Endocrine mucin-producing sweat gland carcinoma (EMPSGC) has recently been recognised as a low-grade carcinoma that almost always occurs on the eyelid. This carcinoma is very rare, with only 20 cases (including the present one) having been reported in the literature. EMPSGC is frequently found in association with invasive mucinous carcinoma. While EMPSGC treatments consist of a complete surgical removal, there has been no consensus regarding the surgical margin. Therefore, reports on surgical management of EMPSGC may potentially provide important therapeutic information. Here, we present a case of a 74-year-old man with EMPSGC of the eyelid that repeatedly recurred despite surgical treatments at another institution. After referral to our department, the patient underwent tumour excision. However, the specimen revealed a positive surgical margin and thus, he subsequently underwent a wider excision. There has been no sign of tumour recurrence or metastasis 6 months after his last operation. This article reviews the current literature and discusses the surgical management of EMPSGC.

Porokeratosis is a rare disorder of skin keratinisation characterised by a cornoid lamella. We reviewed its associations with immunosuppression and phototherapy, as well as the risks of malignant progression. This is a retrospective review on all cases of porokeratosis seen at the National Skin Centre, Singapore, between 2000 and 2010. A total of 94 patients were reviewed. Clinical and histological diagnoses were confirmed in 63% patients. Most patients were Chinese (89%) with a mean age of 51.6 years. The male to female ratio was 1.4:1. The four main clinical variants were classical porokeratosis of Mibelli (56%), disseminated superficial actinic porokeratosis (DSAP) (18%), disseminated superficial porokeratosis (DSP) (11%), and linear porokeratosis (13%). Phototherapy-induced porokeratosis, seen in three patients, is rare. Seven cases of porokeratosis occurred in patients who were immunosuppressed. Progression of porokeratosis to malignancy is uncommon and was observed in three patients. The most common treatments included cryotherapy (26.5%) as well as topical steroids or retinoids (38.1%). A good response, defined as clear or almost clear lesions, occurred in 16% patients. The most common presentation of porokeratosis in our review was a middle-aged male patient with an asymptomatic lesion of porokeratosis of Mibelli over the extremities. No particular immunosuppressive drug was implicated. Porokeratosis associated with ultraviolet phototherapy or malignancy is rare. Progression of porokeratosis to malignancy arose in the disseminated variants, with a possible correlation with age. This is the largest institutional retrospective review of porokeratosis to date and highlights the major epidemiological characteristics of this condition.

Background/Objectives:  Accurate identification of skin lesions is vital in ensuring malignancies are not missed and that they are treated early to avoid mortality. It is also important that appropriate lesions are submitted for biopsy to decrease the costs and morbidity associated with the unnecessary removal of benign lesions. This study attempted to assess current accuracy in skin cancer diagnosis.

Methods:  Histology reports for all biopsies and excisions performed in an 18-month period at a public hospital dermatology department were reviewed. Dermatology registrars and consultants were involved in assessing lesions for biopsy. Calculations were made to quantify the sensitivity of melanoma diagnosis; naevi to melanoma ratio (NMR); biopsy to treatment ratio (BTR), and number needed to treat for melanoma (NNT). NNT was calculated as the number of pigmented lesions (seborrhoeic keratoses, naevi and melanoma) removed to identify one melanoma.

Results:  6546 biopsies/excisions were performed, identifying 55 melanomas. The sensitivity of melanoma diagnosis was 76% and 11% of melanomas were thought to be dysplastic naevi. The NMR was 6.4. The BTR was 1.97, indicating that one in every 1.97 biopsies was identified as a non-melanoma skin cancer. The NNT was 11.9. All dysplastic naevi and 91% of melanomas were biopsied using either shave or excision biopsy.

Conclusions:  These audits are important to ensure quality of care and could aid in identifying doctors and institutions that may benefit from further training in melanoma diagnostic algorithms. These figures can be used as a benchmark to measure the impact of new vectors in skin cancer diagnosis as they are introduced.

We report the cases of two patients who developed eruptive cutaneous squamous cell carcinomas (SCC) soon after commencement of ustekinumab, as treatment of moderate to severe plaque type psoriasis. Ustekinumab is a human monoclonal antibody with a novel mechanism, selectively targeting the shared p40 subunit of interleukin-12 (IL-12) and IL-23. Its efficacy has been well documented in three large phase-III trials (PHOENIX I, PHEONIX 2, ACCEPT). Safety data on this new biological agent continue to grow. To date, no link between ustekinumab and cutaneous carcinogenesis has been demonstrated and, to our knowledge, these cases are the first of their kind. Importantly, both these patients had independent risk factors for developing non-melanoma skin cancers; however, the specific time correlation with the administration of ustekinumab is of note. Our report suggests that ustekinumab may allow the development of cutaneous carcinomata in predisposed individuals.

Background/Objectives:  The willingness to be educated is one of the highest desires among patients with psoriasis. Therefore, a collaborative model of management would appear to be essential in enhancing patient satisfaction in this challenging condition. The present study aimed at examining the applicability of a mobile teledermatology service in this regard and assessing the association between patient acceptance and perceived health-related quality of life.

Methods:  High-need patients with psoriasis performed visits over 12 weeks transmitting clinical images together with some relevant clinical information via mobile phones to teledermatologists, who provided treatment instructions. Ten patients and two teledermatologists completed 20-item patient (weeks 6 and 12) and 10-item physician (at week 12) acceptance questionnaires. In addition, patients answered the dermatology life quality index (DLQI) at weeks 0, 6 and 12.

Results:  Both patients and teledermatologists were pleased with the service with high acceptance rates (patients: 81.0% at week 6 and 82.9% at week 12; teledermatologists: 74.0%). In addition, 80% of the patients considered the service an alternative to in-person consultation and 90% felt they were in good hands but had achieved a more flexible and empowered lifestyle. No significant correlations were found between patient acceptance and DLQI. Both teledermatologists found the service a convenient and reliable tool for patient monitoring. Neither patients nor teledermatologists thought further in-person consultations necessary.

Conclusion:  Mobile teledermatology is a valuable tool for the home monitoring of patients with psoriasis that makes a meaningful difference in their lives. It is well accepted by both patients and the physicians involved.

A 35-year-old Lebanese woman presented with a 3-year history of persistent, localized livedo racemosa over her feet, distal legs and forearms that was associated with the development of lower limb sensorimotor neuropathy. Investigations revealed the patient was heterozygous for prothrombin gene mutation and was also found to have a T-cell receptor gamma chain gene rearrangement. Histological examination revealed a mid-lower dermal medium vessel lymphocytic vasculitis with prominent fibrinoid ring within its wall. These findings are consistent with a recently described condition known as lymphocytic thrombophilic arteritis. This has so far been considered to be a benign clinical condition not associated with extra cutaneous manifestations. The novel findings in the present case are the associated sensorimotor neuropathy and the characteristic fibrin ring appears to be intramural rather intraluminal in location. The findings of a T cell gene rearrangement and a prothombin gene mutation suggest that both immunological and thrombophilic factors might contribute to the development of this condition.

Background/objectives:  Several hair follicle reconstruction models based on the implantation of dissociated cells have been established. However, little has been said about the direction of growth, which is a very important issue for clinical applications. The aim of this study was to reveal the factors that determine the direction of hair follicle growth after reconstruction with dissociated cells.

Methods:  We designed an enclosed chamber assay in which a chamber was buried under nude mouse skin that was not exposed to the air, as in a traditional chamber assay. Inductive dermal cells and epithelial cells dissociated from neonatal C57 BL/6 mice were injected into the chamber and dermis for patch assays. The direction of newly formed hair follicles was analysed.

Results:  Newly formed hair follicles in the enclosed chamber were arranged randomly parallel to the skin on the surface of the panniculus carnosus.

Conclusions:  These results indicate that developmental space provides the possibility for growth of reconstructed hair follicles and that the air–liquid interface determines the final direction of growth.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy that originates from the precursors of plasmacytoid dendritic cells. It commonly presents with findings isolated to the skin although it usually progresses to a leukaemic phase. It has a poor prognosis but is curable, particularly in younger patients treated with allogeneic bone marrow transplantation. We report a case of a 79-year old man who had 6 months of progressive, asymptomatic BPDCN manifestations limited to the skin, before developing a leukaemic phase.

Eccrine angiomatous hamartoma (EAH) is a rare tumoral lesion that consists of vascular and eccrine malformation and often occurs in the distal extremities of children. Although EAH is benign, a therapeutic excision may be required for symptomatic or cosmetic considerations. We hereby report a typical case of EAH presenting as a painful and rapidly growing plaque on the right thigh. The associated symptoms of pain, hyperhidrosis and local hypertrichosis caused walking difficulties until the patient was cured by two excisional surgeries.

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.

Acute generalised exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction and is caused by drugs in >90% of cases. It is rare, with an incidence of 1–5 patients per million per year. The clinical manifestations are characterised by fever and the rapid appearance of disseminated sterile pustules 3–5 days after the commencement of treatment. It is accompanied by marked neutrophilia. Mucous membranes are not typically involved. The drugs conferring the highest risk of AGEP according to the EuroSCAR study are aminopenicillins, pristinamycin, hydroxychloroquine, antibacterial sulphonamides, terbinafine and diltiazem. The pathogenesis of AGEP involves the initial influx of CD8 cytotoxic T-cells resulting in the apoptosis of keratinocytes and formation of vesicles. Then CXCL-8-producing and granulocyte macrophage-colony stimulating factor-producing CD4 cells enter the epidermis, resulting in neutrophil mediated inflammation and the formation of pustules. As a result, the histology reveals intraepidermal, usually subcorneal, pustules and an accompanying neutrophilic and lymphocytic infiltrate. Epicutaneous patch testing may also support the diagnosis by causing a localised pustular reaction 48–96 h after the offending drug is applied. The condition usually resolves by 15 days after the causative drug is withdrawn but oral corticosteroid therapy may be necessary in some individuals. The mortality rate is up to 5% and mostly occurs in elderly people who have significant comorbidities.

Toxic epidermal necrolysis (TEN)-like presentations have been described in non–drug-induced settings. We describe a case of subacute cutaneous lupus erythematosus (SCLE) in a 53-year old woman, which evolved into a TEN-like presentation over the course of 4 weeks. The patient responded rapidly to treatment with intravenous methylprednisolone. This article draws attention to features that may be used to differentiate classical TEN from TEN-like SCLE.

Methotrexate is a synthetic folic acid analogue valued for both its anti-proliferative and anti-inflammatory properties. Considered one of the original immune-modifying agents, it is used widely for the treatment of steroid-recalcitrant inflammatory diseases. While there are abundant studies documenting its efficacy in rheumatic diseases, the use of methotrexate for dermatological conditions, with the exception of psoriasis, has yet to be comprehensively explored. This two-part review firstly outlines current data concerning the pharmacology of methotrexate, including its mechanism of action, side-effect profile and recommended therapeutic approach, and, secondly, examines the emerging evidence for methotrexate's efficacy in a wide range of cutaneous disorders.

Reported is the case of a 17-year old male with sacroiliitis confirmed by magnetic resonance imaging (MRI) while undergoing isotretinoin treatment for acne vulgaris. The cessation of isotretinoin and symptomatic treatment resolved the symptoms within 6 weeks, with no signs of sacroiliitis on repeat MRI 10 months later. The temporal association of disease onset and commencement of isotretinoin along with rapid recovery on withdrawal supports the role of isotretinoin in this case.

Tuberous sclerosis complex (TSC) is an autosomal dominant genodermatosis characterised by the development of hamartomatous tumours in multiple organs including the brain, skin, kidneys, heart and lungs. Facial angiofibromas are the most visible and unsightly of the cutaneous manifestations of TSC, often resulting in stigmatisation for both the affected individuals and their families. Current treatments include vascular laser, ablative lasers and other destructive techniques such as shave excision and electrodessication. For the best outcome these treatments have to be repeated throughout childhood and teenage years, necessitating multiple general anaesthetics. We report a pilot study of topical rapamycin in four children with TSC and facial angiofibromas. Two patients were trialled on 0.1% rapamycin in petrolatum and the other two patients with 0.1% rapamycin solution (Rapamune) applied topically. Both preparations were rapidly and equally effective, however the 0.1% in petrolatum was much better tolerated. Younger patients with smaller angiofibromas had the best response with near complete clearance. Both preparations were more cost effective than pulsed dye laser under general anaesthesia. Although larger studies are needed, this treatment shows a potential to be a first-line management for facial angiofibromas in TSC and appears safe to start in early childhood.

Background/Objectives:  A virtual lesion clinic (VLC) using store-and-forward teledermoscopy was introduced to reduce waiting times and improve access for skin lesion assessment by dermatologists.

We aimed to review the efficiency and patient acceptance of a new community-based teledermoscopy service by comparing it to hospital-based face-to-face (FTF) skin lesion clinics.

Methods:  A prospective study compared patient flow through a community-based VLC and a tertiary hospital FTF dermatology clinic. Surveys were sent to patients and their referring doctors after attendance. Waiting times, diagnosis, outcomes, financial costs, patient acceptability and convenience were compared.

Results:  A total of 300 patients were assessed; 200 were seen in the VLC and 100 in the conventional FTF clinic. Of the 200 patients seen in the VLC, 88% did not require a subsequent FTF clinic assessment to establish the diagnosis. Mean waiting times for first assessment were reduced by two thirds (from 114 days to 39 days) in those seen by the VLC compared to FTF. Financial analysis demonstrated cost savings of 14%. Surveyed patients were highly satisfied and confident in the VLC service.

Conclusions:  A community-based teledermoscopy service may allow improved management of outpatient referrals while providing a better, quicker and more convenient service. It may also provide cost savings, as teledermoscopy assessment can be cheaper than traditional assessment.

Background/Objectives:  Melanoma has become a major public health problem worldwide and its incidence in individuals of Caucasian origin continues to rise. The objective was to determine historical changes in thickness, melanoma proportions and anatomical site of presentation over a 25-year period in our Department.

Methods:  This was a historical retrospective study (January 1985 to December 2009). Only patients born and living in Italy were considered. The following parameters were evaluated: age, gender, year of diagnosis, site of primitive lesion (head, back, chest, anterior and posterior upper limbs, anterior and posterior lower limb, and acral sites) and Breslow thickness of the lesion.

Results:  In the 25-year period, 993 cases of melanoma were diagnosed. The total number of cases per year tripled between 1985–1989 and 1995–1999 and more than doubled between 1995–1999 and 2005–2009. Our results also revealed that thicker melanomas were more frequent in elderly patients and on parts of the body that cannot be readily self-inspected.

Conclusion:  The importance of observation of the posterior parts of the body is stressed, since not only did most melanomas arise in these sites but the diagnosis of lesions in these sites is often delayed.

Background:  There is an increasing trend towards early mobilisation post-split skin grafting of the lower limbs. This study was performed to determine if early mobilisation impacts negatively on graft healing and patient morbidity.

Methods:  A retrospective review of 48 cases of lower limb split skin grafts performed by the plastic surgery department at Royal Perth Hospital was undertaken. Patients were stratified into early and late mobilisation groups.

Results:  No difference in outcome was identified with early mobilisation, but an increased rate of deconditioning with increased length of stay was present with late mobilisation.

Conclusion:  These results suggest that early mobilisation post-split skin grafting of the lower limb is beneficial to patient care and is associated with lower morbidity.

Lung carcinoma is one of the most frequent sources of skin metastases in male patients. Our objective was to analyse the clinical and pathological features of 30 patients with skin metastases from lung carcinoma. Cutaneous biopsies codified as ‘skin metastasis from lung carcinoma’ during 1988–2009 at Bellvitge Hospital (Barcelona, Spain) were reviewed. The histological types of 30 lung carcinomas (29 men, 1 woman) were squamous cell carcinoma (10 cases), undifferentiated carcinoma (7), adenocarcinoma (6), small cell carcinoma (5) and large cell carcinoma (2). The most frequent clinical presentation was as a solitary nodule (16 cases), and the most frequent site was the head (13 cases). Cutaneous metastases were present at the time of diagnosis of the lung primary tumour in 66% of cases. Skin biopsy might be helpful to establish the histological type of tumour, and thus help with therapeutic decision-making. Cutaneous metastases from lung cancer remain a poor prognostic feature.