P. García-Montero

Microcystic Lymphatic Malformation Successfully Treated With Topical Rapamycin

This case report presents the first 2 cases to be described of MLM treated with topical rapamycin with satisfactory results and excellent tolerance. Microcystic lymphatic malformations (MLM) are low-flow vascular malformations composed of multiple small cysts. MLM usually affect deep-lying structures, which makes their treatment even more difficult and complex. A novel and interesting treatment is rapamycin, a mammalian target of rapamycin inhibitor that when orally administrated has offered favorable results. However, until recently, topical rapamycin had not been used in the treatment of MLM. Case 1 is a girl aged 13 years with extensive MLM affecting the muscles in the right buttock. The patient had received frequent cycles of cryotherapy, but they had failed to control the associated symptoms. In the previous 12 months, the patient had reported greater discomfort, swelling, exudate, and superinfection of the affected region. Because no specific treatment has yet been approved for MLM, and as a step before the use of aggressive systemic or intralesional treatments, it was decided to initiate treatment with 1% rapamycin ointment. After 4 months of treatment, the patient presented a marked improvement, with a significant reduction of associated complications and no major side effects. Case 2 is a boy aged 5 years who underwent surgery for an intergluteal lipoblastoma at 3 weeks of life and developed a MLM on the scar 6 months afterward. The lesion showed slow growth and continuous exudation with frequent episodes of superinfection. Treatments with laser multiplex and intralesional bleomycin were performed unsuccessfully. In the previous 4 months, the patient had been treated with 1% rapamycin ointment with significant improvement and no side effects.

Adjusting oral oxybutynin medication for hyperhidrosis to reflect seasonal temperature variations

Most studies of oral oxybutynin (OOx) for the treatment of hyperhidrosis (HH) have assumed a stable treatment protocol, without taking into account adverse effects (AE) or seasonal variations in temperature. The objective is to evaluate adjusting the dose of OOx according to the time of year. Prospective study of patients who began OOx for HH between 2007 and 2017, and maintained treatment for at least 1 year, with a progressively increasing dose was performed. All patients were recommended to vary the dose of medication according to the time of year. Baseline epidemiological data, the response to treatment and AE were analyzed. About 122 patients were included (average age of 33.8 years). Up to 60.7% varied the dose. Significantly better results were obtained in the group that varied the dose. Twenty patients suspended the treatment in winter. Among them the adjusted OR was 3.04 (95% CI 1.24–7.45) for an excellent response. The frequency of AE was 74.6% with no differences among groups. Most patients are able to control their HH without requiring the same dose of OOx throughout the year. Given that the possible AE of OOx are dose‐dependent, it seems reasonable to vary it according to the time of year.

Initial study of transdermal oxybutynin for treating hyperhidrosis

Oral oxybutynin for treating hyperhidrosis is effective and safe. Its side‐effects are mild but frequent so we consider whether transdermal oxybutynin (considered to have a better side‐effect profile) could be an alternative for treating hyperhidrosis. During 2015, a prospective study was conducted. Epidemiological variables, effectiveness (using the Hyperhidrosis Disease Severity Scale) and tolerance to transdermal oxybutynin were compiled concerning two different groups (patients previously treated or untreated with oral oxybutynin), at baseline, and at 3 and 12 months. Seven previously treated and six previously untreated patients were included. Five patients in the first group discontinued the treatment within 3 months. Of the two remaining patients, one reported ineffectiveness and the other obtained an excellent response but discontinued due to local irritation. Among the untreated patients, two showed no response and four experienced improvement (three with “partial response” and one with “excellent response”). All patients discontinued treatment within 12 months. No major adverse effects were observed. The absence of active metabolites after transdermal oxybutynin could result in less effectiveness than oral oxybutynin, although it is usually well tolerated. In conclusion, transdermal oxybutynin could have low effectiveness for the treatment of hyperhidrosis in patients following intolerance to oral oxybutynin but could provide good results in patients who have never tried systemic drugs.

Comment on “Congenital Lupus with Multiorgan Involvement: A Case Report and Review of Literature”

To the Editor, In 2015, in this journal, we reported a case of an infant of Senegalese descent with congenital lupus erythematosus with multiorgan involvement. We now report on a follow-up of this infant and the subsequent findings in his younger brother. The older sibling, as previously published in this journal, was born with neonatal lupus erythematosus (NLE) with congenital multiorgan involvement (cutaneous, hematologic, hepatic, respiratory, neurologic). At 5 months of age, the lupus antibodies were completely cleared, with a favorable course without neurological examination abnormalities. At the age of 1 year, a few atrophic scars remained in areas of congenital skin disease, and developmental milestones were achieved except for mild speech delay that resolved a year later. The mother was diagnosed with cutaneous lupus erythematosus 3 months after delivery. Treatment with hydroxychloroquine 200 mg/d was commenced, with excellent control, and continued during the second case pregnancy. The second infant was born 4 years later, at 37 weeks gestational age. Physical examination of the newborn revealed multiple reddish and brownish desquamative patches limited to the craniofacial area (Figure 1). The remaining physical and neurologic findings were normal. No abnormalities appeared on electrocardiogram or head ultrasound examination. Laboratory findings revealed antinuclear antibodies (mottled pattern 1/1280), anti-Ro, and anti-LA, with no other abnormalities. The diagnosis of NLE with congenital cutaneous presentation was established, and strict photoprotection was recommended. One week after this examination, the lesions had spread to the trunk and limbs and had become reddish annular plaques with an atrophic central area, resembling the typical lesions of discoid lupus erythematosus (Figure 2A).

Survival study of treatment adherence by patients given oral oxibutynin for hyperhidrosis

In recent years, increasing use has been made of oral anticholinergics such as oxybutynin for the management of hyperhidrosis. The primary aim of this study is to determine the variables associated with adherence to this treatment, and secondarily to obtain data on its effectiveness, safety and adverse effects.