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CIC::DUX4 fusion sarcoma represents a rare and aggressive subtype of undifferentiated small round blue cell tumors. We report on a 23-year-old African male who developed a rapidly enlarging inferolateral left buttock nodule with ulceration. After debulking excision of the lesion, histologic sections demonstrated sheets and lobules of atypical round blue cells with significant cytologic atypia. Prominent foci of atypical mitotic figures and tissue necrosis were present. Tumoral cells stained strongly and diffusely using MDM2, vimentin, WT1 and CD99 immunohistochemical (IHC) markers. Molecular testing was performed and highlighted CIC::DUX4 gene fusion positivity, making the diagnosis of a CIC::DUX4 sarcoma (CDS). Post-surgical excision, the patient showed no disease on imaging and underwent five cycles of adjuvant chemotherapy with no recurrence observed at the eight-month follow-up. With fewer than 200 cases reported in the literature and somewhat nonspecific clinicopathologic characteristics, CIC::DUX4 sarcoma presents a diagnostic challenge. This case underlines the importance of molecular diagnostics in undifferentiated sarcomas and presents a rare primary cutaneous manifestation of CIC::DUX4 fusion sarcoma. Additionally, we provide a review of the literature to aid in recognition, diagnosis, and treatment of this rare entity., (© 2024 The Author(s). Journal of Cutaneous Pathology published by John Wiley & Sons Ltd.)

Abstract: Cutaneous ganglioneuromas (GNs) are exceptionally uncommon tumors, and many reported cases describe association with overlying epidermal hyperplasia that may be interpreted as seborrheic keratosis (SK) or SK-like proliferation. We report 5 cases of cutaneous GN in adult patients; all of which were discovered incidentally in the immediate vicinity of epidermal hyperplasia. A review of the literature demonstrates the current-although likely imperfect-understanding of the etiopathogenesis of both SK and GN in the skin. We explore the putative pathophysiologies of other common, well-characterized skin lesions and, taking them into account, provide rationale for the coexistence of cutaneous GN with overlying SK and SK-like epidermal changes. However, we ultimately acknowledge a dilemma of causality and, given the rarity of their co-occurrence, objectively question whether occasional cameo appearances by GN lying subjacent to SK and SK-like hyperplasia may be due merely to chance., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

The authors reviewed outpatients in a tertiary dermatology clinic in Botswana to expand knowledge on patterns of skin disease in this population with a high prevalence of human immunodeficiency virus (HIV). Approximately one-third of new and follow-up patients were HIV positive. Common dermatologic conditions included eczematous eruptions, viral and fungal infections, malignant neoplasms, vascular disorders, disorders of pigmentation, and mechanical/physical injury-related disorders. HIV has impacted patterns of dermatologic disease in Botswana, with Kaposi sarcoma being the most frequently biopsied condition. Given the shortage of dermatology specialists, resources should be allocated toward education and management of these most prevalent skin conditions., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: People with albinism (PWA) are at increased risk of photodamage and skin cancer. In many parts of Africa, there is a significant lack of knowledge regarding albinism which can lead to societal stigma, discrimination, and persecution from an early age. In the Democratic Republic of Congo (DRC), there is limited clinical data on PWA and skin cancer. We aim to better understand sociodemographics, risk factors, clinical features, and outcomes of this population., Methods: Patients with a diagnosis of albinism and skin cancer presenting to Kisangani Albino Clinic were enrolled., Results: Of 205 PWA, 61 patients were diagnosed with skin cancer with a mean age of 26.5 years. Common occupations were student (45.6%) or unemployed (26.4%). Discrimination was experienced from close contacts (24.4%) and society (67.4%). A majority (88.5%) had never used sunscreen, only 4.9% used fully sun protective clothing, and 90.2% spent 4 or more hours in the sun daily. Skin cancers had a mean size of 3.8 cm and were most commonly located on the face (47.7%). Squamous cell carcinoma was the most common histopathological diagnosis. Most patients underwent excision, and 90.2% had clinical clearance of tumors at a mean follow-up of 5.7 months., Conclusion: People living with albinism in the DRC experience a high rate of nonmelanoma skin cancers at a young age and additionally face a number of psychosocial challenges. This study represents the first attempt to analyze a cohort of patients with albinism from the DRC and serves to increase awareness of this vulnerable population., (© 2020 the International Society of Dermatology.)

Background: Primary cutaneous lymphoma (PCL) represents a heterogeneous collection of non-Hodgkin lymphomas originating in the skin. Our study describes the clinical and histological findings of cutaneous lymphoma within Botswana to expand the paucity of data on this rare disease in sub-Saharan Africa., Methods: We conducted a retrospective review from the dermatology clinic at Princess Marina Hospital (Gaborone, Botswana) of patients evaluated by skin biopsy for cutaneous lymphoma between 2008 and 2017. Patients with initial diagnostic suspicion for cutaneous lymphoma had biopsies re-reviewed by experienced dermatopathologists and were given a final diagnosis of either (i) cutaneous lymphoma, (ii) atypical lymphocytic infiltrate (ALI), or (iii) a reactive cutaneous process., Results: Thirty-eight cases were identified with a mean age of 50.0 years and a male:female (M:F) ratio of 13:6. Final diagnoses included: 27 cases of cutaneous lymphoma, eight cases of ALI, and three cases of reactive cutaneous processes. Subtypes of cutaneous lymphoma diagnosed included: mycosis fungoides (MF) (81.5%), plasmablastic lymphoma (7.4%), Epstein-Barr virus-positive T-cell lymphoma (3.7%), subcutaneous panniculitis-like T-cell lymphoma (3.7%), and peripheral T-cell lymphoma, not otherwise specified (3.7%). The most common immunohistochemical staining profile in MF cases was CD8 predominance over CD4., Conclusions: Primary cutaneous lymphoma causes significant morbidity and mortality globally. Given the limited resources in sub-Saharan Africa, it is essential to educate providers on the manifestations and histology of PCL. This study is an important step towards understanding the demographics, clinical presentation, histologic features, and mortality of patients diagnosed with PCL in Botswana and similar low-resource settings., (© 2019 The International Society of Dermatology.)

Most infantile hemangiomas (IHs), the most common vascular tumors of childhood, evolve without complications; however 10% to 12% require specialty referral for treatment. To emphasize the complications of late referral, we present a case of necrotizing infection within a segmental IH leading to sepsis. Early evaluation by a pediatric dermatologist could have prevented this life-threatening and disfiguring complication. We discuss how teledermatology would enable rapid triage of such critical cases in underserved areas, increasing access to high-value care and optimizing outcomes for our most vulnerable patients.