Faculty Bibliography

Pilomatricomas are benign adnexal tumors which differentiate towards hair matrix cells. These lesions present most often as firm nodular tumors in the upper extremities, head and neck areas, with rare cases reporting pilomatricomas appearing on the trunk. Pilomatricomas can range from 0.5 to 3 cm in diameter with the classification of "giant" pilomatricoma reserved for tumors exceeding 5 cm in diameter. Four histopathologic stages have been described: early, fully developed, early regressive, and late regressive, all of which involve anucleated squamous cells known as "ghost" or "shadow" cells. There are known associations of this pathology with other conditions, most notably myotonic dystrophy, sarcoidosis, Gardner syndrome, Turner syndrome, Steinart disease, sternal cleft, and coagulative defects. Rarely, pilomatricomas can degenerate into pilomatricoma carcinoma. Treatment of this condition is excision of the tumor and the prognosis is favorable with virtually no recurrence. Pilomatricomas are most often seen in patients in their first two decades of life. We report a case of a 41-year-old Asian woman with no significant medical history who presented with painful lesions on her upper extremities bilaterally. The patient denied a family history of similar lesions and was worried she had skin cancer. On clinical exam, the patient had on each upper extremity, a roundshaped, blue and white colored, firm nodule. The lesions were completely excised without complications and were consistent with pilomatricomas on histology. Prior to our observations, Fernandez-Flores et al reported a 28-year-old male with multiple pilomatricomas associated with seminoma which was diagnosed several months before the pilomatricomas. In another case, a 22-year-old woman, also with no medical history, presented with multiple pilomatricomas distributed on her forearm, trunk, and eyebrow. Nevertheless, multiple pilomatricomas in an older patient and in the absence of associated disorders remains rare, which may be a product of underreporting rather than actual incidence. This case highlights the importance of considering pilomatricomas in the differential diagnosis in the setting of multiple growths, even in older patients

Scedosporium apiospermum, a ubiquitous environmental mold, is increasingly reported as causing invasive fungal disease in immunocompromised hosts. It poses a therapeutic challenge due to its intrinsic resistance to traditional antifungals and ability to recur despite demonstrating susceptibility. We present an immunocompromised patient with a cutaneous S. apiospermum infection that disseminated despite treatment with voriconazole, the drug of choice. Adding echinocandins and GM-CSF provided partial recovery, indicating a potential synergistic role of dual-antifungal and immunotherapeutic agents.

Cutis verticis gyrata that involves only the face is a rare presentation of this even rarer cutaneous anomaly. We present a 61-year-old man, who developed primary essential progressive cutis verticis gyrata of the face.

Nodular amyloidosis is the rarest form of primary cutaneous amyloidosis. We report the case of a 74-year-old woman with an eight-year history of asymptomatic, hyperpigmented plaques on the pretibial areas. A skin biopsy specimen showed deposits of amorphous eosinophilic material that extended throughout the dermis with apple-green birefringence with a Congo-red stain, which established a diagnosis of nodular amyloidosis. Patients with nodular amyloidosis should be evaluated for systemic disease and followed appropriately due to a small risk of progression to systemic amyloidosis.

A 56-year-old man presented with a 15-year history of scaly red plaques on the trunk and axillae. Skin biopsy was consistent with Hailey-Hailey disease. His condition was refractory to multiple therapies, which included topical and oral antibiotics and topical, intralesional, and oral glucocorticoids. Treatment with subcutaneous botulinum toxin type A at the axillae and on the back led to a nearly complete resolution of plaques in those areas. Botulinum toxin type A should be considered in patients with extensive Hailey-Hailey disease who are fail traditional therapies.

We present a 53-year-old woman with diffuse macular amyloidosis. We discuss the clinical manifestations, pathophysiologic mechanisms, and associations of cutaneous macular amyloidosis.

Lupus miliaris disseminatus faciei (LMDF) is a rare, inflammatory condition, which is characterized by red-brown and yellow-brown papules on the face, with characteristic involvement of the eyelids and with histopathologic findings of suppurative and granulomatous folliculitis and dermatitis. The etiology of this disease is not known, but retinoids, anti-inflammatory, immunosuppressive, and antimicrobial medications are utilized to treat the condition with variable results. We present the case of a patient with LMDF that has thus far been refractory to treatment.

We present a 69-year-old man with type 2 diabetes mellitus and a five-year history of an eruption of follicular pustules, papules, and nodules, which was identified histopathologically as folliculocentric granuloma annulare (GA). Folliculocentric generalized GA is a rarely reported variant of GA, in which the the palisading histiocytes form focal granulomas in a follicular pattern. In this case, the GA may represent an isotopic phenomenon, with lesions developing in hair follicles that were previously affected by a suppurative folliculitis.