Atopic dermatitis in children: what to do when nothing works.

Atopic eczema in children usually responds to a management programme involving the regular use of emollients and the topical use of anti-inflammatory agents combined with patient education and the avoidance of environmental irritants or allergens (where necessary). If treatment fails, non-compliance should be considered first. Parents need to be reassured; a standardized educational program how to handle the disease with all its implications seems helpful. Persistent allergens or irritative triggers should be identified and eliminated. Systemic immunosuppression with either ciclosporin or azathioprine, mycophenolate mofetil or others should only be considered when parental compliance is warranted and all efforts have been made to eliminate external triggers. This article gives a short review on possible causes of treatment failures and ways to cope with them.

Neonatal haemangiomatosis associated with placental chorioangiomas: report of three cases and review of the literature.

We report three cases of neonatal haemangiomatosis associated with large placental chorioangioma. Pregnancies were complicated by polyhydramnios, and all mothers underwent amniocentesis to drain the liquid. Steroid treatment was required for two children. Although the theory has been largely disproved in normal haemangiomas, embolization of precursor endothelial cells derived from placental vessels is a likely explanation for the pathogenesis of haemangiomatosis associated with large placental chorioangiomas.

Cutis marmorata telangiectatica congenita: a prospective study of 27 cases and review of the literature with proposal of diagnostic criteria.

BACKGROUND: Cutis marmorata telangiectatica congenita (CMTC) is a congenital vascular anomaly of unknown aetiology. About 300 cases have been reported in the literature. The rate of associated anomalies varies between 20% and 70%. METHODS: We report a series of 27 children with CMTC, 18 of whom were followed-up prospectively for a median of 22 months (range 2 months-5.3 years). RESULTS: Both genders were equally affected (13 male/14 female). The legs were involved in 20 cases (74%), the arms in 10 (37%), the face in 4 (15%) and the trunk in 18 (67%). There were 20 (74%) patients who presented with involvement of both trunk and limbs, a further 20 patients had lesions affecting the limb on only one side of the body, and 7 children (26%) had bilateral lesions; 1 child had generalized CMTC lesions. The involved areas covered a mean of 18% of body surface area (range 3-90). Associated anomalies were found in 15 patients (56%), with some exhibiting more than one. There was body asymmetry (hypertropy or hypotrophy of the affected limb) in nine patients (33%), seven patients had a variety of other malformations (congenital glaucoma, syndactyly, lipoma, macrocephaly, renal hypoplasia, Kartagener's syndrome), and other vascular lesions were present in four patients (15%). There was no correlation between the extent of skin lesions and likelihood of associated anomalies. On follow-up, fading of skin lesions was noted in 67% of our patients. CONCLUSION: Body asymmetry is the most common anomaly associated with CMTC; other associations might be pure chance. In order to separate CMTC from other vascular malformations, notably Klippel-Trénaunay syndrome, we suggest diagnostic criteria for their differentiation.

Infantile-onset cutaneous T-cell lymphoma.

BACKGROUND: Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma (CTCL), is mainly a disease of the elderly. OBJECTIVES: To review paediatric CTCL cases reported in the literature, with a focus on the time between onset of symptoms and establishment of a correct diagnosis. METHODS: A review of the literature was carried out and a case reported. RESULTS: A total of 254 cases of CTCL have been reported in children aged<16 years, 13 cases (<1% of all reported cases) in children below the age of 2 years, and only seven cases (including ours) during the first year of life. CTCL was most prevalent in children aged 10-12 years. The delay between age of onset and establishment of diagnosis was largest in the youngest age group (0-3 years), and declined steadily thereafter, thus reflecting the increasing likelihood that CTCL is considered in the differential diagnosis of skin disorders with increasing age of the patient. CONCLUSIONS: The diagnosis of CTCL is frequently delayed in young children. It needs to be considered in chronic 'eczematous' skin lesions irrespective of the patient's age, and including infants.

Nicolau's syndrome induced by intramuscular vaccinations in children: report of seven patients and review of the literature.

BACKGROUND: Nicolau's syndrome (NiS), or embolia cutis medicamentosa, is a rare condition characterized by the acute onset of cutaneous and soft-tissue necrosis following intramuscular drug injection. Intramuscular injections are the main route for vaccinations in children. METHODS: This is a retrospective study of seven children (mean age 9.8 months) who developed NiS subsequent to intramuscular vaccination. RESULTS: The reactions were observed after different combinations of vaccine antigens, and were no more common after repeated than after primary injection of the respective vaccine. Three children developed scars without functional impairment, two made a full recovery, and the final outcome is unknown for four. CONCLUSION: Given the high prevalence of intramuscular injections during infancy, NiS seems to be a rare event, but there is a possibility of under-reporting of less severe reactions. Our retrospective data do not allow a true risk assessment. The most worrying aspect of NiS, however, is its lack of predictability. As long as complete avoidance of the intramuscular route is not an option, it is obvious that NiS cannot be completely prevented.

Myokymia as a presenting sign of congenital smooth muscle hamartoma.

We report a 4-week-old male infant with intermittent asymptomatic bouts of vermicular movements of the skin (myokymia). Myokymia started at the age of 4 days. Attacks occurred approximately every 30-60 minutes and could be elicited by gently stroking the skin. Macroscopically no abnormalities typical for smooth muscle hamartoma such as hypertrichosis or hyperpigmentation were observed. Histologic examination of a skin biopsy specimen showed bundles of hyperplastic smooth muscle tissue within the reticular dermis which were associated with hair follicles. Karyotyping did not reveal any structural or numeric chromosomal anomaly. The findings led to the diagnosis of congenital smooth muscle hamartoma. On follow-up at the age of 24 months myokymia persisted, but with a marked reduction in the number of attacks per day. The phenomenon of myokymia results from neurally induced spontaneous contraction of dermal smooth muscles. It is also known as "pseudo-Darier's sign." While most of those affected previously reported had hypertrichosis or hyperpigmentation, this patient seems to be the first to present with myokymia alone.

[The influence of a new surface treatment of silicone intracoular lenses with fluoralkylsitan on the adherence of endophthalmitic bacteria in vitro]]. / Effekt einer neuen Oberflächenbehandlung von Intraokularlinsen aus Silikon durch ein Fluoralkylsilan auf das Adhärenzverhalten Endophthalmitis-relevanter Bakterien in vitro.

INTRODUCTION: Dynasilan is a fluoroalkylsilan which is able to bind to surface active molecules of intraocular lenses (IOLs), thereby offering a new option for surface modification of silicone lenses. The purpose of this in vitro study was to investigate the influence of this new surface treatment on the adherence of two typical endophthalmitis-inducing bacteria ( Staphylococcus epidermidis, Propionibacterium acnes). MATERIALS AND METHODS: A total of 14 Dynasilan-treated and 14 untreated silicone lenses were incubated at 37 degrees C for 24 h in brain heart infusion broth (10(8) CFU/ml) either with Staphylococcus epidermidis or with Propionibacterium acnes for 1 h. Subsequently, the adherent bacteria were resuspended using ultrasonification at 35 kHz for 3 x 45 s. After a dilution series and incubation at 37 degrees C for 24 h or 3 days the colonies were counted. RESULTS: On untreated IOLs incubated with Staphylococcus epidermidis the average number of bacteria was 3.6 x 10(7)/ml, and on treated IOLs the number of counted colonies was reduced to 1.09 x 10(7)/ml. Incubated with Propionibacterium acnes the average number of adherent bacteria on untreated IOLs was 4.75 x 10(4)/ml and on modified IOLs the number was reduced to 2.94 x 10(4)/ml. CONCLUSION: Dynasilan surface treatment may reduce the adherence of Staphylococcus epidermidis and Propionibacterium acnes on silicone intraocular lenses. Further studies regarding the stability of this treatment, its biocompatibility and influence on lens epithelial cell adhesion are in progress.

[Neuromonitoring of the recurrent laryngeal nerve--a critical analysis of patients with postoperative nerve palsy]. / Neuromonitoring des Nervus recurrens - kritische Betrachtung bei Patienten mit postoperativer Recurrensparese.

The transligamental intraoperative neuromonitoring of the recurrent nerve is established in many surgical clinics as an useful and reliable technique. It is especially suitable to identify the recurrent nerve and to monitor its function during thyroid operation. We have analysed 14 early postoperative recurrent nerve palsies between June 1997 and December 2000 (1,23 % related to nerves at risk). As a result of complete follow up we found 4 permanent nerve palsies (0,35 % related to nerves at risk). In 11 out of 14 cases the neuromonitoring revealed a dysfunction of the recurrent nerve. The following long distance microsurgical preparation showed no interruption of the continuity of the nerve. Knowing the fact of unilateral negative monitoring signal we adapted our operative strategy. Therefore we did not observe bilateral nerve palsies. In 3 patients we found a positive nerve signal but nevertheless these patients showed postoperative unilateral recurrent palsies. The possible reasons are discussed. We are convinced that the neuromonitoring of the recurrent laryngeal nerve is an important progress in thyroid surgery.

[Intraoperative neuromonitoring of the recurrent laryngeal nerve--routine use in thyroid gland surgery]. / Intraoperatives Neuromonitoring des Nervus laryngeus recurrens--routinemässiger Einsatz in der Schilddrüsenchirurgie.

We present a simple and reliable method for identification of the recurrent nerve by intraoperative stimulation. An electromyographic record from the vocal muscle is obtained using a bipolar needle electrode inserted through the cricothyroid membrane. The recurrent nerve could be easily identified in 176 cases out of 181 nerves at risk (97.2%). We have not seen any cases of recurrent nerve paralysis and discuss the advantages of intraoperative neuromonitoring as a routine procedure.