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.