Summary Sarcoidosis is a multisystem granulomatous disease that can affect almost any organ including the skin, liver, ocular, cardiac, renal, nervous, musculoskeletal and endocrine systems. Systemic evaluation is indicated in all patients diagnosed with cutaneous sarcoidosis, as it is associated with asymptomatic systemic disease in 30%–40% of patients. Guidelines recommend that patients diagnosed with sarcoidosis undergo baseline and surveillance investigations including full blood count (FBC), renal and liver profile, Vitamin D, serum calcium, electrocardiography (ECG), chest radiography, pulmonary function tests (PFTs) and ophthalmology examination to assess for systemic involvement. Recommendations for surveillance monitoring vary on interval duration but include regular FBC, biochemistry, chest radiography and PFTs, with additional investigations and prompt referral to respective specialties as indicated.. We conducted a retrospective analysis to evaluate extracutaneous involvement and systemic evaluation of patients diagnosed with cutaneous sarcoidosis during the period 2004–2020, and compared our findings with international guidelines. Cutaneous manifestation was the primary presentation for 67% of the patients (12 of 18), an extracutaneous disease subsequently developed in 67% (8 of 12) of these patients. Baseline investigations included chest radiography (94%; 17 of 18), PFTs (39%; 7 of 18), FBC (94% (17 of 18), renal profile (89%; 16 of 18), liver function tests (83%; 15 of 18) and serum calcium (89%; 16 of 18); ECG was performed for 4 (25%) of 16 patients. No Vitamin D levels were recorded. Specialist referral was required for 89% (16 of 18); of these 16 patients, 94% (15 of 16) required referral to the Respiratory Medicine department, 69% (11 of 16) to Ophthalmology and 19% (3 of 16) to Nephrology. The results highlight the importance of a structured protocol for the systemic evaluation of patients diagnosed with cutaneous sarcoidosis. We subsequently developed a baseline and surveillance protocol for the assessment of extracutaneous disease in patients at University Hospital Limerick.

The incidence of cutaneous squamous cell carcinoma (cSCC) is increasing in the White population. Despite a favourable prognosis in the majority, it remains a cause of morbidity and mortality, especially in immunosuppressed and elderly individuals. Large cSCCs can be challenging for surgical clearance, recurrence and metastatic disease, despite guidelines for the management and staging of cSCC. We sought to identify high-risk features of those who develop metastatic disease. Our centre typically manages 300 cSCCs annually, but only cSCCs discussed at our two multidisciplinary team (MDT) meetings (head and neck and skin cancer) at our level 4 hospital over a 3-year period (2019–2021) were included in a retrospective observational study. A total of 124 patients with high-risk cSCC (due to patient or tumour factors) were included. Male sex predominated. Sixteen cases had metastatic disease. Median age at diagnosis was 80 years in the total MDT group and 86.5 years in the metastasis group. Of 16 patients with metastases, eight had complete primary excisions. Five cases had incomplete primary excision (peripheral or deep margins involved), and three cases had a narrow margin of < 1 mm. Primary tumour size was similar in both the MDT and metastasis groups (median size 17 vs. 20 mm). Head and neck sites predominated in both (88%). In the metastatic group, one had chronic lymphocytic leukaemia, but no other patients with immunosuppression were identified, and the majority (81%) suffered with multimorbidity (≥ 2 chronic illnesses). Nine patients with metastatic cSCC received radiotherapy, two systemic chemotherapies, five neck dissections and one immunotherapy. Most patients presenting with metastatic cSCC at our centre were > 85 years old, a group sometimes known as ‘the oldest old’, and many had multimorbidity. This, coupled with tumour location, poses challenges for healthcare professionals in managing the initial cSCC aggressively and implementing a reasonable follow-up and surveillance schedule. In comparing both groups, advanced age was the predominant difference, with twice as many aged > 85 years in the metastasis group vs. the MDT group. Immunosenescence is a process of immune dysfunction that occurs with age and includes remodelling of lymphoid organs, leading to changes in the immune function of the elderly and relates to the development of malignant tumours. It is estimated that 5 million people in the UK will be in this age group by 2050 (www.ageuk.org.uk/globalassets/age-uk/documents/reports-and-publications/reports-and-briefings/health–wellbeing/rb_feb13_understanding_the_oldest_old_improving_later_life.pdf). There is a need to continue to consider this dynamic group (those aged > 85 years) and those with multimorbidity when strategies for the projected management of cSCC are being considered.

Fibromatoses are a heterogeneous collection of tumours of the skin and soft tissue with a large variability in presentation. We present a case of diffuse infantile fibromatosis in a 17-day-old infant boy. He was born at 40 + 7 weeks gestation with a vacuum-assisted delivery following an uncomplicated pregnancy. On physical examination, there were two well-circumscribed circular plaques that coalesced centrally on his left elbow. The plaque measured 2 × 1 cm and was firm, nontethered and nontender. Full skin examination was otherwise normal. Histopathological examination showed hyperkeratotic skin with an attenuated epidermis overlying a dermally based bland-appearing spindle cell proliferation. The lesion was infiltrative throughout the dermis and was arranged in short fascicles and interdigitated with subcutaneous fat, which was extensively trapped by spindle cells. There was no significant atypia and mitotic activity was identifiable but not brisk. Immunohistochemical stains showed strong and diffuse reactivity for smooth muscle actin. Infantile fibromatosis is the childhood counterpart of musculoaponeurotic fibromatosis. It is a benign mesenchymal tumour of infancy and early childhood with diffuse proliferations of oval-to-spindled fibroblasts that infiltrate regional structures. There is a male predominance and in most cases is noted within the first 8 years of life. Clinically, it presents as an asymptomatic, white-yellowish mass that is nontethered to the overlying skin. Although any site may be involved, the majority of cases affect the upper limbs, head and neck region. Histological findings include a wide morphological spectrum. In infants, small oval cells appearing between primitive mesenchymal cells and fibroblasts are in a myxoid background. The cells are loosely arranged and infiltrate skeletal muscle and other regional structures. Peripheral lymphocytic inflammation blends with more cellular proliferation composed of spindle-shaped fibroblasts arranged in bundles and fascicles. There is no well-documented immunohistochemical profile. An important differential diagnosis to exclude is congenital/infantile fibrosarcoma, which, characteristically, has high cellularity, uniform herringbone pattern, high mitotic rate, and zones of haemorrhage and necrosis. In terms of management, as there is a high likelihood of local recurrence in 65% of cases, evidence supports complete excision with documented margins. Where the lesion is diffusely infiltrative, a partial resection to maintain the best postoperative function is recommended.