Cutaneous manifestations of congenital malignant rhabdoid tumor: Unusual papillomatous plaques and other skin presentations.

BACKGROUND/OBJECTIVES: Malignant rhabdoid tumors (MRT) are highly aggressive tumors with a predilection for the kidney, central nervous system, and soft tissues that usually affect young children under three years of age. Primary presentation in the skin is rarely reported, and features of the cutaneous manifestations are not well described. We report six cases of metastatic MRT that first manifested with congenital nodules and masses in the skin. METHODS: Retrospective case series. RESULTS: The cutaneous presentation of MRT may be heterogeneous and can present with solitary or multifocal skin lesions. Congenital polypoidal and papillomatous plaques, including those with histologic features of neurovascular hamartoma, appear to be a unique presentation of MRT in the infant. CONCLUSIONS: Malignant rhabdoid tumor should be considered in the differential diagnosis of unusual skin tumors in neonates and infants.

Wilms tumor screening in diffuse capillary malformation with overgrowth and macrocephaly-capillary malformation: A retrospective study.

BACKGROUND: CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies) syndrome is associated with regional bony and/or soft tissue overgrowth, capillary malformation, and an increased risk for Wilms tumor. OBJECTIVE: To evaluate the frequency of Wilms tumor in patients with 2 similar conditions: diffuse capillary malformation with overgrowth (DCMO) and macrocephaly-capillary malformation (M-CM). METHODS: Culling our Vascular Anomalies Center database, we retrospectively reviewed patients in whom DCMO and M-CM had been diagnosed and who were evaluated between 1998 and 2016 for possible development of Wilms tumor. Patients younger than 8 years of age at their last visit and not seen in more than 2 years were contacted for follow-up. RESULTS: The study comprised 89 patients: 67 with DCMO, 17 with M-CM, and 5 with an indeterminate diagnosis. No case of Wilms tumor was found in these groups. LIMITATIONS: Some patients were younger than 8 years of age at last follow-up visit and the sample size was small. CONCLUSION: Patients with DCMO do not appear to be at increased risk for Wilms tumor. Screening is probably unnecessary in DCMO unless there is associated hemihypertrophy. Although there were no cases in our cohort, there are 2 reports of M-CM associated with Wilms tumor in the literature.

Sonographic screening for Wilms tumor in children with CLOVES syndrome.

BACKGROUND: CLOVES syndrome is associated with somatic mosaic PIK3CA mutations and characterized by congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies. Wilms tumor (WT) is a malignant embryonal renal neoplasm associated with hemihypertrophy and certain overgrowth disorders. After identifying WT in a child with CLOVES, we questioned whether ultrasonographic screening was necessary in these patients. METHODS: We retrospectively reviewed patients with CLOVES syndrome in our Vascular Anomalies Center at Boston Children's Hospital between 1998 and 2016 to identify those who developed WT. A PubMed literature search was also conducted to find other patients with both conditions. RESULTS: A total of 122 patients with CLOVES syndrome were found in our database (mean age 7.7 years, range 0-53 years). Four patients developed WT; all were diagnosed by 2 years of age. The incidence of WT in our CLOVES patient population (3.3%) was significantly greater than the incidence of WT in the general population (1/10,000) (P < 0.001). Four additional patients with WT and CLOVES syndrome were identified in our literature review. CONCLUSION: Patients with CLOVES syndrome have an increased risk of WT. Given the benefits of early detection and treatment, children with CLOVES syndrome should be considered for quarterly abdominal ultrasonography until age 7 years. Screening may be most beneficial for patients under 3 years of age.

Pediatric Pemphigus Herpetiformis: Case Report and Review of the Literature.

Pemphigus herpetiformis (PH) is a rare autoimmune bullous condition usually seen in adults and likely underrecognized in children. We describe a 2-year-old girl who presented with a chronic, generalized, blistering rash consistent with pediatric PH based on histology and immunohistochemistry and review the literature. Prognosis is generally more favorable in children than adults. Prednisone and dapsone should be considered as first-line agents to induce remission.

Treatment of Palmar Hyperhidrosis With Needle Injection Versus Low-Pressure Needle-Free Jet Injection of OnabotulinumtoxinA: An Open-Label Prospective Study.

BACKGROUND: OnabotulinumtoxinA (OnabotA) injections are effective to treat palmar hyperhidrosis (HH) but are quite painful. OBJECTIVE: To evaluate efficacy and pain of OnabotA injection using a needle-free jet apparatus compared with the traditional needle injection to treat palmar HH. METHODS: Twenty patients were recruited for a prospective open-label study. Their right hand was injected with 1% lidocaine with a jet injector, after which OnabotA was injected with a needle. The left hand was injected with OnabotA directly using the jet injector. Pain scores were recorded for both techniques. At 0, 1, 3, and 6 months, severity of palmar HH was evaluated with the Hyperhidrosis Disease Severity Scale (HDSS). RESULTS: One point reduction in the HDSS score at 1 month showed no statistical difference between both hands (p = .451). However, the HDSS score at 1 month from baseline dropped by 1.6 for the hand treated with traditional needle injection of OnabotA compared with 1.25 for the hand treated with jet injections (p = .031). There was no statistical difference in the pain on injection with both techniques (p = .1925). CONCLUSION: This study demonstrates effective and relatively painless use of a low-pressure jet injector for OnabotA in palmar HH.

Flagellate Pattern of Toxic Erythema of Chemotherapy Due to Doxorubicin: A Case Report.

BACKGROUND: Doxorubicin is an antineoplastic agent frequently used in diverse cancer regimens. Cutaneous adverse effects have frequently been reported with its use. However, a flagellate-like dermatitis is not mentioned in the literature. OBJECTIVE: The investigators report a case of toxic erythema of chemotherapy with a flagellate pattern induced by doxorubicin. METHODS AND RESULTS: A 75-year-old woman with endometrial cancer received doxorubicin as part of her treatment. After her third cycle, she presented a pruritic vesiculobullous eruption, with linear elements that left hyperpigmented streaks on follow-up. A biopsy was compatible with a drug eruption. CONCLUSION: Doxorubicin is a well-known cause of toxic erythema of chemotherapy. As seen in this patient, the investigators suggest that it also be added to the list of causes of flagellate dermatosis.

Multiple Xanthogranulomas in an Adult: Known Entity, New Association.

BACKGROUND: Multiple xanthogranulomas (XGs) in adults are rare, although an increasing number of case reports are being published. The most frequent association is hematologic malignancies, but the majority of cases remain idiopathic, with occasional spontaneous resolution. OBJECTIVE: The aims of this report are to describe a case of eruptive XG in a woman with a solid neoplasia who was receiving imatinib and to review the literature. METHODS AND RESULTS: This 33-year-old woman had a gastrointestinal stromal tumor. After undergoing surgical removal and being on imatinib for 1 year, the patient developed multiple slightly erythematous papules with an orange hue on the axillary region, trunk, abdomen, and thighs. A biopsy confirmed the diagnosis of XG. CONCLUSION: This is to the investigators' knowledge the first case of eruptive XG in the setting of a solid neoplasia. The possibility of drug-induced XG lesions due to imatinib cannot be excluded. This presentation could be added to the list of associations of adult XG.

Prevalence of anemia among Quebec Cree infants from 2002 to 2007 compared with 1995 to 2000.

OBJECTIVE: To determine if screening of infants for anemia at 9 months in the Cree region of Quebec should continue,by comparing the prevalence of anemia in the initial years of screening (1995 to 2000) with prevalence data from infants screened between 2002 and 2007. DESIGN: Comparison of anemia prevalence from 2 cross-sectional surveys. Nonoverlapping 95% CIs were used to determine if results were significantly different. SETTING: Nine Quebec Cree communities. Participants Infants screened for anemia between 1995 and 2000 (n = 716) or 2002 and 2007 (n = 1325). Main outcome measures Anemia was diagnosed based on hemoglobin concentration. An erythrocyte mean cell volume of less than 71 fL was used as a proxy for iron deficiency. RESULTS: Hemoglobin concentration among infants screened from 2002 to 2007 was, on average, 7 g/L greater than among infants screened from 1995 to 2000 (mean [standard deviation] 121 [11] g/L vs 114 [11] g/L). The prevalence of anemia (hemoglobin < 110 g/L) from 1995 to 2000 was 31.7% (95% CI 28.3% to 35.1%), but from 2002 to 2007 it was significantly lower at 12.5% (95% CI 10.7% to 14.2%). Using a hemoglobin concentration more specific to iron deficiency anemia (IDA) (hemoglobin < 100 g/L), from 1995 to 2000 7.5% (95% CI 5.6% to 9.4%) of infants had IDA, whereas from 2002 to 2007 only 2.0% (95% CI 1.2% to 2.8%) had IDA. The prevalence of iron deficiency based on mean cell volume declined from 18.3% (95% CI 15.5% to 21.1%) from 1995 to 2000 to 4.2% (95% CI 3.1% to 5.3%) from 2002 to 2007. CONCLUSION: The 12.5% prevalence of anemia (hemoglobin < 110 g/L) among Cree infants from 2002 to 2007 was much lower than the prevalence from 1995 to 2000 but somewhat higher than among nonaboriginal infants (8.0%). The low anemia prevalence among Quebec Cree infants after 2002 suggests that replacing universal screening with targeted screening of higher-risk infants needs to be considered following studies to identify risk factors for anemia.