Children with dermatological conditions admitted to paediatric intensive care: analysis of a national clinical audit database.

There is little published literature about dermatological conditions in paediatric intensive care units (PICUs). The aim of this study was to describe the range of skin disorders in children admitted to PICUs in the UK and Ireland using data from a national audit. An analysis was conducted using data for 2002 - 2010 from the Paediatric Intensive Care Audit Network (PICANet). In total, 999 admissions of 882 children were identified, representing 0.8% of all PICU admissions. The most frequent dermatological conditions were skin infections, including cellulitis and necrotizing fasciitis, and inflammatory conditions. In 28% of cases, the dermatological diagnosis was considered the reason for PICU admission, in 35% it was a manifestation of systemic disease and in 37% it was considered incidental. Overall mortality was similar to the general PICU population, with 52 deaths (5.2%), but was greater in children with vascular/haematological conditions.

A case of focal dermal hypoplasia (Goltz) syndrome with exophytic granulation tissue treated by curettage and photodynamic therapy.

Focal dermal hypoplasia (Goltz) syndrome is a rare genetic disorder characterized by cutaneous, ectodermal and mesodermal defects. We present a case in which painful, exophytic granulation tissue has been the main symptom over the past 15 years. After unsatisfactory results with a number of treatment modalities including topical steroids, silver-nitrate applications, cryotherapy, curettage, excision and pulsed-dye laser, we achieved significant benefit with curettage in combination with photodynamic therapy. Although impaired wound healing has been described in focal dermal hypoplasia, this is, to our knowledge, the first time that pyogenic granuloma-like lesions have been reported.

Glaucoma induced by periorbital topical steroid use--a rare complication.

Eye complications arising during systemic and ophthalmic steroid use are well known. In contrast, there is little highlighting the similar risks associated with topical steroid use around the eyelids. We now describe a 29-year-old lady who used topical steroids in prolonged fashion throughout most of her life for severe eczema, with the recent application of large quantities of potent steroid continuously for one month, including to the periorbital region. Soon after, she presented to her ophthalmologist with severe bilateral glaucoma and irreversible visual loss attributed to the steroid use. Here we emphasise the possible risks of periorbital topical steroid use, as well as the importance of patient education and ophthalmological follow-up.

Cutis verticis gyrata of the scalp in a patient with autosomal dominant insulin resistance syndrome.

Cutis verticis gyrata (CVG) is a rare disorder; it is characterized by thickening of the scalp which becomes raised to form ridges and furrows resembling the cerebral gyri. We report a case of CVG associated with the autosomal dominant insulin resistance syndrome. This syndrome is characterized by obesity, mild mental retardation, delayed puberty, acanthosis nigricans and hyperinsulinaemia. The association of CVG and autosomal dominant insulin resistance has not been previously described.

Childhood dermatitis herpetiformis: an unusual presentation.

Childhood dermatitis herpetiformis (DH) is rare. The true prevalence and incidence of this condition are unknown. We report a 7-year-old boy presenting with nonpruritic inflammatory papules on the buttocks and extensor surfaces, clinico-pathologically consistent with Sweet's neutrophilic dermatosis. Immunofluorescence studies showed IgA deposits in the dermal papillae consistent with DH. Remission was achieved with a gluten-free diet and dapsone. Childhood DH may present different clinical signs to the adult form and misdiagnosis can occur if immunofluorescence is not requested on skin biopsy.

Xanthoma disseminatum: a case with hepatic involvement, diabetes insipidus and type IIb hyperlipidaemia.

Xanthoma disseminatum (XD) is a rare benign non-X-histiocytic disorder of unknown aetiology. We report a 37-year-old man who presented with XD preceded by a decade of cranial diabetes insipidus, with associated type IIb hyperlipidaemia and computed tomographic evidence of hepatic involvement. A review of the literature is also included.

The prevalence of skin disease in HIV infection and its relationship to the degree of immunosuppression.

A cross-sectional study of human immunodeficiency virus (HIV) positive patients who attended the HIV clinic in Brighton over a 4-month period was carried out to describe the prevalence and severity of skin manifestations in HIV-positive patients and to elucidate their association with the peripheral CD4 cell count and with the HIV disease stage. The subjects were consecutively examined by an experienced dermatologist. Skin manifestations were classified into infections, dermatoses, pruritus and neoplasm. A severity index was derived by scoring each condition as either absent, mild, moderate or severe. One hundred and fifty-one patients were enrolled with a mean age of 38.3 years. One hundred and thirty-nine were homo/bisexual men; 58 were asymptomatic and 35 had acquired immune deficiency syndrome (AIDS); 37 had CD4 counts below 200. Skin conditions were present in 138 of the 151 subjects (91.4%). The total number of events was 331. The most frequent problem was infection followed by dermatoses, pruritus and malignancy. The most frequent condition was seborrhoeic eczema followed by tinea and xerosis. We have demonstrated a statistically significant association between CD4 count, disease stage and skin manifestations in HIV-positive individuals.

Unilateral naevoid telangiectasia syndrome in pregnancy.

We report the case of a 22-year-old woman presenting with the unilateral naevoid telangiectasia syndrome in her third pregnancy. The development of this is well documented but the occurrence in a third pregnancy is unusual. A brief review of the literature is included.

Safety and efficacy of calcipotriol ointment (Dovonex) in treating children with psoriasis vulgaris.

Calcipotriol (Dovonex) ointment has been shown to be an effective, well tolerated, and acceptable treatment for psoriasis vulgaris in adults. This open study was conducted in 16 U.K. centres to assess the safety and efficacy of calcipotriol ointment in treating psoriasis vulgaris in children. Following a 2-week washout, patients were treated with calcipotriol ointment, 50 micrograms/g twice daily, for up to 8 weeks. A blood sample was taken on entry and a second 'on treatment' sample was taken after either 2 or 8 weeks treatment. Sixty-six children (26 boys, 40 girls, age range from 3 to 14 years) entered and 58 completed the study. There was a statistically significant reduction in the mean (+/- SD) Psoriasis Area and Severity Index (PASI) from 6.1 +/- 3.5 at the start of treatment to 2.7 +/- 1.9 at the end of treatment (P < 0.001). Marked improvement or clearance of psoriasis at the end of treatment occurred in 65% of patients as assessed by the investigator and 62% as assessed by the patient. Cosmetic acceptability of calcipotriol ointment was found to be good or excellent in 79% of patients. Eight patients withdrew from the study (four defaulted, two unacceptable responses, two adverse events). Adverse events were reported by 16 patients: the most common being local irritation (seven patients). There was no significant change in the mean serum ionized calcium from baseline to 2 or 8 weeks treatment. Similarly, there were no consistent or clinically important changes in haematological, or other biochemical parameters, measured during the study period. Calcipotriol ointment has been shown to be an effective, well tolerated, and acceptable treatment for psoriasis vulgaris in children.

Phaeohyphomycosis caused by Exophiala dermatitidis following intra-articular steroid injection.

A patient with long-standing rheumatoid arthritis presented with a painful pigmented chronic nodule on the dorsum of the right hand, at the site of intra-articular steroid injections undertaken 5 years previously. Histology showed pigmented fungal elements consistent with phaeohyphomycosis. Cultures yielded black yeast-like colonies, identified as Exophiala dermatitidis and sensitive to itraconazole and amphotericin. A 1-month course of itraconazole resulted in marked clinical improvement but surgical excision and skin grafting were required for complete resolution. Phaeohyphomycosis has been related to inoculation injury but association with intra-articular steroid injection appears hitherto to be unreported.

Comedonal Darier's disease.

Darier's disease is an inherited disorder with well-recognized patterns of presentation. Lesions commonly affect the trunk and flexures. The diagnosis is based on the typical clinical appearance and histology showing acantholytic dyskeratosis. We report two unusual cases with prominent nodular, comedonal lesions on the face and scalp.

The use of cytology in the diagnosis of basal cell carcinoma.

Two hundred and forty patients with basal cell carcinomas (BCCs) on the head or neck were studied. Scrapings of the lesions were taken for cytological examination, and a 3-mm punch biopsy was performed for histopathological study. The accuracy of diagnosis by each method was compared. Both methods confirmed the clinical diagnosis in 226 cases, and both were negative in 10 cases. Cytopathology gave one false negative result (0.42%), and histopathology gave two false negative results (0.83%). Cytopathology gave one false positive result (0.42%), and histopathology did not produce any false positive results. We conclude that cytological examination of skin scrapings from suspected BCCs is a rapid and reliable method of diagnosis.