Mohs micrographic surgery in immunosuppressed vs immunocompetent patients: Results of a prospective nationwide cohort study (REGESMOHS, Spanish registry of Mohs surgery).

BACKGROUND: Immunosuppressed (IS) patients, particularly solid organ transplant recipients and those on immunosuppressive therapy, face a higher incidence and recurrence of nonmelanoma skin cancers (NMSC), including basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). Mohs micrographic surgery (MMS) is the preferred treatment for high-risk NMSC due to its high cure rate and margin examination capabilities. However, IS patients may experience more complications, such as surgical site infections, and a greater risk of recurrence, making their outcomes a subject of interest. OBJECTIVES: This study aimed to compare IS and immunocompetent (IC) patients undergoing MMS for NMSC in terms of baseline characteristics, intra- and post-surgical complications, and postoperative recurrence rates. METHODS: The study utilized data from the REGESMOHS registry, a 7-year prospective cohort study in Spain. It included 5226 patients, categorizing them into IC (5069) and IS (157) groups. IS patients included solid organ transplant recipients, those on immunosuppressive treatments, individuals with haematological tumours and HIV-positive patients. Patient data, tumour characteristics, surgical details and outcomes were collected and analysed. RESULTS: IS patients demonstrated a higher proportion of SCC, multiple synchronous tumours and tumours invading deeper structures. Complex closures, unfinished MMS and more surgical sections were observed in the IS group. Although intra-operative morbidity was higher among IS patients, this difference became non-significant when adjusted for other variables such as year of surgery, antiplatelet/anticoagulant treatment or type of closure. Importantly, IS patients had a substantially higher recurrence rate (IRR 2.79) compared to IC patients. CONCLUSIONS: This study suggests that IS patients may be at a higher risk of development of AE such as bleeding or tumour necrosis and are at a higher risk of tumour recurrence. Close follow-up and consideration of the specific characteristics of NMSC in IS patients are crucial. Further research with extended follow-up is needed to better understand the long-term outcomes for this patient group.

Real-World Experience with Vismodegib on Advanced and Multiple BCCs: Data from the RELIVIS Study.

BACKGROUND: Vismodegib is approved for advanced cases of basal cell carcinomas not amenable to surgery or radiotherapy. Large studies on the use of vismodegib in clinical practice are scarce. OBJECTIVES: The main objective of the study was to analyse the evolution and therapeutic management of relapses and lack of response in patients who had received vismodegib for locally advanced and/or multiple basal cell carcinomas in a real-life multicentre setting. METHODS: This nationwide retrospective study collected data on patients treated with vismodegib in 15 specialized centres. We included patients who first received vismodegib until intolerable toxicity, maximum response, or progressive disease. Exploratory research variables referred to patient and tumour characteristics, vismodegib effectiveness and safety, relapse rate and management, and mortality. A multivariable logistic regression model was used to identify predictors of complete clinical response. RESULTS: 133 patients with advanced BCC were included in the registry. The objective response rate (ORR) was 77.5% and nearly half of the patients (45.9%) achieved complete remission. Long-term information and detailed information of subsequent treatments after a regime of vismodegib was available for 115 patients. Only 34% of the patients in this group were subsequently treated with other therapies or vismodegib rechallenge. Sixty-nine percent of the patients who had shown a complete remission with vismodegib remained free of recurrence while 30.7% relapsed. Almost half of the patients who received additional therapies after the first course of vismodegib achieved complete tumour remission. Three and 2 out of 9 patients who were rechallenged with vismodegib achieved complete and partial responses, respectively, with an ORR of 55.5%. CONCLUSION: Our study confirms efficacy of vismodegib in routine clinical practice. The risk of recurrence after achieving complete response with vismodegib was lower than previous reports. Rechallenge with vismodegib is feasible and most patients responded to re-treatment.

Complications Associated with Mohs Micrographic Surgery: Data from the Nationwide Prospective Cohort REGESMOHS.

BACKGROUND: Large prospective studies on the safety of Mohs micrographic (MMS) surgery are scarce, and most focus on a single type of surgical adverse event. Mid-term scar alterations and functional loss have not been described. OBJECTIVES: To describe the risk of MMS complications and the risk factors for them. METHODS: A nationwide prospective cohort collected all adverse events on consecutive patients in 22 specialised centres. We used multilevel mixed-effects logistic regression to find out factors associated with adverse events. RESULTS: 5,017 patients were included, with 14,421 patient-years of follow-up. 7.0% had some perioperative morbidity and 6.5% had mid-term and scar-related complications. The overall risk of complications was mainly associated with use of antiaggregant/anticoagulant and larger tumours, affecting deeper structures, not reaching a tumour-free border, and requiring complex repair. Age and outpatient setting were not linked to the incidence of adverse events. Risk factors for haemorrhage (0.9%) were therapy with antiaggregant/anticoagulants, tumour size, duration of surgery, and unfinished surgery. Wound necrosis (1.9%) and dehiscence (1.0%) were associated with larger defects and complex closures. Immunosuppression was only associated with an increased risk of necrosis. Surgeries reaching deeper structures, larger tumours and previous surgical treatments were associated with wound infection (0.9%). Aesthetic scar alterations (5.4%) were more common in younger patients, with larger tumours, in H-area, and in flap and complex closures. Risk factors for functional scar alterations (1.7%) were the need for general anaesthesia, larger tumours that had received previous surgery, and flaps or complex closures. CONCLUSIONS: MMS shows a low risk of complications. Most of the risk factors for complications were related to tumour size and depth, and the resulting need for complex surgery. Antiaggregant/anticoagulant intake was associated with a small increase in the risk of haemorrhage, that probably does not justify withdrawal. Age and outpatient setting were not linked to the risk of adverse events.

Risk Factors and Rate of Recurrence after Mohs Surgery in Basal Cell and Squamous Cell Carcinomas: A Nationwide Prospective Cohort (REGESMOHS, Spanish Registry of Mohs Surgery).

Randomized studies to assess the efficacy of Mohs micrographic surgery in basal cell and squamous cell carcinomas are limited by methodological and ethical issues and a lack of long follow-up periods. This study presents the "real-life" results of a nationwide 7-years cohort on basal cell carcinoma and squamous cell carcinoma treated with Mohs micrographic surgery. A prospective cohort was conducted in 22 Spanish centres (from July 2013 to February 2020) and a multivariate analysis, including characteristics of patients, tumours, surgeries and follow-up, was performed. A total of 4,402 patients followed up for 12,111 patient-years for basal cell carcinoma, and 371 patients with 915 patient-years of follow-up for squamous cell carcinoma were recruited. Risk factors for recurrence included age, non-primary tumours and more stages or unfinished surgeries for both tumours, and immunosuppression for squamous cell carcinoma. Incidence rates of recurrence were 1.3 per 100 person-years for basal cell carcinoma (95% confidence interval 1.1-1.5) and 4.5 for squamous cell carcinoma (95% confidence interval 3.3-6.1), being constant over time (0-5 years). In conclusion, follow-up strategies should be equally intense for at least the first 5 years, with special attention paid to squamous cell carcinoma (especially in immunosuppressed patients), elderly patients, non-primary tumours, and those procedures requiring more stages, or unfinished surgeries.

Cutaneous adverse events and quality of life in outpatients receiving anticancer agents: results from an observational, cross-sectional study.

BACKGROUND: Despite growing interest in cutaneous adverse events (CAEs) and their management in patients with cancer, they are often underreported and there are no extensive data on their impact on quality of life (QoL). Healthcare professionals should consider this issue in order to minimize its negative impact on QoL and improve patient outcomes. This study evaluates the impact of CAEs on QoL in outpatients receiving anticancer drugs and aims to determine the differences in QoL between conventional chemotherapy versus targeted therapies. METHODS: A total of 114 cancer patients with CAEs were included in this observational, cross-sectional study. Patient-reported outcomes instruments (Functional Assessment of Cancer Therapy - General, Dermatology Life Quality Index, and Skindex-16) were used. RESULTS: Mean scores in QoL indices were 65.3±13.4, 8.4±5, and 30.8±16.9 in Functional Assessment of Cancer Therapy - General, Dermatology Life Quality Index, and Skindex-16, respectively. The CAEs that had the greatest impact on dermatologic-related QoL were hand-foot skin reaction, rash, palmo-plantar erythrodysesthesia, and papulopustular eruption. No significant differences in QoL indices according to the type of treatment (conventional chemotherapy versus targeted therapy) were observed. CONCLUSIONS: CAEs, and particularly hand-foot toxicities, rashes, and papulopustular eruptions, can have an impact on QoL in outpatients receiving anticancer drugs as evaluated with three different patient-reported outcomes instruments. No differences in QoL related to CAEs were observed between conventional chemotherapy and targeted therapy.

State of the art of Mohs surgery for rare cutaneous tumors in the Spanish Registry of Mohs Surgery (REGESMOHS).

BACKGROUND: The use of Mohs micrographic surgery (MMS) for rare cutaneous tumors is poorly defined. We aim to describe the demographics, tumor presentation and topography, surgery characteristics and complications of MMS for rare cutaneous tumors in a national registry. METHODS: Prospective cohort study of patients treated with MMS in Spain between July 2013 and June 2018. The inclusion criteria were patients with cutaneous tumors with final diagnosis different from basal cell carcinoma, squamous cell carcinoma, dermatofibrosarcoma protuberans, or any kind of melanoma. RESULTS: Five thousand and ninety patients were recorded in the registry, from which only 73 tumors (1.4%) fulfilled the inclusion criteria: atypical fibroxanthoma (18), microcystic adnexal carcinoma (10), extramammary Paget's disease (7), Merkel cell carcinoma (5), dermatofibroma (4), trichilemmal carcinoma (4), desmoplastic trichoepithelioma (4), sebaceous carcinoma (3), leiomyosarcoma (2), porocarcinoma (2), angiosarcoma (2), trichoblastoma (1), superficial acral fibromyxoma (1), and others (10). No intra-surgery morbidity was registered. Postsurgery complications appeared in six patients (9%) and were considered mild. Median follow-up time was 0.9 years during which three Merkel cell carcinomas, one angiosarcoma, one microcystic adnexal carcinoma, and four others recurred (12.3%). CONCLUSION: This national registry shows that rare cutaneous tumors represent a negligible part of the total MMS performed in our country with a low complication rate.

Ectima gangrenoso primario como primera manifestación de leucemia aguda / Ecthyma gangrenosum as first manifestation of acute leukemia

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Epidermolisis ampollosa distrófica pruriginosa con lesiones albopapuloides / Dystrophic epidermolysis bullosa pruriginosa with albopapuloid lesions

Las epidermolisis ampollosas son un grupo de enfermedades genéticas, que presentan erosiones y ampollas con traumatismo mínimo. Cuando el plano de clivaje se sitúa por debajo de la lámina basal se denominan dermolíticas o distróficas. El prurito, las excoriaciones y las lesiones liquenoides e hipertróficas caracterizan el subtipo denominado pruriginoso. En escasas ocasiones se ha descrito asociando lesiones albopapuloides. Caso clínico. Mujer de 34 años de edad que consulta por dermatosis de larga evolución, con ampollas y prurito intenso. Se observan lesiones cicatriciales atróficas, erosiones y ampollas tensas sobre base no inflamatoria. Además existen lesiones liquenificadas hipertróficas y distrofia ungueal. En el tronco presenta lesiones papulosas de coloración blanquecina. La biopsia cutánea muestra una ampolla con despegamiento subepidérmico. Comentario. Se trata de un caso de epidermolisis ampollosa distrófica pruriginosa, que asocia lesiones albopapuloides. Estas lesiones son características de un subgrupo de epidermolisis ampollosa distrófica con herencia autosómica dominante, si bien no son patognomónicas

Epidermolysis bullosa is a collection of genetic disorders, with skin fragility and after minor trauma blistering. Dystrophic epidermolysis bullosa associates scarring, milia, and nail dystrophy, and blisters are subepidermal. Epidermolysis bullosa pruriginosa is a subtype with intense itching and lichenifi ed lesions. Albopapuloid lesions have been rarely reported associated to this type. Case report. A 34-year-old woman refers blisters and intense pruritus since she was a child. Examination reveals scars, erosions, and isolated blisters, located mainly on limbs. Violaceous hypertrophic lichenifi ed lesions occur on dorsal arms and hands. Most fi nger and toe nails are dystrophic. Whitish papules are present on trunk. Skin biopsy shows a subepidermal blister with mild inflammatory cell infi ltrate. Comment. We present a case of dystrophic epidermolysis bullosa pruriginosa that associates albopapuloid lesions. These lesions are found mainly in dominant dystrophic epidermolysis bullosa, but they are not pathognomonic

Vasculitis cutánea, neutropenia y fiebre, en una paciente con artritis reumatoide grave / Cutaneous vasculitis, neutropenia and fever, in a patient with severe rheumatoid arthritis

La vasculitis reumatoide es una complicación rara de la artritis reumatoide con una alta tasa de mortalidad debido a vasculitis sistémica y a infecciones. Presentamos el caso de una mujer de 70 años con una artritis reumatoide grave que presentó un brote intenso de vasculitis reumatoide, afectando a la piel, así como neutropenia y fiebre. En este caso la neutropenia autoinmune incrementa la susceptibilidad a infecciones bacterianas y es una factor limitante para el tratamiento con agentes citostáticos. Creemos que es una paciente interesante debido a la combinación de vasculitis, neutropenia y fiebre que represena una situación crítica en dermatología, y estas situaciones son poco frecuentes en nuestra práctica diaria (AU)