Five-year pattern of adnexal tumors of the skin in Ethiopia.

BACKGROUND: Adnexal tumors of the skin are rare neoplasms that encompass a wide range of dermatologic entities. Here, we investigated the pattern of adnexal tumors of the skin in the All African Leprosy and Tuberculosis Rehabilitation and Training Center (ALERT) hospital retrospectively. METHODS: A hospital-based retrospective study was conducted at ALERT from histopathology records in the Armauer Hansen Research Institute (AHRI) pathology laboratory of patients diagnosed with any of the skin adnexal tumors during the time period January 2017 to December 2021. A structured data extraction sheet was used. Data entry was done using EpiData 4.6.0.6. Data were analyzed using SPSS version 25. RESULT: A total of 146 skin adnexal tumors were identified making the magnitude 2.8% of total biopsies. The 3rd decade of life was found to be the most common age group. Male-to-female ratio was 1 : 1.05. Majority of the tumors were benign (82.2%) and had sweat gland differentiation at 48.6%. Poroma (10.9%) was the most frequent tumor, whereas porocarcinoma (6.8%) made up the most frequent malignant tumor. The most common site was the head and neck region (48.6%). Only 21.2% of the tumors were correctly identified clinically. CONCLUSION: The magnitude of skin adnexal tumors is found to be slightly higher than other similar studies which could be because it was carried out in the largest dermatologic center in the country. The most common skin adnexal tumors identified, their localizations, and lines of differentiation are all in line with other studies. Histopathologic examination is mandatory for the accurate diagnosis of these tumors.

Role of the NLRP1 inflammasome in skin cancer and inflammatory skin diseases.

Inflammasomes are cytoplasmic protein complexes that play a crucial role in protecting the host against pathogenic and sterile stressors by initiating inflammation. Upon activation, these complexes directly regulate the proteolytic processing and activation of proinflammatory cytokines interleukin (IL)-1ß and IL-18 to induce a potent inflammatory response, and induce a programmed form of cell death called pyroptosis to expose intracellular pathogens to the surveillance of the immune system, thus perpetuating inflammation. There are various types of inflammasome complexes, with the NLRP1 (nucleotide-binding domain, leucine-rich-containing family, pyrin domain-containing-1) inflammasome being the first one identified and currently recognized as the predominant inflammasome sensor protein in human keratinocytes. Human NLRP1 exhibits a unique domain structure, containing both an N-terminal pyrin (PYD) domain and an effector C-terminal caspase recruitment domain (CARD). It can be activated by diverse stimuli, such as viruses, ultraviolet B radiation and ribotoxic stress responses. Specific mutations in NLRP1 or related genes have been associated with rare monogenic skin disorders, such as multiple self-healing palmoplantar carcinoma; familial keratosis lichenoides chronica; autoinflammation with arthritis and dyskeratosis; and dipeptidyl peptidase 9 deficiency. Recent research breakthroughs have also highlighted the involvement of dysfunctions in the NLRP1 pathway in a handful of seemingly unrelated dermatological conditions. These range from monogenic autoinflammatory diseases to polygenic autoimmune diseases such as vitiligo, psoriasis, atopic dermatitis and skin cancer, including squamous cell carcinoma, melanoma and Kaposi sarcoma. Additionally, emerging evidence implicates NLRP1 in systemic lupus erythematosus, pemphigus vulgaris, Addison disease, Papillon-Lefèvre syndrome and leprosy. The aim of this review is to shed light on the implications of pathological dysregulation of the NLRP1 inflammasome in skin diseases and investigate the potential rationale for targeting this pathway as a future therapeutic approach.

Follicular mycosis fungoides: Clinicohistopathologic features and outcomes in a series of 12 Chinese cases.

Background Follicular mycosis fungoides is a distinct variant of mycosis fungoides with a broad clinical spectrum. Recently, many studies have indicated that follicular mycosis fungoides should be divided into different subtypes with disparate prognoses. Objective To define the clinicohistopathologic features and outcomes of follicular mycosis fungoides and to identify risk factors that may be related to the prognosis of Chinese patients with follicular mycosis fungoides. Materials and methods We conducted a single-centre retrospective study and reviewed the clinical, histopathologic and immunophenotypic data of 12 patients diagnosed with follicular mycosis fungoides between 2009 and 2020 in the Department of Dermatology of West China Hospital of Sichuan university. Results A total of 12 patients (seven males and five females) with a mean age of 30 ± 14 years (age range 16-55 years) were included. Scalp and face were the most common involved sites (100%). Follicular papules, acneiform lesions, plaques, and nodules, were the main clinical presentations. Histopathological findings were consistent with the classic manifestations of follicular mycosis fungoides, including folliculotropism, perifollicular and intrafollicular lymphocytic infiltrates and mucinous degeneration. Interferon α-1b was the most common treatment. Four patients died of follicular mycosis fungoides in three years. Notably, immunohistochemical analysis revealed a decreased number of CD20+ cells in the deceased patients. Limitations This is a retrospective evaluation with a small number of cases; further prospective studies are warranted to support our inferences. Conclusion Our patients were much younger than in previous studies. The observed difference in this cohort may be explained by race, in addition to the limited number of cases. A decreased number of B cells might be associated with a poor prognosis, and more studies are necessary to discover the role of B cells in follicular mycosis fungoides as well as in mycosis fungoides.

Clinical analysis of 20 cases of cutaneous extranodal NK/T-Cell lymphoma.

Background To investigate the clinical features, pathological features and prognostic factors of cutaneous extranodal natural killer/T-cell lymphoma (CENKTL). Methods A total of 20 cases with CENKTL from February 2013 to November 2021 were analysed retrospectively. Results The patients included 15 men and five women, and their ages ranged from 19 to 92 (median age of 61) years. The most common lesions were on the extremities, followed by the trunk. Histopathological examination showed atypical lymphocyte infiltrate in dermis and subcutaneous fat. The tumour tissue showed vascular proliferation, vascular occlusion, and coagulation necrosis. In situ hybridisation revealed that 20 patients were positive for Epstein-Barr virus-coding ribonucleic acid. Immunohistochemistry showed that the tumour cells were positive for CD3 (18/20 and 90%), CD56 (19/20 and 95%), T-cell intracellular antigen (TIA-1) (13/14 and 92.9%) and CD20 (5/20, 25%). About 20 patients were positive for Ki-67 with values of 30-90%. A total of 11 of the 20 patients died, and two patients were lost to follow-up. The 2-year overall survival was 24%, and the median overall survival was 17 months. Univariate analysis revealed that involvement of lymph nodes (P = 0.042) correlated with worse survival. Limitation This is a retrospective study design and has a limited number of patients. Conclusion CENKTL is rare and has a poor prognosis. Diagnosis is challenging due to non-specific clinical symptoms and histopathology results. A comprehensive judgement should be made based on related clinical manifestations and histopathological and molecular examination. Lymph node involvement is an independent prognostic factor for CENKTL.

Reactive Eccrine Syringofibroadenoma (ESFA) in Association With a Merkel Cell Carcinoma Excision Scar.

ABSTRACT: Eccrine syringofibroadenoma (ESFA) is a rare benign skin adnexal lesion of the acrosyringium of eccrine sweat ducts. Reactive ESFA, a subtype of ESFA, is usually associated with non-neoplastic cutaneous dermatoses or neoplastic skin tumors. Clinically, the lesions can be solitary or multiple, pink, or skin-colored coalescing papules or nodules of variable sizes. Histopathologically, this tumor is composed of numerous anastomosing cords of monomorphic cuboidal epithelial cells with eccrine duct formation. The association of reactive ESFA with benign conditions, such as psoriasis, diabetic polyneuropathy, scars, and leprosy, has been reported. However, the association of reactive ESFA with malignant tumors is extremely rare, with very few cases reported in the literature. We present a case of a 72-year-old woman who developed reactive ESFA associated with Merkel cell carcinoma excision scar. The ESFA tumors developed in the area of the surgical graft 10 months after the Merkel cell carcinoma had been excised. New ESFA tumors have continued to appear in the scar on a yearly basis while, so far, has been no recurrence of the original tumor. However, the presence of new tumor growths in the area suggested the possibility of recurrence of the Merkel cell carcinoma. That possibility was enhanced by the fact that PET scans revealed hypermetabolic activity in the ESFA papules.

Dermoscopic features of basal cell carcinoma in skin of color: A retrospective cross-sectional study from Puducherry, South India.

BACKGROUND: Dermoscopy is useful in the diagnosis of basal cell carcinoma (BCC). However, most descriptions of the dermoscopic features of BCCs are in Caucasians (skin types I-III) and there is a paucity of data in dark-skinned Indian patients. AIMS: The aim of this study was to describe the various dermoscopic features of BCC in dark-skinned patients from South India and correlate these with the histopathologic subtypes. METHODS: A retrospective observational study of biopsy-proven cases of BCC was conducted at a tertiary care center in South India using nonpolarized contact dermoscopy. RESULTS: Sixty BCCs in 35 patients predominantly of skin phototypes IV or V were studied. These included 32 nodular, 27 superficial and 1 infiltrative type of BCC. The most common dermoscopic features noted were maple leaf-like areas (61.7%), blue-white veils (53.4%), ulceration (48.4%) and short fine telangiectases (46.7%). Ulceration, blue-white veils and arborizing vessels were significantly associated with nodular BCCs, while maple leaf-like areas, red-white structureless areas, multiple small erosions and spoke wheel areas were noted with superficial BCCs. LIMITATIONS: The limitations of this study include its retrospective nature, the use of only nonpolarized light for examination, the lack of other histopathological variants of BCC as well as the lack of a comparison group. CONCLUSION: We report a dermoscopic study of BCC in dark-skinned patients from Puducherry, South India. The blue-white veil was observed in half of the patients and was significantly associated with nodular BCCs. The addition of the blue-white veil to the diagnostic criteria for pigmented BCC could improve the diagnostic accuracy of dermoscopy in Indian patients.

Onychomatricoma on the fourth toenail: A rare tumor in a rare localization.

Onychomatricoma is a rare benign tumor of the nail matrix, characterized by finger-like projections that invade the nail plate. The fingernails of Caucasian women are most commonly affected. Because this tumor can easily mimic other more prevalent ungual diseases, it is crucial to be acquainted with its characteristic clinical and histopathologic features. The authors present a case of a 40-year-old man with an onychomatrichoma in the fourth left toenail, which was initially misdiagnosed and treated as onychomycosis.