Giant Koenen Tumors as the Presenting and Only Clinical Sign of Tuberous Sclerosis Complex in a 56-Year-Old Man and Its Surgical Management.

Introduction: Tuberous sclerosis complex (TSC) is a genetic multisystem disorder with prominent skin involvement. Multiple ungual fibromas, also known as Koenen tumors (KTs), are one of the major diagnostic criteria and occur approximately in 50% of patients with TSC. They are disfiguring, painful, and challenging to treat as they frequently recur. There are 2 previous cases in the literature of KTs as the only clinical sign of TSC. Case Presentation: A 56-year-old male patient with intellectual disability presented with a history of ungual growths that had gradually increased in size and number for 20 years. The diagnosis of KTs was established on clinical grounds, without the requirement of histological confirmation. No additional clinical evidence of TSC was found. Complete surgical excision of the tumors located in the proximal nail matrix, periungual folds, and nail bed of each toe was successfully performed. Discussion: The association between KTs and intellectual disability with TSC supported the clinical diagnosis. To date, there is no standard first-line treatment for KTs. Complete surgical excision up to the proximal base of the tumor offers satisfactory results in terms of functionally and esthetic appearance and could be considered the preferred therapeutic modality for patients with severe functional and cosmetic impairment. Prompt recognition of the many signs and symptoms associated with TSC is important to achieve early diagnosis, preventing further complications.

Nail Changes in People Living with Human Immunodeficiency Virus: Observational and Cross-Sectional Study in a Third-Level Hospital.

Introduction: Nail changes in people living with human immunodeficiency virus (HIV) have been scarcely reported. The aim of this study was to establish the frequency and characteristics of nail alterations observed in adults with HIV infection in a third-level hospital in Mexico. Method: Observational and cross-sectional study carried out in 205 patients receiving care at the HIV/AIDS Clinic of the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán (INCMNSZ) in Mexico City. We performed a nail and iconographic assessment of both hands and toenails. We collected information of demographic and clinical variables, as well as drugs use, and antiretroviral treatment used by the participants through a questionnaire and from medical records. We performed direct cytological examinations and nail mycological cultures in participants with symptoms of onychomycosis. Results: The participants were predominantly male patients (91.2%), with a mean age of 41 (range 21-78) years, under antiretroviral therapy (91.2%), with a suppressed viral load (78.5%) and mean CD4+ lymphocyte count of 379.5 (range 20-1,162) cells/µL. Fitzpatrick's IV phototype was prevailing in the studied population (70%). Nail changes were documented in 72.2% of the patients; being pigmentary changes (37.1%) and trauma (30.7%) the most frequent. Onychomycosis was observed in 26.3%; with total dystrophic onychomycosis as the most frequent clinical variant (68.5%). We obtained fungal isolates in 59.3% of participants and Candida parapsilosis was the most frequent of these (37.5%). Conclusions: We observed a high prevalence of nail changes with very diverse etiology, as well as a variety of nondermatophytic yeasts and molds isolates associated with cases with onychomycosis. These findings reinforce and confirm the need for routine nail examination and stress the importance of medical personnel working with people living with HIV to have broad knowledge of nail pathology.

Onycholemmal Horn: An Exceedingly Rare Subungual Tumor.

The onycholemmal horn is an exceedingly rare subungual tumor characterized by trichilemmal-like keratinization pattern. The only previous instance of onycholemmal horn dates back to 1983 when Haneke reported a keratotic subungual tumor clinically and histologically comparable to a trichilemmal horn. No other case of this condition has been reported so far. We present the case of a 72-year-old female patient, with a history of a slowly growing tumor originating from the nail bed epithelium of the left thumb. The lesion was surgically removed, and pathological examination was consistent with the diagnosis of an onycholemmal horn. Additionally, this study aims to elucidate the correlation between the onycholemmal and trichilemmal keratinization.

Pain Management in Patients with Severe Pemphigus Vulgaris.

To describe the pain management and clinical course of patients with severe Pemphigus Vulgaris (PV) admitted to a third-level Intensive Care Unit (ICU). This was a retrospective cohort study conducted in the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán over the period 2013-2020. Study population comprised patients with severe PV admitted to the ICU. Eleven patients with severe PV were included. Mean age of presentation was 43.6 years. Percentage of the total body surface area affected ranged from 70 to 85% (mean: 80%). Visual Analogue Scale was used for pain assessment upon admission. Nine patients (72%) reported a 10/10 pain. The median Morphine Equivalent Daily Dose was 200 mg (range: 90-518 mg). Mortality was 27% during the ICU stay. One patient (9%) continued to experience severe pain and consume opioids after discharge. PV is a life-threatening disease characterized by painful, persistent erosions and ulcers. Integrated and multidisciplinary approach is often required. Opioids remain the mainstay for acute pain control in patients with severe PV. Biological, psychological, and social factors influence patients' daily opioid requirements and dose escalation. Successful pain management contributes to improving the quality of life, and the suppression and remission of PV.

Frontal Fibrosing Alopecia: Is There a Link in Relatives?

Frontal fibrosing alopecia (FFA) is an acquired primary lymphocytic cicatricial alopecia characterized by frontotemporal hairline recession, leading to scarring alopecia with a band-like distribution. Prevalence is increasing worldwide, being the most frequent cause of primary scarring alopecia. The natural history of this condition is variable; however, slow progression with spontaneous remission is the most frequent reported outcome. The etiopathogenesis of FFA remains to be elucidated; numerous hypotheses concerning hormonal effects, environmental factors, and genetic predisposition have been proposed. Special interest on genetic basis has emerged since the first familial case was reported. Only a few more familial cases have been published. We report 6 additional cases of female patients with familial FFA (F-FFA) from 3 different families. Sixty-six percent had a family history of autoimmune disease in first-degree relatives; these same patients had a personal history of autoimmune disease. The families described in this cohort study plus the personal and family history of autoimmune disease, as well as the recently described involved genomic loci; reinforced the hypothesis of this disease being genetic. It is important to consider studying this entity since there are scarce data regarding familial cases and this might give us a better insight toward understanding its pathogenesis.

Painful nails: A practical approach to the diagnosis and management of painful nail conditions.

Because several nail disorders share similar clinical features, their diagnosis and management can be challenging to clinicians. The physical examination may disclose localized abnormalities or point to an underlying systemic disease, requiring additional workup. Furthermore, cosmetic distress and nail-related symptoms (e.g., tingling, stinging, numbness, and pain) are common factors that influence the patient's search for medical assistance. Nail pain (i.e., onychalgia) can accompany both localized and systemic pathology. Onychalgia can be acute or chronic according to the time of evolution; patients may describe it as intermittent or constant, and as a throbbing, burning, sharp, or shooting sensation denoting the nature of the pain. It may be exacerbated by colder temperatures, touch, and increased activity (e.g., manipulating objects, walking). We present four main groups of conditions that might cause nail pain: nail tumors, nail deformities, inflammatory or infectious diseases, and external or traumatic agents. Our article includes an overview of the clinical features, as well as diagnosis and management pearls for each entity. Physicians (dermatologists and nondermatologists) should be aware that abnormalities of the ungual and subungual space are not exclusive of dermatological disorders but may also be present in noncutaneous contexts.