Our aim was to describe the types of psoriasis seen in a large series of patients presenting to a tertiary referral pediatric dermatology department using a classification system combining conventional terminology and additional categories based on the site and characteristics of the rash. A total of 1262 patients seen consecutively in the dermatology department of the Royal Alexandra Hospital for Children, Sydney, Australia, between 1981 and 1995 are described and classified according to the pattern of psoriasis at the time of presentation. Additional information recorded included family history, facial involvement, and history of a psoriatic type of diaper rash in infancy. The ages of the children ranged from 1 month to 15 years. There was an equal gender distribution and a high rate of positive family history at 71%. Twenty-six percent of children had a history of a psoriatic diaper rash and facial involvement occurred in 38% of children. Plaque psoriasis was the most common type overall, affecting 430 patients (34%). Three hundred forty-five children were less than 2 years of age, and this is the largest series of children with psoriasis in this age group presented to date. An entity defined by us as psoriatic diaper rash with dissemination was the most common type of psoriasis in the less than 2-year age group, affecting 155 (45%) patients. This large series offers information on the manifestations of psoriasis in childhood, but is particularly useful in examining the previously less well-described infant age group. The classification used is proposed as a practical way to describe psoriasis in children, particularly with respect to future descriptive studies.

Mutations predicted to result in the loss of RECQL4 protein function occurred in approximately two-thirds of RTS patients and are associated with risk of osteosarcoma. Molecular diagnosis has the potential to identify those children with RTS who are at high risk of this cancer.

Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive genodermatosis characterized by a poikilodermatous rash starting in infancy, small stature, skeletal abnormalities, juvenile cataracts, and predisposition to specific cancers. We have identified a contemporary cohort of 41 patients to better define the clinical profile, diagnostic criteria, and management of patients with RTS. Patients with the diagnosis of RTS were ascertained by referrals from dermatology, ophthalmology, genetics, and oncology or from direct contact with the patient's family. Medical information was obtained from interviews with physicians, patients, and their parents and a review of medical records. The age range at ascertainment was 9 months to 42 years (28 males and 13 females; M:F, 2:1). All subjects displayed a characteristic rash. Thirteen subjects had osteosarcoma (OS) (32%), eight had radial defects (20%), seven had gastrointestinal findings (17%), two had cataracts (6%), and one had skin cancer (2%). Twenty-two of 28 patients without OS were less than 15 years old and thus remain at significant risk for this tumor. This case-series study reveals a clinical profile of RTS that includes a higher prevalence of OS and fewer cataracts, compared with historical reports. These differences may reflect either allelic or genetic heterogeneity. This study documents the frequency of clinical anomalies in a contemporary cohort of RTS patients and revises guidelines for diagnosis and management of RTS.

This is a retrospective review of the case files and clinical photographs of 173 children diagnosed with cutaneous mastocytosis by a dermatologist in an exclusively paediatric practice. Of the 98 males and 75 females, 51% had mastocytomas, 47% had urticaria pigmentosa and three patients had diffuse cutaneous mastocytosis. Of these cases, 87% first appeared prior to or at 6 months of age. Flushing occurred in 26% of urticaria pigmentosa cases, 29% of mastocytomas and 100% of diffuse cutaneous mastocytosis. Blistering was noted in 23% of urticaria pigmentosa, 31% of mastocytomas and 100% of diffuse cases. Neither symptom was noted in 59% of urticaria pigmentosa and 49% of mastocytomas. There were three cases with a positive family history. The finding of a palmar mastocytoma has only once been previously reported. Illustrated descriptions of our cases are provided.

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Twenty-seven cases of infantile myofibromatosis presenting with dermatological manifestations were retrospectively reviewed. Approximately 80% were solitary lesions and 50% of these appeared on the head and neck. Around 60% were present at or soon after birth. Most lesions were dermal or subcutaneous, although some were intramuscular and intraosseous. The clinical appearance was non-specific leading to frequent misdiagnosis. While most patients presented with nodules, atrophic depressed lesions and warty pedunculated lesions were also seen. Although 7% of lesions recurred after excision, spontaneous resolution was also documented.

We evaluated 130 prepubertal girls presenting with a vulvar complaint to determine the spectrum and frequency of conditions seen in this age group. Of the patients, 41 (33%) had atopic or irritant dermatitis, 23 (18%) had lichen sclerosus, 21 (17%) had psoriasis, 15 (12%) had vulvar lesions, most often hemangiomas and nevi, and 13 (10%) had streptococcal vulvovaginitis. Diagnoses less frequently seen were staphylococcal folliculitis (four patients), labial fusion (three patients), genital warts (two patients), molluscum contagiosum of the vulva only (one patient), vulvar bullous pemphigoid (two patients), scabies nodules (one patient), erythema annulare centrifugum (one patient), tinea (two patients), and vitiligo (one patient). We also encountered vulvar presentations of systemic diseases (varicella, staphylococcal scalded skin syndrome, and Henoch-Schönlein purpura, all one patient each). We did not see candidal vulvovaginitis in this age group nor did we encounter bacterial infection with pathogens other than Staphylococcus aureus and S. pyogenes.

As in adults, artefactual skin disease in children and adolescents has heterogenous presentations with multifactorial aetiology. We report a series of 32 young patients aged 8-16 years. There were 24 females and 8 males. In over half of the cases the lesions were on the head and neck. The types of lesions encountered included physical injury producing grazing, erosions and deep ulcers, chemical and thermal burns, hair cutting and shaving and skin painting. We discuss the approach that we believe should be taken with these patients, emphasizing the role of dermatology-psychiatry liaison in their management. Of great importance is the avoidance of confrontation of the patient but the clear exposition of the nature of the problem to the parents. The major aim should be to have the family accept the need for expert psychiatric assistance.