Thursday, 7 December 2017

Steatocystoma Multiplex: A Case Report of a Rare Disease Diagnosed in a Trauma Patient


Steatocystoma Multiplex is a rare, benign skin disorder that can manifest itself in the head and neck region and can be significantly disfiguring. There is minimal reference of this condition in oral and maxillofacial surgery literature. Steatocystoma Multiplex is characterized by multiple lesions of the skin in more than one location. The distribution of steatocystomas typically include the regions of the body that contain sebaceous follicles, most notably the face, anterior chest wall, and limbs [1, 2].However, steatocystomas can occur anywhere and have been reported on the oral mucosa and in the subdermal tissue. The prevalence of these lesions is not currently known, which is likely due to the fact that they are rarely reported. The lesions are smooth and round macroscopically, ranging in size from a few millimeters to several centimeters. This case report describes the work-up and diagnosis of a 35-year-old male with Steatocystoma Multiplex who was clinically triaged by the Emergency Department at Nassau University Medical Center on two separate occasions as having other epithelial pathologies.

On January 22, 2015, a 35-year-old African American male presented to Nassau University Medical Center status post-assault with the chief complaint of pain to the right malar region. The previous medical history of this patient included well-controlled hypertension and multiple lumps throughout the body described as neurofibromas, suggestive of neurofibromatosis type I (“NF1”). Evaluation of the patient’s chief complaint by examination and computed tomography revealed a moderately displaced right zygomaticomaxillary complex (“ZMC”) fracture. Subsequent evaluation of the patient revealed asymptomatic, non-inflamed, well-circumscribed, yellowish, raised lesions located throughout the body with increased frequencies in the head and neck.

The overlying epithelium was normal in appearance, with the largest lesion approximately 2 centimeters in diameter. The patient stated these lesions initiated around the time of puberty and that he believed they were related to his acne. In addition, he stated his mother had similar lesions on her cheeks, but they were significantly smaller and fewer in frequency. Routine laboratory tests and chest imaging were within normal limits (Figure 4). The patient elected to undergo an open reduction and internal fixation of his right ZMC fracture. A biopsy of a lesion was performed through the existing incision in the right lateral brow region and submitted for histological evaluation. The histologic diagnosis of the lesion was reported as a steatocystoma, but unfortunately not conveyed to the patient as he failed to follow-up.

1 comment: