaining the persistence of these effects, however, remain unknown. On postnatal day 1 (P1), mice were fostered in control (C) or small litters (SL). SL mice had increased body weight and adiposity at weaning (P21), which persisted to adulthood (P180). Detailed metabolic studies indicated that female adult SL mice have decreased physical activity and energy expenditure but not increased food intake. Genome-scale DNA methylation profiling identified extensive changes in hypothalamic DNA methylation during the suckling period, suggesting that it is a critical period for developmental epigenetics in the mouse hypothalamus. Indeed, SL mice exhibited subtle and sex-specific changes in hypothalamic DNA methylation that persisted from early life to adulthood, providing a potential mechanistic basis for the sustained physiological effects. Expression profiling in adult hypothalamus likewise provided evidence of widespread sex-specific alterations in gene expression. Together, our data indicate that early postnatal overnutrition leads to a reduction in spontaneous physical activity and energy expenditure in females and suggest that early postnatal life is a critical period during which nutrition can affect hypothalamic developmental epigenetics.