Introduction: pemphigus vulgaris is a rare autoimmune blistering disease that involves the skin and mucous membranes and rarely occurs in pediatric age. Methods: we present a case of childhood pemphigus in a 9-year-old patient from Burkina Faso, which initially manifested with erosive lesions symmetrically distributed in the oral cavity. After a few months, we also observed hyperchromic lesions of the back. Histopathological examination of skin samples showed intraepidermal acantholysis, while direct immunofluorescence showed deposits of complement (C3) and immunoglobulins G (IgG) in the epidermidis; an ELISA test highlighted the presence of circulating autoantibodies against desmoglein 3. Results: the follow-up of this patient was made difficult by the advent of the COVID-19 outbreak. However, after about one year of combined therapy with systemic steroids and azathioprine the patient reached clinical remission.
1 March 2018 in Italian Journal of Dermatology and Venereology
Background/Objectives: Melanoma is an aggressive cutaneous malignancy with a rising incidence. While most cases are sporadic, 5–10% are hereditary, especially in patients with multiple or familial melanomas. The aim of this study is to explore the epidemiological, clinical, histological, and genetic features of this class of patients to identify risk factors for better management and surveillance. Methods: Between 2021 and 2024, patients with multiple melanomas or a familial history of melanoma were recruited. Collected data included demographic, clinic-pathologic features, and genetic analyses. Results: Patients >60 years had a higher prevalence of multiple melanomas (>50%, p = 0.0002), while familial melanoma was more common in those <40 years (54.3%). UV exposure increased with age, while sunscreen use decreased (p = 0.0004). Younger patients showed the highest nevi counts (mean: 139.6) and density (p < 0.0001). Dermatologists more frequently detected subsequent melanomas in older patients (>60 years) (p = 0.001). Genetic testing and melanoma subtypes showed no significant age-related differences. Conclusions: melanoma can develop at any age, and early detection through regular screening is crucial. Older patients (>60 years) have a higher prevalence of multiple melanomas, influenced by UV exposure and genetics. Indeed, in our cohort, a history of sun exposure, sunburns, and tanning bed use emerged as key risk factors, particularly among older individuals. Genetic testing showed a 4.3% rate of pathogenic/likely pathogenic variants, mainly in CDKN2A. Family history and nevus burden are significant risk factors, highlighting the need for targeted surveillance in high-risk populations.
Background: Melanoma is currently the most prevalent malignant neoplasm among adults and represents the second most common cancer in both sexes among individuals aged 0 to 39 years. This retrospective multicenter study delves into the distinctive clinical, anamnestic, histopathologic, and prognostic attributes of melanoma in Adolescent and Young Adults (AYA), defined as patients diagnosed at ≤40 years, across four Italian centers. Methods: Through a computer-based review of clinical records from 1 January 2010 to 30 September 2023, AYA melanomas were contrasted with non-AYA melanomas (>40 years) among 1452 patients. Data on demographics, melanoma localization, histological type, Breslow thickness, ulceration, and sentinel lymph node (SLN) biopsy status were meticulously collected and analyzed. Results: Our analysis revealed a female predominance in the AYA group and a male predominance in the non-AYA group, with significant differences in anatomical localization and histological types between the two. AYA melanomas showed nearly equal trunk and limb involvement, contrasting with the trunk predominance in non-AYA melanomas. While Breslow thickness was similar across both groups, the presence of ulceration and total number of nevi showed no significant difference. Survival analysis indicated a marginally higher Disease-Free Survival (DFS) in AYA patients compared to non-AYA patients, without a significant difference in Overall Survival (OS). Conclusions: This study highlights demographic and clinical distinctions between AYA and non-AYA melanoma patients, underscoring the need for tailored follow-up and treatment strategies. Despite these insights, the heterogeneity of melanoma among young adults calls for further research, including genetic analyses, to fully understand this unique melanoma subgroup. Indeed, AYA melanoma patients could represent a different and specific target for both follow-up and treatments.
We describe the second case of infection by Emergomyces pasteurianus that occurred in Italy. The patient presented ulcerated nodular lesions primarily in the forehead, beneath the orbital and nasal areas, but also in the neck and fingers in the early stages. Treatment involved amphotericin B, followed by long-term itraconazole, which resulted in complete clinical resolution. A review of the literature is also included.
Linear immunoglobulin (Ig) A disease (LAD) is a rare autoimmune blistering dermatosis caused by linear deposition of IgA1 along the dermoepidermal junction of skin and mucosae. It can affect both children and adults. Although idiopathic in most cases, it can also be associated with infections, malignancies, or drug administration. Among medications, antibiotics are most commonly responsible, particularly vancomycin. We report the case of a 51-year-old female patient who developed mucocutaneous LAD after oral metronidazole administration, which was confirmed by histopathological examination and direct immunofluorescence of one of the bullous lesions. Lesions rapidly cleared after metronidazole discontinuation and a short course of oral methylprednisolone and dapsone. To our knowledge, this is the third reported case of metronidazole-induced LAD, and the first in which metronidazole was not administered in combination with other antibiotics such as ceftriaxone.