bluuebunny/crossref_metadata_2025_split huggingface dataset:
DP05 (P061) Squamoid eccrine ductal carcinomas: challenges in diagnosis and management
Authors: Cunliffe Amy , Elsheikh Somaia , Odedra Sunita , Varma Sandeep 
Published: June 2024

Abstract
Squamoid eccrine ductal carcinomas (SEDCs) are rare adnexal carcinomas displaying both squamous and duct differentiation. We wish to remind clinicians of these recently described cancers, which can have aggressive behaviour (van der Horst MPJ, Garcia-Herrera A, Markiewicz D et al. Squamoid eccrine ductal carcinoma. Am J Surg Pathol 2016; 40: 755–60). We identified five cases in our department: four female patients and one male. All lesions developed on the face, in patients aged 69–83 years. The majority developed over one to several years, apart from one rapidly growing lesion, and measured between 5 and 40 mm. Clinical examination suggested squamous cell carcinoma (SCC) in two cases and basal cell carcinoma in three cases due to features such as puckering, rolled borders, telangiectasia, shine, ulceration and crust. Punch biopsies were performed in all five lesions; however, histological diagnosis of SEDC was suggested in only two. One of these patients underwent Mohs micrographic surgery (MMS), which successfully cleared the lesion after two stages. The second patient, diagnosed on biopsy, had a large 30-mm lesion with invasion into the lacrimal sac and left nasal bone. The patient opted for palliative radiotherapy rather than extensive surgery and subsequently developed a left-cheek metastasis and symptoms suggestive of metastatic disease. Three of our cases were diagnosed after wide local excision (WLE) based on the histological and immunological features. The punch biopsies had suggested either SCC or adenosquamous/adnexal infiltrative carcinoma. One of these patients required adjuvant radiotherapy due to perineural involvement (&lt; 0.1 mm). The other two cases were incompletely excised on WLE and the patients were offered further surgery or radiotherapy. Histologically these lesions were poorly defined infiltrative neoplasms consisting of atypical squamoid cells extending into the deep dermis or subcutaneous tissue with ductal differentiation, pronounced cytological atypia and associated desmoplastic stroma. Tumour thickness measured 3–10.3 mm. Immunohistochemistry was positive for CK7, AE1/AE3, CK5/6, p63, CAM5.2, carcinoembryonic antigen and epithelial membrane antigen. These five cases highlight the challenges faced in the diagnosis of SEDC for both the dermatologist and the dermatopathologist. MMS is recommended (Lim MM, Macdonald JA. Squamoid eccrine ductal carcinoma: treatment and outcomes. Am J Dermatopathol 2022; 44: 249–53) and lesions misdiagnosed as SCC may receive less effective treatment; our three patients who underwent WLE have required further interventions. We also report one patient with locally aggressive and likely metastatic disease. Patients with SEDC benefit from early diagnosis, MMS and close follow-up to reduce the risk of recurrence and improve patient outcomes.
DP06 (P001) A descriptive, observational and cross-sectional study of clinicoepidemiological, dermoscopic and histopathological aspects of primary localized cutaneous amyloidosis and its effect on the quality of life of the patient
Authors: Rane Meghana , Mahajan Sunanda 
Published: June 2024

Abstract
The aims of this study were to examine the clinicoepidemiological factors related to primary localized cutaneous amyloidosis; to investigate its dermoscopic and histopathological findings; to determine the clinicohistopathological and dermoscopic correlation; and to study its effect on the quality of life of the patients (Al-Ratrout JT, Satti MB. Primary localized cutaneous amyloidosis: a clinicopathological study from Saudi Arabia. Int J Dermatol 1997; 36: 428–34; Rasi A, Khatami A, Javaheri SM. Macular amyloidosis: an assessment of prevalence, sex and age. Int J Dermatol 2004; 43: 898–9). An open, prospective, descriptive and observational study was conducted in patients with primary cutaneous localized amyloidosis suspected clinically. It was carried out for a period of 18 months, with 100 patients in the study. Only patients who gave written informed consent for the study were included. Pregnant and lactating patients were also excluded. Clinical examination with clinical photographs, skin biopsy and dermoscopy of the same lesions was done using a disposable handheld biopsy punch under local anaesthesia. The final diagnosis was then made after compiling all of the clinical, dermoscopic and histopathological features. Microsoft Excel and SPSS version 19 (IBM, Armonk, NY, USA) were used for statistical analysis. Out of the 100 patients, 56 had macular amyloidosis, 33 had lichenoid amyloidosis, eight had biphasic amyloidosis, two had dyschromic amyloidosis and one had primary amyloidosis of the concha. The most common age group was 40–60 years, and the condition was more common in women. A history of use of scrubbing and rubbing while bathing was reported in 45 patients: 33 (73%) of these had macular amyloidosis, nine (20%) had lichenoid amyloidosis, two (4%) had mixed amyloidosis and one (2%) had dyschromic amyloidosis. Pruritus was seen in 92 patients (92%). Dermoscopy of amyloidosis showed a hub-and-spoke pattern and amorphous pigmented globules; 11 patients showed nonspecific pigmented globules and seven showed scales and a central scar pattern. On histopathology of macular amyloidosis, 94 patients showed amyloid deposits stained positively with Congo red. The mean Dermatology Life Quality Index was 3 in macular amyloidosis, and 4 in lichenoid and biphasic amyloidosis. Studies using confocal laser scanning microscopy and direct immunofluorescence could further provide insight and can allow better outcomes. Primary cutaneous amyloidosis has a small-to-moderate effect on the quality of life of patients as it is pruritic and disfiguring. Dermoscopy is a valuable tool to diagnose characteristic features and thus helps to make quick and accurate diagnosis and avoid skin biopsies, as shown in this study.
DP10 Scalp disease in dermatomyositis
Authors: Connolly Aveen , Philippidou Marianna , Gordon Patrick , Wincup Chris , Worsnop Fiona , Creamer Daniel 
Published: June 2024

Abstract
The clinical presentation of dermatomyositis (DM) is heterogeneous, with patients expressing varying degrees of muscle and skin inflammation. Scalp inflammation is often disregarded in the clinical assessment and is seldom the subject of clinical studies. However, DM at this site contributes significantly to the symptom burden, a feature heightened by the scalp’s comparative lack of response to standard immunosuppressant therapy. As well as a sparsity of studies detailing clinical features in scalp DM, the literature on scalp histopathology is also limited. In an attempt to define the range of clinicopathological features, and to consider potential associations with myositis-specific antibodies, we undertook a prospective observational study of scalp disease in a cohort of patients with DM. Over a 10-year period, 96 consecutive adult patients with clinically and immunologically proven DM were prospectively recruited. The extent of cutaneous DM was quantified using the Cutaneous Dermatomyositis Area and Severity Index (CDASI). For the purposes of our study, the scalp-CDASI was recorded separately. In patients with scalp DM, sites of involvement were recorded: frontal, parietal, occipital and vertex. The impact of cutaneous involvement was assessed using the Dermatology Life Quality Index and a scalp itch numerical rating score (NRS). In total, 47 of 96 patients with DM had scalp involvement, and all patients had cutaneous DM at other body sites. In the scalp DM cohort, scalp-CDASI ranged from 1/9 to 8/9. The distribution of DM involvement by scalp zone demonstrated 19 of 47 global, 17 of 47 occipitoparietal, and 11 of 47 frontovertex. The mean scalp itch NRS for the scalp DM group was 4.8, 33 of 47 patients reported scalp itching and/or soreness disturbing sleep, and 38 of 47 patients reported hair loss. In the scalp DM group, myositis-specific antibodies were demonstrated in 41 of 47 patients. Overall, 17 of 41 patients were positive for TIF-1g, 11 of 41 SAE, 6 of 41 MDA5, 3 of 41 Mi-2, 3 of 41 NXP-2, and 1 of 41 Jo-1. Assessment of scalp biopsies from nine patients demonstrated a number of dermatopathological features that appear exclusive to scalp DM. Unlike other scarring alopecias, there was no typical perifollicular fibroplasia, but in these cases, there was thickening of the basement membrane around the follicles. A further consistent observation involved the dermal collagen, which showed hyalinization, homogenization and areas of necrobiosis, as well as areas of very severe elastosis. Our findings suggest that the scalp possesses a site-specific susceptibility to DM inflammation, a hypothesis that mirrors the disease tropism seen in psoriasis, lupus and other inflammatory dermatoses. The evidence collected in this study has unequivocally demonstrated that scalp DM produces a symptom burden associated with considerable morbidity. Our study is the first to examine the subjective effects of scalp involvement in DM and to quantify its impact.
DP13 First case of distant metastases from a BAP1-inactivated melanocytoma with melanoma transformation
Authors: Guckian Jonathan , Mathew Bipin , Merchant Will , Muinonen-Martin Andrew , Mitra Angana , Smith Hayley 
Published: June 2024

Abstract
A 43-year-old man presented with a long-standing back lesion causing irritation and bleeding. A presumed pedunculated seborrhoeic keratosis was shave excised by his general practitioner; however, histology revealed a BRCA1-associated protein (BAP1)-inactivated melanocytoma (BIM) with melanoma transformation. Histologically, BIMs are identified by the presence of a dermally located expansile clone of epithelioid melanocytes, sometimes associated with a pre-existent conventional naevus. Clones have abundant amphophilic cytoplasm and nuclear polymorphism with distinct cell membranes. BAP1 immunohistochemistry showing loss of nuclear expression in altered melanocytes confirms the diagnosis. BIMs typically harbour a BRAF V600E mutation as the initial oncogenic driver, with subsequent biallelic loss of the BAP1 gene leading to BAP1 protein inactivation. They typically run a benign, indolent course, with no cases with distant metastasis previously reported. Our case had BAP1-inactivated clones with typical BIM morphology. However, there was also a dominant, aggressive, BAP1-inactivated subclone with 15 mitoses, Ki67 &gt; 10%, ulceration, P16 loss and PRAME positivity. These features are unusual for BIMs and are considered high risk in conventional melanoma subtypes. Overlying this was a junctional component with focal architectural and cytological atypia, bordering on melanoma in situ. Next-generation sequencing showed an NRAS driver mutation. DNA methylation profiling showed more than three copy number variations: 8q12.1q24.3, 90.2-Mb gain; whole chromosome 9 loss; 9p21.3, 3-Mb biallelic loss including CDKN2A and CDKN2B (whole genes); and 11q22.3q25, 31.7-Mb loss. There was no significant family history, and germline BAP1 genetic testing was negative. The patient underwent narrow margin excision followed by wide local excision. Sentinel lymph node biopsy was not offered due to previous pulmonary emboli and obesity. After 11 months he reported persistent abdominal pain. Computed tomography showed widespread metastatic disease (right axillary lymph nodes, brain and liver). Lymph node biopsy revealed atypical naevoid to epithelioid cells with focal cytoplasmic pigmentation and mitotic activity lying within fibrous stroma. Immunohistochemistry demonstrated strong diffuse expression of SOX-10 and melan-A, with loss of BAP1 expression. We report the first case of BIM with melanoma transformation (malignant BAP1 inactivated tumour) resulting in stage IV melanoma. Our centre has identified four further lesions in three patients with similar histological presentation. Two patients with three lesions developed locoregional recurrence. Our cases demonstrate that not all BIMs run a benign to indolent course. Identifying high-risk features as seen in these cases will aid future risk stratification.
DP17 Alveolar soft part sarcoma: a not-so-common entity
Authors: Li Chia-Lun , Wu Chen-Yi 
Published: June 2024

Abstract
A 24-year-old man presented with an asymptomatic mass on his left thigh that had been present for 1 month. On examination, a well-defined subcutaneous nodule measuring approximately 2.1 × 2.1 cm was observed on his left thigh, with no apparent epidermal change. Magnetic resonance image showed a hyperintense lesion over the left thigh with contrast enhancement. An incisional biopsy revealed nests of eosinophilic cells displaying nuclear pleomorphism, prominent nucleoli, and positive transcription factor enhancer (TFE)3 expression. A diagnosis of alveolar soft part sarcoma (ASPS) was made. ASPS, a rare sarcoma primarily occurring in young patients, typically affects deep soft tissues of the buttock and thigh. While it shows a relatively indolent clinical progression, ASPS often indicates a poor prognosis due to late-stage metastases upon diagnosis. Microscopically, ASPS is characterized by uniform nests of polygonal tumour cells within fibrovascular septa and vascular channels, displaying distinctive cellular dyscohesion, resulting in the characteristic ‘pseudoalveolar pattern’. The cells have defined borders, abundant eosinophilic to granular cytoplasm, and an epithelioid appearance. ASPS is caused by a specific unbalanced translocation, der(17)t(X:17)(p11;p25), forming an ASPSCR1–TFE3 fusion gene. In this patient, further imaging was arranged for tumour staging, which confirmed localized disease without lymph node or distant metastasis. The patient underwent wide excision and local flap surgery and has remained recurrence free during a 4-year follow-up. This case emphasizes the importance of recognizing the clinical features of ASPS in young patients and considering skin biopsy and image studies for long-standing subcutaneous nodules, as this can provide an early diagnosis and improve patient outcomes.
Geriatric DermatologyOral presentationsBG01 The impact of menopause on eczema
Authors: Kamp Erin , Nielsen-Scott Anna , DeGiovanni Claudia 
Published: June 2024

Abstract
The falling oestrogen levels seen in menopause have implications on the structure and function of the skin (Duarte GV, Trigo AC, Paim de Oliveira Mde F. Skin disorders during menopause. Cutis 2016; 97: E16–23). Improvement in skin barrier function and reduction in transepidermal water loss have been seen with transepidermal oestrogen. However, further research is required to understand the role of oestrogen within the epidermis. A range of dermatological conditions have been reported in up to 64% of women attending menopause clinics (Duarte et al.). These include pruritus, xerosis and eczema (Pariath K, Nair PA. A cross-sectional study on the dermatoses in postmenopausal patients at a rural-based tertiary health care center. Indian J Dermatol 2019; 64: 360–5). Our aim was to further understand the impact of the menopause and hormone replacement therapy (HRT) on eczema severity. We sent a survey by text and/or email to patients with eczema aged ≥ 40 years. We received 12 responses from patients with an age range from 40 to 85 years. Overall, 92% patients self-reported as White (11 of 12) and 8% (1 of 12) as Asian or Asian British. The mean age of menopause was reported as 47.3 years (range 35–58). The majority of patients were diagnosed with eczema as a child (9 of 12). Interestingly, three patients were diagnosed with eczema between the ages of 49 and 53 years. Previous and current treatments included emollients, topical corticosteroids, conventional systemics and biologics. One-third of patients reported a deterioration in eczema with or before menstruation. Two-thirds (67%, 8 of 12) of patients’ eczema flared with menopause, and one patient developed eczema for the first time after menopause. None of the 12 patients noticed an improvement in eczema associated with menopause. The majority of patients did not notice a change in their eczema with HRT. Dryness, irritation, contact allergy, sensitivity, skin thinning and nonspecific rash were all noticed by patients with menopause and/or after menopause. Two-thirds of patients who completed our survey reported a flare of eczema with menopause, and we believe this is an exacerbating factor likely to be an under-recognized in the disease. It is vital that further research is carried out to further understand the role of oestrogen within the epidermis and skin barrier and the possible therapeutic benefit of HRT.
BG02 A rare case of necrolytic migratory erythema
Authors: Katira Arnav , Gaikwad Sophie 
Published: June 2024

Abstract
We present the case of a 72-year-old White man who had a several months’ history of multiple crusted annular plaques starting in the groin and spreading to the lower limbs and upper back. He also had an erythematous scaly rash on the face in a seborrhoeic distribution and a sore tongue. Systemic enquiry highlighted significant weight loss. Additionally, he had a recent diagnosis of borderline diabetes mellitus and later developed steatorrhoea. He also had a palpable mass behind his umbilicus. Laboratory studies showed a mild normocytic anaemia. Plasma glucagon levels were markedly raised at 230 pmol L−1. Levels of zinc, other trace metals and haematinics were normal. Computed tomography imaging identified a lesion to the body and tail of the pancreas, which was locally invasive and partly calcified. Histology from the rash showed confluent parakeratosis containing few neutrophils. The epidermis was mildly acanthotic with mild spongiosis and dyskeratotic cells and some vacuolated keratinocytes. There was mild lymphocytic exocytosis, but no significant lichenoid reaction. There were moderate perivascular lymphoplasmacytic infiltrates with mild oedema in the epidermis. No immune deposits were seen on direct immunofluorescence. He was referred to the general surgeons and the neuroendocrine multidisciplinary team (MDT). A well-differentiated neuroendocrine tumour, in keeping with a glucagonoma, was seen on histology of the tail of the pancreas. He was treated with zinc supplements and topical steroids, as well as having staging scans and further management of his glucagonoma. Necrolytic migratory erythema is a rare clinical entity usually occurring with a glucagonoma, an alpha-cell tumour of the pancreatic islets. The elevated glucagon levels may lead to diabetes mellitus, weight loss, diarrhoea, neuropsychiatric problems, venous thromboembolism and anaemia. The rash normally improves with management of the underlying tumour. This may include surgical management or suppression of raised glucagon levels by a glucagon antagonist. Patients should be referred to a specialist neuroendocrine MDT and have staging scans. Zinc supplementation may also improve the rash. Early diagnosis of the rash may help to prevent morbidity and mortality from the underlying neoplasia and reduce the chance of metastasis.
Poster presentationsBG05 Menopause and the skin: prevalence of skin symptoms in patients attending a menopause clinic
Authors: Salih Hamisha , Hum Olivia , Schaedel Zoe , DeGiovanni Claudia 
Published: June 2024

Abstract
The peri- and postmenopausal period of a person’s life is a natural part of physiological ageing driven by declining ovarian function. A hypo-oestrogenic state follows the onset of the menopause and persists for the remainder of their lives. The skin contains oestrogen receptors, and impairment of the skin barrier is noted in oestrogen-deficient skin (Rzepecki A, Murase J, Juran R et al. Estrogen-deficient skin: the role of topical therapy. Int J Womens Dermatol 2019; 5: 85–90). It is therefore expected that this may affect the normal function of the skin and common skin conditions. We suspect that these symptoms experienced may be under-reported and, when they are reported, may not be considered associated to the menopause. We have conducted an observational study exploring the symptoms that patients experience during the perimenopausal and postmenopausal time. Patients seen in a community-based specialist menopause clinic were invited to complete a questionnaire enquiring about skin, oral, genital and hair symptoms. In total, 32 responses were collected. Overall, 46% of respondents still had menstrual periods and 77% were on hormonal treatments, of which oestrogen therapy was most frequently taken. The most reported skin symptoms included dry skin (78%) and itchiness (75%). Dry mouth was the commonest reported oral symptom, and 84% of patients had experienced at least one hair symptom since the onset of their menopause. In total, 91% reported experiencing at least one vulval symptom, with the most common being dryness (66%), itchiness (55%) and soreness (41%). One-half (50%) noticed worsening in a previously diagnosed dermatological disorder. In total, 63% patients reported skin symptoms that they had attempted to manage themselves without consulting their doctor. The Dermatology Life Quality Index was also collected for each patient, which showed an average score of 6 out of 30 and ranged up to 17 out of 30, indicating that skin symptoms cause a moderate impact on the quality of life of peri- and postmenopausal people. Our results highlight the significant effect the menopause can have on quality of life and the wide range of dermatological symptoms associated with it. Patients appear reluctant to seek medical support for skin symptoms related to the menopause and consequently may not receive optimal treatment. It is paramount that dermatologists and menopause specialists are aware of the immense impact menopause has on the skin, hair, vulva and mouth in order to improve the quality of life for affected patients. Further research is required to explore management options, including hormone replacement therapy, for skin symptoms associated with the menopause.