Pediatric pyoderma gangrenosum associated with leukocyte adhesion deficiency type 1: A case report and review of the literature.

Pyoderma gangrenosum is a rare neutrophilic dermatosis characterized by painful skin ulcers with necrotic, undermined margins. In severe cases, particularly in pediatric patients, work-up for an associated autoimmune, inflammatory, malignant, or genetic disorder should be considered based on the clinical presentation. We report a unique case of pediatric pyoderma gangrenosum with a leukemoid reaction, secondary to an autosomal recessive leukocyte adhesion deficiency type 1.

A Pilot Study of Intralesional Injection of Triamcinolone Acetonide for Desmoid Tumors: Two-Year Outcomes.

PURPOSE: The purpose of this pilot study was to examine the clinical efficacy and safety of serial triamcinolone injections for the treatment of desmoid tumors. PATIENTS AND METHODS: Nine patients were enrolled into this prospective study and underwent three serial ultrasound-guided triamcinolone injections (120 mg) at 6-week intervals. MRI was compared at baseline and every 6 months, out to 24 months. Safety and tolerability were assessed by clinical evaluation and questionnaires, including the 12-item short form survey (SF-12), visual analog scale (VAS), and desmoid patient-reported outcome (PRO) tool. RESULTS: At 24 months, 8 (88.9%) patients demonstrated a reduction in the volume of their tumor while 1 (11.1%) enlarged. Median tumor volume change was -26.9% (-81.1% to 34.6%; P = 0.055) All 9 tumors remained stable based on World Health Organization criteria, whereas 2 (22.2%) demonstrated partial response based on RECIST. There was a significant decrease in the tumor:muscle postcontrast mean signal intensity ratio at 6 months (P = 0.008) and 24 months (P = 0.004). There was a similar decrease in the tumor:muscle T2 mean signal intensity ratio at 24 months (P = 0.02). We found no difference in the SF-12 and VAS scores, but there were significant improvements in the desmoid PRO. CONCLUSIONS: Treatment of desmoid tumors with serial triamcinolone injections appears safe and well tolerated by patients, with a 22% partial response based on RECIST. Further research is needed to confirm our results and determine factors predictive of response.

Impact of Skin Biopsy and Clinical-Pathologic Correlation in Dermatology Inpatient Consults.

Background While studies of hospital dermatology have demonstrated diagnostic discordance between primary teams and dermatology consultants, little is known about the impact of biopsy and clinical-pathologic correlation (CPC) in consultation. This study compares biopsy performance based on diagnostic discordance and evaluates the impact of CPC on the diagnosis. Methods This was a retrospective review of 376 dermatologic consultations at a single academic medical center between July 1, 2017, and June 27, 2018. Results Biopsy was significantly less likely to be performed when the diagnosis by the referring primary team was unspecified (p < 0.001). In 24 percent of cases, the diagnosis based on histopathology alone differed from the diagnosis reached by formal CPC consensus review with either potential or significant impact on management. Conclusion Dermatologists who perform inpatient consultations and rely on hospital-based pathology services may consider a consensus review for CPC. Requests to perform a biopsy may be interpreted as a request for diagnostic assistance rather than pressure to perform a procedure.

Techniques for Decreasing Bacterial Load for Open Shoulder Surgery.

¼: Benzoyl peroxide (BPO) 5% has been shown to reduce Cutibacterium acnes load on the skin. BPO 5% with miconazole nitrate (MN) 2% may be beneficial, whereas BPO 5% with clindamycin cream 1% to 1.2% does not seem to have additive effects when compared with BPO 5% alone. Chlorhexidine gluconate solutions reduce the total bacterial load on the skin, but do not seem to have a significant effect on C. acnes. ¼: ChloraPrep seems to be the best surgical skin preparation to decrease overall positive skin cultures. Preincisional hydrogen peroxide 3% application has been shown to be a cost-effective practice to inhibit growth of C. acnes. Vancomycin powder before deltopectoral interval closure has antimicrobial effects against C. acnes and is a cost-effective practice. Finally, Bactisure surgical lavage is protective against the formation of biofilms. ¼: IV cefazolin has been shown to be more effective for shoulder arthroplasty infection prophylaxis than antibiotic alternatives such as vancomycin. Thus, patients with a questionable history of penicillin allergy should undergo additional testing. ¼: For shoulder surgery infection prophylaxis, we recommend the use of BPO 5% cream for 5 days preoperatively with chlorhexidine wipes the night before and the morning of surgery. IV cefazolin should be administered perioperatively, and patients with a questionable history of penicillin allergy should be tested. Surgeons should consider preincisional application of hydrogen peroxide 3% for 5 minutes, followed by standard ChloraPrep preparation. Normal saline should be used for preclosure lavage. Finally, application of vancomycin powder deep to the deltopectoral interval closure should be considered.

Generalized infantile myofibromatosis with visceral involvement presenting as diffuse hypopigmented macules at birth.

The initial clinical presentation of infantile myofibromatosis can vary from subtle skin changes to large tumors. Here, we describe a case of congenital generalized infantile myofibromatosis which presented with diffuse hypopigmented macules, some with subtle atrophy and telangiectasia. Further workup revealed visceral involvement which led to treatment with systemic chemotherapy. Awareness of this rare clinical presentation is crucial to expedite workup and treatment given the poor prognosis in infants with visceral involvement.

Intralesional immunotherapy for pediatric warts: A review.

Cutaneous warts are a common pediatric complaint with modest response to first-line treatments. Warts are a manifestation of human papillomavirus (HPV) infection and are cleared by cell-mediated immunity (CMI). Intralesional immunotherapy treatments have been studied as alternative therapies, particularly for recalcitrant or multiple warts, including Candida antigen, mumps antigen, the combined measles, mumps, and rubella (MMR) vaccine, tuberculin purified protein derivative (PPD), and bacille Calmette-Guerin (BCG) vaccine. These treatments are thought to increase HPV recognition by stimulating CMI. In this review, we evaluate and compare the efficacy and adverse effects of intralesional immunotherapy in the treatment of pediatric warts. Articles met inclusion criteria if they specifically evaluated the effects of intralesional immunotherapy (candida, MMR, tuberculin PPD, or BCG) as treatment for cutaneous warts in a pediatric population, and if they quantified treatment effect in a reproducible manner. Twenty-one studies met criteria. Many studies demonstrated complete clearance of injected common warts in pediatric patients with clearance rates ranging from 23.3% to 95.2%. Distant wart resolution was common. Intralesional immunotherapy is a promising treatment option for cutaneous warts in children.

Venous thromboembolic and bleeding complications among pregnant women with Klippel-Trenaunay syndrome.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a vascular malformation overgrowth syndrome characterized by capillary malformation, venous malformation, and limb overgrowth, with or without lymphatic malformation. Patients are at an increased risk of hemorrhage and venous thromboembolism (VTE). Consequently, women with this condition often are counseled to avoid pregnancy, but minimal data are available on the relationship between pregnancy, VTE, and bleeding risk. OBJECTIVE: To review the risk of VTE and bleeding in pregnant and nulligravid women with KTS. METHODS: A retrospective medical record review was performed of women with KTS, aged ≥18 years, evaluated at Mayo Clinic Rochester, Minnesota, from August 1945 to April 2018. RESULTS: We identified 75 women with ≥1 pregnancy and 64 nulligravid women. VTE prevalence was 14 of 70 (20%) for women with a history of pregnancy and 16 of 64 (25%) for nulligravid women (P = .93). Among the 70 women with a history of pregnancy, 7 of 18 VTE events (39%) occurred in association with pregnancy, with VTE affecting 7 of 151 pregnancies (4.6%). Significant bleeding prevalence was 6 of 70 (8.6%) for women with a history of pregnancy and 6 of 64 (9.4%) for nulligravid women (P = .54). LIMITATIONS: This was a retrospective review. CONCLUSION: The prevalence of VTE and bleeding was similar in patients with KTS, irrespective of pregnancy status.

Acquired disorders with hypopigmentation: A clinical approach to diagnosis and treatment.

Acquired hypopigmented skin changes are commonly encountered by dermatologists. Although hypopigmentation is often asymptomatic and benign, occasional serious and disabling conditions present with cutaneous hypopigmentation. A thorough history and physical examination, centered on disease distribution and morphologic findings, can aid in delineating the causes of acquired hypopigmented disorders. The second article in this 2-part continuing medical education series focuses on conditions with a hypopigmented phenotype. Early diagnosis and appropriate management of these disorders can improve a patient's quality of life, halt disease progression, and prevent irreversible disability.

Acquired disorders with depigmentation: A systematic approach to vitiliginoid conditions.

Acquired disorders with depigmentation are commonly encountered by dermatologists and present with a wide differential diagnosis. Vitiligo, the most common disorder of acquired depigmentation, is characterized by well-defined depigmented macules and patches. Other conditions, such as chemical leukoderma, can present with similar findings, and are often easily mistaken for vitiligo. Key clinical features can help differentiate between acquired disorders of depigmentation. The first article in this continuing medical education series focuses on conditions with a vitiligo-like phenotype. Early recognition and adequate treatment of these conditions is critical in providing appropriate prognostication and treatment.

Temporal trends in prenatal risk factors for the development of infantile hemangiomas.

BACKGROUND/OBJECTIVES: Specific maternal risk factors have recently been identified in the development of infantile hemangiomas (IH), including gestational diabetes (GDM), maternal antihypertensive medication use or gestational hypertension (GHTN), maternal progesterone use, and artificial reproductive technologies (ART). We sought to explore the change in incidence of these risk factors over time and determine their association with the increased incidence of hemangiomas over 35 years, as previously reported. METHODS: The charts of 869 mother and infant pairs (infants previously diagnosed with IH between January 1, 1976, and December 31, 2010) were reviewed for prenatal complications. Rates of the prenatal complications over the 35-year period in birth mothers of infants diagnosed with IH were determined and evaluated by year of diagnosis (1976-1990, 1991-2000, and 2001-2010). RESULTS: Over the 35-year period in which the incidence of IH was previously examined, maternal age at delivery, prepregnancy body mass index (BMI), use of ART, maternal progesterone use, placental abnormalities, and GDM also increased. CONCLUSIONS: GDM, ART, and maternal progesterone use increased over the past 35 years, mirroring the previously reported trend of increasing incidence of IH. Maternal age and BMI also increased in mothers of infants with IH. Further exploration of this association may direct future research in the pathogenesis of infantile hemangiomas.

Pain, psychiatric comorbidities, and psychosocial stressors associated with Klippel-Trenaunay syndrome.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is characterized by the triad of capillary malformation, venous malformation with or without lymphatic malformation, and limb overgrowth. Patients with KTS have lower scores in general in mental health, physical function, and quality of life than the general population. OBJECTIVE: To determine the prevalence of pain and psychiatric comorbidity in patients with KTS. METHODS: A retrospective review of 410 patients with KTS evaluated during 1976-2012 was conducted to identify the presence of pain, psychiatric comorbidities, and psychosocial stressors. RESULTS: Pain was reported by 260 patients (63.4%) and was associated with any KTS complication (P < .0001) and venous malformations of the lower extremities (P = .0008) and feet (P = .0007). Ninety-five patients had a diagnosed psychiatric condition (23.2%), most commonly depression (15.1%) and anxiety (5.1%). Pain (P = .0016), superficial thrombosis (P = .0269), deep embolic/thrombotic events (P = .0005), gastrointestinal complications (P = .0085), genitourinary complications (P = .0163), and capillary malformation of the hands (P = .0040) were associated with having a psychiatric diagnosis. LIMITATIONS: This is a retrospective study that relied on physician detection and reporting of variables. CONCLUSION: Pain and psychiatric conditions, particularly depression and anxiety, are common in patients with KTS. Awareness of the psychosocial impact of KTS and appropriate screening are important.

Orthopedic complications of linear morphea: Implications for early interdisciplinary care.

Linear morphea of the limb primarily affects children, and extracutaneous manifestations are common. Orthopedic surgeons are often essential in the care of patients with linear morphea, yet there are few reports outlining specific orthopedic complications in this population. We sought to improve the understanding of orthopedic complications in linear morphea of the limb. Between 1999 and 2014, 51 children were evaluated for linear morphea of an extremity. Twenty-six (51%) had documented orthopedic manifestations. Outcome measures included limb length discrepancy, angular malalignment, limb atrophy, and orthopedic surgical intervention. Joint contractures were most common, affecting 88% of patients, followed by limb atrophy, angular deformity, and limb length discrepancy; 14% required surgical intervention. Despite the use of systemic immunosuppressive therapy in many patients, approximately half of patients with linear morphea of an extremity have orthopedic disease. Early orthopedist involvement is crucial to improve limb alignment and preserve function.

Characterizing the Diagnosis and Treatment of Allergic Contact Dermatitis to 2-Octyl Cyanoacrylate Used for Skin Closure in Elective Orthopedic Surgery.

BACKGROUND: While studies have shown that postoperative wound complications can predispose to deep infection following orthopedic surgery, the best form of skin closure has not been elucidated. Furthermore, the unique risks and benefits of each type of wound closure have not been studied. The goal of this study is to present the diagnosis and treatment of patients with allergic contact dermatitis (ACD) from 2-octyl cyanoacrylate, a skin adhesive commonly used in wound closure. METHODS: Twenty-nine patients with ACD to 2-octyl cyanoacrylate (Prineo, Ethicon, NJ) following elective orthopedic surgeries from 2013 to 2016 were retrospectively reviewed; this occurred in 29 of 6088 units of Prineo used at our institution, for an estimated incidence of 0.5%. Nineteen patients (66%) had knee operations. Mean age was 55 years (range, 15-92 years). We classified patients by symptom severity and treatment requirements into mild, moderate, and severe reactions. RESULTS: Most reactions were moderate (48%) or severe (38%) reactions. Mean time from surgery to diagnosis was 11.8 days (range, 2-42 days). All patients underwent removal of the Prineo dressing and daily dressing changes with a specific protocol. Twenty patients (69%) received oral antihistamines, 16 patients (55%) required topical corticosteroids, and 5 patients (17%) required oral corticosteroids. All cases of ACD ultimately resolved at a mean of 22 days (range, 13-56 days) postoperatively. CONCLUSION: 2-Octyl cyanoacrylate skin adhesive occurs in an estimated 0.5% of cases and can lead to severe postoperative ACD when used following orthopedic operations. However, with early recognition and appropriate treatment, patients' symptoms resolve without a significant impact on wound healing.

Successful Treatment of Keratosis Pilaris Rubra with Pulsed Dye Laser.

BACKGROUND: Keratosis pilaris rubra is a common but rarely reported condition characterized by follicular-based hyperkeratotic papules on a background of erythema. It can be embarrassing and symptomatic for patients, particularly adolescent boys. We sought to explore the efficacy of pulsed dye laser (PDL) in the treatment of keratosis pilaris rubra. METHODS: Eight patients were treated with PDL for keratosis pilaris rubra. RESULTS: All patients reported noticeable improvement after one to four treatments. CONCLUSIONS: PDL is an effective, easily accessible, and underused therapy in the treatment of keratosis pilaris rubra.