[Show abstract][Hide abstract] ABSTRACT: A 6-year-old girl with type VI skin presented with a 2-year history of multiple mildly pruritic, hard nodules on the labia majora, which did not respond to topical steroids or antibiotics given in the community. Some of these resolved spontaneously with hyperpigmentation. There was no history of sexual abuse or trauma. She was systemically well and had no other past medical history. On examination there were several firm, nontender, white-topped papules bilaterally on the labia majora with several hyperpigmented macules. There was no obvious inflammatory component. Histology revealed a giant- cell reaction surrounding calcific debris. No epithelial structures were identified and this was thought to be consistent with dermal calcified nodules. Her calcium and phosphate levels and full blood count were within normal limits and there was no evidence of connective tissue disease. Calcium deposition can be dystrophic, metastatic, iatrogenic or idiopathic. Dystrophic calcinosis is often seen as a consequence of connective tissue disease or metabolic abnormalities. Idiopathic calcinosis cutis is much less common and involvement of the vulva is very rare, particularly in children. Thus far there have been limited cases reported in the literature of idiopathic calcinosis cutis of the vulva, very few of which are in children. Idiopathic calcinosis cutis is defined by abnormal deposition of calcium in the dermis in previously normal tissue without tissue damage, in the context of normal calcium and phosphorus levels with no underlying disease. It typically affects only one area. The aetiology is unknown. Proposed mechanisms include calcification of inflamed epidermal cysts or an unknown trigger resulting in deposited apatite crystals of calcium phosphate. These are seen only on electron microscopy and might act as a source of injury leading to secondary deposition of acid mucopolysaccharides, which become a matrix for crystal formation and calcification (Cornelius CE, Tenenhouse A, Weber JC. Calcinosis cutis. Metabolic, sweat, histochemical, X-ray diffraction and electron microscopic study. Arch Dermatol 1968; 98: 218). In conclusion, we report the case of a young girl with idiopathic calcinosis cutis of the vulva, which is a very rare entity especially in childhood. Recognition and diagnosis are important in order to rule out a sexually transmitted disease, sexual abuse, abnormalities of calcium and phosphate metabolism or connective tissue diseases.
British Journal of Dermatology 07/2015; 173(Suppl S1):163. DOI:10.1111/bjd.13819 · 4.10 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Details of evidence are given in the text, and summarized in Table 3. Tinea capitis is a common scalp infection, seen predominantly in childhood. The clinical presentation is highly variable and dependent on the causative organism. The condition is most commonly due to Microsporum and Trichophyton dermatophyte species, with T. tonsurans now accounting for the majority of scalp isolates in the U.K.
British Journal of Dermatology 09/2014; 171(3):454-63. DOI:10.1111/bjd.13196 · 4.10 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Generalized pustular psoriasis (GPP) is a rare and severe variant of psoriasis. We report a case of a 79-year-old woman who presented with generalized pustular psoriasis and significant Epstein–Barr virus (EBV) viraemia. Serial measurements of EBV DNA showed a correlation with the deterioration in her clinical condition. We speculate that EBV reactivation triggered the development of GPP, and propose that further investigation is required into the association between EBV and GPP.
[Show abstract][Hide abstract] ABSTRACT: INTRODUCTION: Sun exposure is a major risk factor for the development of skin cancer. This is particularly relevant in immunosuppressed liver-transplant recipients (LTRs). Preventative strategies may help minimize the skin-cancer risk in this patient group. METHODS: We assessed 670 patients in our post-transplant clinic, using questionnaires. Patient data were collected, and we assessed whether patients had received education (such as formal talks or information from transplant coordinators or from hepatologists) on skin, sun exposure and skin cancer. In a subset of 280 of the LTRs who responded, we recorded their recall of sun-protection advice and assessed the level of patient adherence to such advice. RESULTS: The response rate was 57.5% (349/607), with a mean responder age of 51.1 years (range 19-84) and an average post-transplant time of 7.1 years (range 0-27). In the recall assessment, 37.2% reported that they were given advice about their skin, while 18.1% were seen by a dermatologist, and education on sun exposure and the risks of skin cancer was given to 65.6% and 47.9%, respectively. Over three-quarters (78%; 185/280) of the patients used mechanical sun protection (i.e. hats/clothing), while 66% reported using sunscreen; 31.8% of these used a sunscreen of the recommended sun protection factor (SPF) of > 30. Twelve patients had developed squamous cell carcinoma after a mean of 10.9 years (1-23) post-transplant; half of these had used either no sunscreen or one with an SPF of < 15. CONCLUSIONS: Despite the fact that LTRs are given information on sun-exposure and SC before and after transplantation, recall of such advice and use of sun-protection methods was only moderate, indicating that regular reinforcement of SC education is needed.
[Show abstract][Hide abstract] ABSTRACT: Background Drug reaction with eosinophilia and systemic symptoms (DRESS) describes a heterogeneous group of severe adverse reactions to medications. The cutaneous phenotype has a number of guises, accompanied by a variety of systemic features including fever, haematological abnormalities and visceral involvement, most commonly the liver. Clinical markers of prognosis have not been identified.
Objectives To assess the cutaneous signs and dermatopathological features of DRESS in order to identify potential prognostic markers.
Methods We reviewed the clinical features, dermatopathology and outcomes of 27 consecutive cases of DRESS presenting to a single unit.
Results Four distinct patterns of cutaneous involvement were identified: an urticated papular exanthem (13/27 patients), a morbilliform erythema (three of 27), an exfoliative erythroderma (three of 27) and an erythema multiforme-like (EM-like) reaction consisting of atypical targets (eight of 27). All patients mounted a fever, most developed lymphadenopathy (24/27) and peripheral eosinophilia (25/27) and the most common organ involved was the liver (27/27). Review of the dermatopathic features of patients with DRESS demonstrated a superficial spongiotic dermatitis in the majority of cases (16/27). A smaller number of cases showed basal cell vacuolar degeneration and necrotic keratinocytes (nine of 27). The patients with these biopsy findings more commonly had an EM-like cutaneous phenotype, and more severe hepatic involvement. Three patients died, two following failed liver transplants.
Conclusions Our series is the first in which a detailed dermatological assessment has been made of consecutive patients presenting with DRESS, and the largest U.K. series to date. Our results suggest a possible prognostic role of the cutaneous and dermatopathic findings in DRESS in predicting the severity of visceral involvement in this syndrome.
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British Journal of Dermatology 10/2012; 168(2). DOI:10.1111/bjd.12081 · 4.10 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Kerion celsi is the inflammatory extreme of tinea capitis, representing a delayed hypersensitivity reaction to the causative dermatophyte. Some authors have advocated the use of oral corticosteroids in patients with kerion formation to inhibit the host inflammatory response and minimize the risk of scarring. This retrospective study analyzed the management and outcome of all children younger than 10 years old presenting to our pediatric dermatology service with tinea capitis resulting in kerion formation between 2003 and 2009. We propose that kerion treatment be directed toward the underlying dermatophyte. Oral and intralesional corticosteroids are an unnecessary adjunct to oral antifungal therapy for children with tinea capitis presenting with kerion in urban areas.
[Show abstract][Hide abstract] ABSTRACT: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, life-threatening, drug-induced illness characterised by a widespread polymorphic eruption, fever and multivisceral involvement. There is little published on the management of DRESS. Prompt recognition and withdrawal of the causative drug is essential, along with supportive treatment. However, the condition commonly progresses despite these measures. Oral corticosteroids are usually given but the response can be suboptimal and result in a prolonged exposure to systemic glucocorticoid. We conducted a prospective single-centre study to determine the efficacy of pulsed intravenous methylprednisolone followed by a short reducing course of oral prednisolone in ten patients with confirmed DRESS. Rash and fever responded rapidly to methylprednisolone in all patients. Compared to pre-treatment assessments, there was a significant reduction in eosinophil count at day 14 and AST level at day 90 post-treatment. One patient developed acute hepatic failure, necessitating a liver transplant, and died 4 months later. In the immediate post-treatment phase, 1 patient developed type 1 diabetes and 1 patient developed a corticosteroid-induced psychosis. Long-term follow-up on 8/10 revealed all patients to be well, although one patient had persistent pruritus. An aggressive corticosteroid regimen in the management of DRESS is associated with good clinical outcome and acceptable tolerance.
European journal of dermatology: EJD 04/2011; 21(3):385-91. DOI:10.1684/ejd.2011.1300 · 1.95 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Alagille syndrome (arteriohepatic dysplasia) is a genetic disorder with autosomal dominant transmission which has been localized to chromosome 20p. Cutaneous manifestations include jaundice, pruritus, and widespread xanthomata. We report a child with severe Alagille syndrome in whom orthotopic liver transplantation caused rapid resolution of disfiguring xanthomas.
[Show abstract][Hide abstract] ABSTRACT: There is currently an epidemic of tinea capitis in urban areas of developed countries caused by Trichophyton tonsurans. Recurrence or re-infection with dermatophyte is not uncommon after adequate oral treatment. Asymptomatic carriers who are household contacts may partly explain this observation by forming a reservoir for infection.
Two-hundred and nine household contacts of patients with tinea capitis were examined and screened for asymptomatic carriage of dermatophyte.
Only 7.2% had clinically evident disease yet 44.5% had silent fungal carriage on the scalp. Children under 16 years were much more likely to be carriers than adults (P < 0.001) and males were less likely than females to be affected (P < 0.01).
This evidence poses questions about factors relevant in transmission of dermatophytes. The authors propose that all household contacts of patients with tinea capitis should be offered screening to eradicate a potential reservoir of infection.
Journal of the European Academy of Dermatology and Venereology 10/2007; 21(8):1061-4. DOI:10.1111/j.1468-3083.2007.02173.x · 3.11 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: In humans, Saccharomyces cerevisiae (baker's yeast) is found infrequently as a commensal of mucosal surfaces and rarely causes infections. We describe a case of cutaneous septic emboli developing in a patient with relapsing acute myeloid leukaemia M6 who had recently been treated with clofarabine. Yeast forms were seen on skin biopsy and S. cerevisiae was isolated from her Hickman line. We are not aware of any previous case reports of cutaneous emboli associated with this organism.
[Show abstract][Hide abstract] ABSTRACT: The surgical advances made in the area of organ transplantation along with the use of more efficacious immunosuppression have meant an increase in patient survival. This longer-living transplant population has started to exhibit cutaneous problems, some of which lead to an increased mortality while others lead to a decline in the quality of life.
The primary objective was to determine the different types of cutaneous lesions encountered in the adult liver transplant population. Secondary objectives were to determine the impact, if any, of the duration of transplant, the type of immunosuppression involved and the degree of sun exposure and skin phototype, on the skin cancers encountered in this transplanted population.
Two dermatologists examined 100 consecutive liver transplant recipients (LTRs) attending the transplant outpatient department. Skin examination included the face and whole body and lesions found were categorized into the following groups: cutaneous malignancies, squamoproliferative lesions, cutaneous infections and others that did not fall into any of these categories.
The reasons for organ transplantation were numerous. The mean age at transplantation was 42.5 years. The average time since transplantation was 5.5 (range 0.75-16 years). Four patients developed skin cancers; among them there were a total of seven skin cancers (one squamous cell carcinoma, six basal cell carcinomas). Fungal infections accounted for 19% of all cutaneous infections seen, viral infections 2% and bacterial infections 5%. Triple-drug immunosuppressive therapy (ciclosporin A, azathioprine and prednisolone) was used in 35% of LTR patients, while dual therapy (tacrolimus and prednisolone) was used in 48% and monotherapy (tacrolimus) was used in 17% of LTRs.
Immunosuppressive therapy is believed to be one of the most important risk factors in the development of skin cancer in solid organ transplant recipients. The relatively low prevalence of skin cancer in our liver transplant population may in part be explained by the relatively high percentage of recipients on dual and monotherapy (48% and 17% respectively), and the shorter duration of therapy. Our study suggests that although LTRs are at higher risk of developing nonmelanoma skin cancer than the general population, the risk is comparable with other solid organ transplant recipients.
British Journal of Dermatology 06/2006; 154(5):868-72. DOI:10.1111/j.1365-2133.2006.07154.x · 4.10 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Molluscum contagiosum is a common viral infection of the skin that frequently affects children. Lesions take between 6 and 18 months to resolve spontaneously and are a source of great embarrassment to both caretakers and children, often affecting attendance at school and limiting social activity. Treatment options to date have been poorly tolerated by children but recent studies have suggested that potassium hydroxide may be beneficial. This double-blind, randomized, placebo-controlled study compared 10% potassium hydroxide with placebo (normal saline). Twenty patients, aged 2 to 12 years, were recruited. Parents applied a solution twice daily to lesional skin until signs of inflammation appeared. Children were examined by the same observer on days 0, 15, 30, 60, and 90. Seventy percent of children receiving topical potassium hydroxide cleared, compared with 20% in the placebo group. Further dosing studies are required to identify whether weaker concentrations of potassium hydroxide are as efficacious, with less irritancy.
[Show abstract][Hide abstract] ABSTRACT: We report the rare instance of four family members with numerous cutaneous lesions of Leishmania major contracted while on holiday in Algeria. Treatment was successful with oral itraconazole for the children and intralesional sodium stibogluconate for the mother. Cutaneous leishmaniasis should be considered in those with apparently sterile plaques returning from endemic areas. These results suggest that itraconazole, which is ideally suited for use in children, is an effective monotherapy for L. major.