Clinical and Experimental Dermatology最新文献

Folliculitis decalvans (FD) is a primary cicatricial alopecia characterised by recurrent pustulation, tufting and progressive scarring hair loss. Familial clustering is rarely reported, limiting understanding of potential genetic susceptibility. We describe five cases of FD occurring in two unrelated families: three sisters with adolescent-onset disease and a mother-daughter pair with adult-onset disease, with a possible history in a preceding generation. Clinical and histopathological findings were consistent with FD in all cases. Staphylococcus aureus was identified in three of the affected individuals. Compared with previously reported familial cases, which predominantly involve male relatives or twins, this series represents the largest female-predominant familial cluster and the first clear mother-daughter occurrence. These findings support a potential heritable component in FD and highlight the need for further genomic and mechanistic studies.

Introduction: Cutaneous T-cell lymphomas (CTCL) comprise a clinically, histologically, and molecularly heterogeneous group of subtypes. They fulfil aspects of both chronic and malignant skin conditions and may thus affect health-related quality of life (HRQL) in various ways. To date, there is little information known about the topology of CTCL patients in routine care.

Objectives: This study aims to characterize the frequency and distribution of body sites affected by CTCL in routine care and to analyze the impact on HRQL.

Methods: Cross-sectional survey study as part of the routine care at a University Medical Center. Topical distribution was identified with a detailed grid scheme filled by the patient and the affected body surface area (BSA) was recorded by the physician. Skindex-29 measured HRQL.

Results: 153 patients with CTCL were included, with a mean age of 59.5 ± 16.5 years and a majority of male patients (61.4%). The mean number of grids marked was 129.2, corresponding to 12.7% of the BSA, and the most frequently affected body areas were thighs, back, and lower legs. Skindex-29 revealed that HRQL in CTCL patients is impaired on a mild level, with a mean of 27.5 ± 21.3. Patients with involvement of the visible or genital area showed a significantly poorer HRQL. Overall, 70% of patients reported relevant pruritus (NRS ≥3), which was strongly associated with worse HRQL, independent of localization. Linear regression analysis revealed a significant correlation between an increased impairment of HRQL and a higher BSA, a manifestation in visible areas, and a higher itching intensity.

Conclusion: This analysis provides data on the distribution of CTCL in routine care. Our study highlights the importance of specific areas, such as visible body areas or the genital area, as determinants for the reduction of HRQL. This knowledge can help to improve patient-centered healthcare in CTCL.

Extramammary Paget disease (EMPD) is defined by the World Health Organisation (WHO), as 'a rare adenocarcinoma characterized by a predominant intraepithelial growth of neoplastic cells originating in the skin (primary EMPD) or representing intraepithelial spread of an underlying visceral carcinoma (secondary EMPD)'(1).It affects areas rich in apocrine glands, such as genital and perianal skin. First described by Radcliffe Crocker in 1889, EMPD often remains confined to the epidermis for years, following a quiescent course. Diagnosis is frequently delayed or missed due to its insidious onset and non-specific symptoms, which can mimic other inflammatory dermatoses. Importantly, EMPD is linked to underlying visceral malignancies although the risk varies anatomically. Data from England on EMPD incidence and 5-year survival from 2013 to 2020 shows crude incidence rate of 0.17 per 100,000 person-years (PY). Additionally, the 5-year net survival for EMPD diagnosed in 2013 was 90.9%. The reported net survival for EMPD is comparable to melanoma (89.6%), prostate (85.8%) and breast cancers (85.9%) overall i.e. for all stages combined. Management of EMPD focuses on identifying and addressing any associated underlying malignancies. Other treatment options include surgery or topical treatments, while non-surgical approaches such as radiotherapy and systemic treatment are used for advanced or metastatic disease. Treatment approaches may not be curative and recurrence rates can be up to 50%, highlighting the importance of close surveillance. This review covers EMPD's history, classification, epidemiology, aetiology, pathogenesis, diagnosis and highlights current strategies for malignancy screening and patient management. These findings are important as they contribute to our understanding of the trends and outcomes of this rare disease.