Although most infantile haemangiomas do not require treatment due to a natural history of spontaneous involution, some require early intervention. The Australasian Vascular Anomalies Network and the Australasian Paediatric Dermatology Network have developed a consensus statement for the treatment of infantile haemangiomas with oral propranolol. Infants with haemangiomas that are life threatening, at risk of ulceration, or at risk of causing a significant functional impairment, psychological impact or physical deformity should be treated early with oral propranolol. Oral propranolol is safe and effective and in most healthy infants oral propranolol can be started in an outpatient setting.

Avian mite infestation is a rare cause of pruritic dermatoses in humans. The mites spend the majority of their life cycle on the avian host but may be transmitted to man as a result of direct contact and also through airborne spread. We describe a case of infestation with the Northern Fowl Mite (Ornithonyssus sylviarum) from an abandoned birds nest in the roof of a patients home. This caused a pruritic erythematous papular eruption on exposed sites that settled with topical steroids. We discuss the diagnosis and range of clinical manifestations produced by avian mites.

A 3 1/2-year-old boy presented on three occasions with painful, itchy, oedematous plaques on his limbs. On two occasions he had received hepatitis B vaccination 11-13 days previously, and on the third occasion received triple antigen (DTP) vaccination 10 days earlier. Skin biopsy revealed a prominent infiltrate of eosinophils involving the entire thickness of the dermis. In addition there were prominent 'flame figures' consisting of eosinophilic necrotic collagen surrounded by granular basophilic debris. The clinical and histological pictures were consistent with Wells' syndrome. The eruption settled on the second and third occasions with 0.1% mometasone furoate cream. Subsequent patch testing showed 2+ reaction to preservative thiomersal at 96 hours. This is the first description of Wells' syndrome with typical clinical and histopathological features associated with thiomersal in two different vaccines.

BackgroundThe lack of validated outcome measures for epidermolysis bullosa (EB) presents major barriers to evaluating disease severity and comparing the efficacy of therapies. The Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) was recently introduced as a valid and reliable instrument for EB; however, its interpretation for use in clinical practice and clinical trials is yet to be defined.ObjectiveTo assess the interpretability of the EBDASI in classifying patients according to disease severity and clinical response.MethodsA total of 53 outpatients with EB at two interstate institutions were prospectively evaluated. At each visit, the principal dermatologist completed the EBDASI and global assessments of disease severity and change. Classifications for mild, moderate and severe disease using the EBDASI were determined using receiver operating characteristic curves. Minimal clinically important differences for the EBDASI activity subscale were calculated and compared with the standard error of measurement.ResultsTotal EBDASI score ranges of 0–42, 43–106 and 107–506 corresponded to mild, moderate and severe disease respectively. Reduction in EBDASI activity scores of greater than 9 indicated clinically significant improvement. An increase of 3 in the activity score indicated deterioration.ConclusionThe EBDASI is a responsive tool and may be useful in characterizing disease severity and response. The cut‐offs proposed in this study provide the first practical guide for interpreting the EBDASI, further supporting its use for longitudinal patient assessment and in clinical trials.

Erythropoietic protoporphyria is a rare photodermatosis for which treatment options are limited. The present report describes the clinical features of a patient with erythropoietic protoporphyria and liver function test abnormalities associated with treatment with beta-carotene. Subsequent treatment with narrow-band UVB phototherapy resulted in marked subjective improvement in photosensitivity, which was confirmed by abolition of demonstrated abnormalities on monochromator phototesting. The therapeutic options for photosensitivity in erythropoietic protoporphyria are reviewed and discussed.

Ticks are blood-sucking parasites of vertebrates that may embed in human skin and are therefore of clinical relevance to dermatologists and their medical colleagues. Depending on the species involved, consequences of tick attachment vary from minor local reactions to significant systemic sequelae. It is possible to minimize morbidity by removing the tick in its entirety as soon as it is detected. Some techniques to achieve this are described. This review will aid clinicians in the recognition and practical management of tick bites in Australia.

Ectodermal dysplasia-skin fragility syndrome (ED-SFS) is a rare autosomal recessive genodermatosis resulting from mutations in the PKP1 gene, encoding the desmosomal plaque protein plakophilin-1 (PKP1). Mutations in PKP1 may manifest with skin fragility and erosions, patches of scale crust on the trunk and limbs, peri-oral cracking and inflammation, hypotrichosis, palmoplantar keratoderma with painful fissuring and other somewhat variable ectodermal anomalies. Ten cases of the syndrome have been reported. We report a further case of this desmosomal genodermatosis. A 14-month old child, born to consanguineous parents, presented with a history of neonatal bullae and subsequent development of dystrophic nails, sparse eyelashes and eyebrows, woolly scalp hair, abnormal dental development and a desquamating erythematous rash at sites of trauma. A clinical diagnosis of ED-SFS was supported by skin biopsy findings of suprabasal intraepidermal clefting and a loss of immunoreactivity for PKP1. Sequencing of genomic DNA revealed a homozygous 5 base pair deletion in exon 5 of the PKP1 gene, designated c.897del5 (CAACC). This new mutation creates a frameshift, leading to a downstream premature termination codon, p.Pro299fsX61. This case highlights the clinicopathological consequences of inherited mutations in the PKP1 gene and illustrates the key role of desmosomes in skin biology.

The spectrum of disease requiring specialist dermatology input varies between urban South Australia and rural NT. The inclusion of dermatology trainees in outreach visits broadens their clinical exposure. It is recommended that other dermatology service providers in Australia consider documenting clinical casemix comparisons to assess dermatology demand, outcomes and trainee exposure.