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Primary cutaneous mucinous carcinoma (PCMC) is a rare adnexal tumour of the skin. Clinically, it appears as a benign cyst, but it has characteristic histopathology. It is a slow-growing tumour that rarely metastasizes but is associated with significant morbidity due to its high recurrence rate. Standard practice has been to surgically remove it with a wide local excision of 1–2-cm margin. In the last decade, increasing reports of Mohs micrographic surgery (MMS) for the treatment of PCMC have been described. MMS appears to reduce recurrence rates while allowing for more conservative margins. Given the rarity of PCMC, there are no prospective randomized control trials on treatment. This is the largest case series of PCMC treated by MMS to date.

Skin cancer is the most common cancer in the UK, and incidence rates are continuing to increase. We discuss the common presentations, clinical features, examination techniques, referral guidelines, management and prognosis of both non-melanoma skin cancer (basal cell carcinoma, squamous cell carcinoma) and melanoma.

UNSTRUCTURED Skin cancer is the most common type of cancer in the UK, with over 100,000 cases diagnosed each year. Melanoma is a type of skin cancer that can develop from a mole. However, moles that are suspicious histologically are often difficult to diagnose clinically by eye, even for experts working in specialist skin cancer screening clinics. In the UK, 350,000 patients per year are referred to hospital clinics with suspicious moles, and approximately half undergo a biopsy to identify the 5-10% who require further treatment. If cancer cannot be ruled out clinically and on the basis of biopsy results, the lesion is surgically removed. One type of pre-cancerous mole, called lentigo maligna, is particularly challenging to treat. It develops insidiously, can become large, and is difficult to assess when it transforms into a lentigo maligna melanoma. It is also difficult to identify the margins by eye or with the aid of a dermatoscope. This results in high excision rates. Excision is often incomplete despite large areas of tissue removal (5 mm around tumour’s visible margin), and there are high recurrence rates even when the lesion is considered successfully removed. Reflectance confocal microscopy is an imaging technique that can supplement dermoscopy in identifying if a clinically suspicious mole is malignant and can better assess lentigo maligna margins. It allows clinicians to see the skin lesion in greater detail than dermoscopy and therefore better guide more accurate diagnoses. This prospective study will examine patients with suspicious moles and lentigo maligna to determine whether confocal microscopy can both reduce the number of unnecessary biopsies of moles and more accurately guide the surgical excision margins of lentigo maligna.

Optical coherence tomography (OCT) has been shown to be of clinical value in imaging basal cell carcinoma (BCC). A novel dual OCT-video imaging system, providing automated registration of OCT and dermoscopy, has been developed to assess the potential of OCT in measuring the degree of sub-clinical spread of BCC. Seventeen patients selected for Mohs micrographic surgery (MMS) for BCC were recruited to the study. The extent of BCC infiltration beyond a segment of the clinically assessed pre-surgical border was evaluated using OCT. Sufficiently accurate (2.5 mm. In addition, the OCT border lay between 0.5 mm and 2.0 mm inside the final MMS defect in those cases where this could be assessed. In one case, where the final MMS defect was over 17 mm from the clinical border, OCT showed >2.5 mm infiltration across the clinical border at the FOV limit. These results provide evidence that OCT allows more accurate assessment of sub-clinical spread of BCC than clinical observation alone. Such a capability may have clinical value in reducing the number of surgical stages in MMS for BCC. There may also be a role for OCT in aiding the selection of patients most suitable for MMS.

“Mutational signatures” are patterns of mutations that report DNA damage and subsequent repair processes that have occurred. Whole-genome sequencing (WGS) can provide additional information to standard diagnostic techniques and can identify therapeutic targets. A 32-yr-old male with xeroderma pigmentosum developed metastatic angiosarcoma that was unresponsive to three lines of conventional sarcoma therapies. WGS was performed on his primary cancer revealing a hypermutated tumor, including clonal ultraviolet radiation-induced mutational patterns (Signature 7) and subclonal signatures of mutated DNA polymerase epsilon (POLE) (Signature 10). These signatures are associated with response to immune checkpoint blockade. Immunohistochemistry confirmed high PD-L1 expression in metastatic deposits. The anti-PD-1 monoclonal antibody pembrolizumab was commenced off-label given the POLE mutation and high mutational load. After four cycles, there was a significant reduction in his disease with almost complete resolution of the metastatic deposits. This case highlights the importance of WGS in the analysis, interpretation, and treatment of cancers. We anticipate that as WGS becomes integral to the cancer diagnostic pathway, treatments will be stratified to the individual based on their unique genomic and/or transcriptomic profile, enhancing classical approaches of histologically driven treatment decisions.

Xeroderma pigmentosum (XP) is a rare DNA repair disorder characterized by increased susceptibility to UV radiation (UVR)-induced skin pigmentation, skin cancers, ocular surface disease, and, in some patients, sunburn and neurological degeneration. Genetically, it is assigned to eight complementation groups (XP-A to -G and variant). For the last 5 y, the UK national multidisciplinary XP service has provided follow-up for 89 XP patients, representing most of the XP patients in the United Kingdom. Causative mutations, DNA repair levels, and more than 60 clinical variables relating to dermatology, ophthalmology, and neurology have been measured, using scoring systems to categorize disease severity. This deep phenotyping has revealed unanticipated heterogeneity of clinical features, between and within complementation groups. Skin cancer is most common in XP-C, XP-E, and XP-V patients, previously considered to be the milder groups based on cellular analyses. These patients have normal sunburn reactions and are therefore diagnosed later and are less likely to adhere to UVR protection. XP-C patients are specifically hypersensitive to ocular damage, and XP-F and XP-G patients appear to be much less susceptible to skin cancer than other XP groups. Within XP groups, different mutations confer susceptibility or resistance to neurological damage. Our findings on this large cohort of XP patients under long-term follow-up reveal that XP is more heterogeneous than has previously been appreciated. Our data now enable provision of personalized prognostic information and management advice for each XP patient, as well as providing new insights into the functions of the XP proteins.

The incidence of skin cancer is increasing dramatically worldwide, and with heightened public awareness, patients present with skin cancers at an earlier stage. Although most non-melanoma skin cancers occur on exposed sites, potentially lethal malignant melanoma tends to occur under clothing. Complete skin examination allows the detection of the lesion the patient presents with and any other the physician may find. The skin examination also allows for a ‘teachable moment' to educate the patient in self-examination.

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.

Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering disease affecting the skin and mucosa and is associated with increased morbidity and mortality. Once the diagnosis is established, the main stay of treatment of PV is with systemic corticosteroids to control the disease and then to consolidate the management with other immunosuppressive agents. A small group of patients with severe pemphigus, however, remain relcalcitrant to both steroids and azathioprine, and disease is difficult to control. When considering the treatment of refractory PV, there is increasing evidence for the successful use of the monoclonal anti-CD20 antibody, rituximab. We report here six cases of patients presenting with oral and skin PV with recalcitrant or rapidly progressive disease treated with a novel dosing regimen of rituximab as a single agent. All patients achieved a complete response to a maximum follow-up of 34 months.

Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering disease affecting the skin and mucosa and is associated with increased morbidity and mortality. Once the diagnosis is established, the main stay of treatment of PV is with systemic corticosteroids to control the disease and then to consolidate the management with other immunosuppressive agents. A small group of patients with severe pemphigus, however, remain relcalcitrant to both steroids and azathioprine, and disease is difficult to control. When considering the treatment of refractory PV, there is increasing evidence for the successful use of the monoclonal anti-CD20 antibody, rituximab. We report here six cases of patients presenting with oral and skin PV with recalcitrant or rapidly progressive disease treated with a novel dosing regimen of rituximab as a single agent. All patients achieved a complete response to a maximum follow-up of 34 months.

A 33 year old woman presented with an intensely itchy, erythematous, blistering eruption bilaterally on the distal upper and lower limbs a week after coming back from a holiday in India. The rash was associated with oedema. She had attended a friend’s wedding while in India, and as part of the festivities she took part in the ritual of “mehndi,” which involved having an intricate henna design painted on her hands and feet. Five days after this ritual she developed a sharply demarcated erythematous, itchy, papulovesicular swelling in the proximal distribution of the tattoo design, which spread distally (figure⇓). Other than the skin irritation, she had no other symptoms and was systemically well. ### 1 What is the diagnosis? #### Short answer The diagnosis is contact dermatitis to paraphenylenediamine (PPD). This chemical is widely used in hair dye and is an ingredient in many other products, including black henna, which is used in temporary tattooing. #### Long answer PPD is an aromatic amine compound that is primarily used as a hair colouring agent. It is also found in a variety of products including textile dyes, photography development agents, certain local anaesthetics, cosmetics, and printing inks. It is becoming increasingly used in temporary tattoos as an addition to henna.1 Henna is a greenish brown vegetable colouring from the leaves of the plant Lawsonia inermis or L alba , which grows in India, North Africa, and Sri Lanka.2 The active ingredient is lawsone (2-hydroxy-1,4-naphthoquinone). On its own, …