Abstract Pigmented epithelioid melanocytoma (PEM) represents a group of rare, heavily pigmented melanocytic tumors encompassing lesions previously designated as “animal‐type melanomas” and “epithelioid blue nevi.” Despite the association of multiple such tumors in the setting of Carney complex, most cases of PEM occur spontaneously as solitary neoplasms in otherwise healthy patients. PEM may arise in both children and adults, and has a known propensity to spread to the regional lymph nodes. Despite this latter finding, recurrence at the biopsy site or spread beyond the lymph node basin is exceptionally uncommon. Although the molecular basis for PEM continues to be characterized, findings to date suggest that this category of melanocytic neoplasia has genetic alterations distinct from those seen in common nevi, dysplastic nevi, Spitz nevi, and melanoma. Herein, we present an in‐depth clinical, histopathologic, and molecular analysis of a case of PEM occurring on the scalp of a young African American girl found to have a novel NTRK3‐SCAPER gene fusion.
Keratoacanthoma (KA) is a common skin tumour that remains controversial regarding classification, epidemiology, diagnosis, prognosis and management. Classically, a KA manifests as a rapidly growing, well-differentiated, squamoid lesion with a predilection for sun-exposed sites in elderly people and a tendency to spontaneously regress. Historically, KAs have been considered a variant of cutaneous squamous cell carcinoma (cSCC) and are often reported as KA-type cSCC. However, the penchant for regression has led many to categorize KAs as biologically benign tumours with distinct pathophysiological mechanisms from malignant cSCC. The clinical and histopathological similarities between KA and cSCC, particularly the well-differentiated variant of cSCC, have made definitive differentiation difficult or impossible in many cases. The ambiguity between entities has led to the general recommendation for surgical excision of KAs to ensure a potentially malignant cSCC is not left untreated. This current standard creates unnecessary surgical morbidity and financial strain for patients, especially the at-risk elderly population. There have been no reports of death from a definitive KA to date, while cSCC has an approximate mortality rate of 1·5%. Reliably distinguishing cSCC from KA would shift management strategies for KAs towards less-invasive treatment modalities, prevent unnecessary surgical morbidity, and likely reduce associated healthcare costs. Herein, we review the pathophysiology and clinical characteristics of KA, and conclude on the balance of current evidence that KA is a benign lesion and distinct from cSCC.
Port-wine birthmark (PWB) is a congenital slow-flow capillary malformation of the cutaneous superficial vascular plexus. Occurring in up to 0.5% of newborns, PWB shows no gender predilection and is equally prevalent in premature and full-term infants.1 PWB appears most often on the face and persists throughout life. At birth, PWB typically presents as a well-defined, bright or deep red patch. With time, it expands commensurate with the child's growth, but has a propensity to become darker, hypertrophic, nodular, and potentially disfiguring, leading to significant functional impairment and psychosocial morbidity later in life.2
The current modality of choice for treatment of PWB is high-energy pulsed dye laser (PDL), which selectively targets hemoglobin and results in photocoagulation of the PWB vessels.1 When performed by a highly trained expert at sufficient frequency, PDL is a safe, effective treatment and successful in most patients, with earlier treatment yielding maximal clearance.3
Infants can begin treatment of PWB safely within the first few days after birth as an in-office procedure. Early initiation of PDL therapy during infancy is well-tolerated, provides superior long-term results, and produces a much less evident lesion by the time the child is psychologically aware.1,3,4 However, optimal timing of the initiation of treatment has yet to be established in prematurely born infants.
We present a case of facial PWB in a premature infant who initiated treatment 20 days before his due date using the 595-nm PDL with no adverse effects. This early approach appears to represent a safe treatment option for premature infants with PWB.
