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.

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.

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.