A Unique Case of Progressive Hemi-facial Atrophy Successfully Treated with Methotrexate

Abstract

The effects of many dermatologic syndromes are not exclusive to the skin. Disorders commonly involve a complex interplay between multiple organ systems, thus not relying solely on the dermatologist for proper work up, diagnosis, and treatment. Morphea is one such rare disease which involves progressive loss or atrophy of subcutaneous tissue, muscle, and bone with a relatively mysterious etiology. The initial lesion of morphea can be subtle and appear as a pink to red plaque without any additional symptomatology. A biopsy at this early stage is non-specific and will only show the presence of a T cell infiltrate, vascular swelling, and edema. This active or progressive stage will continue for years before "burning out," or halting progression, although still affecting underlying tissues. Many times, the sclerosis becomes severe enough to cause deformity and secondary systemic symptoms. Five general subtypes of morphea exist, including: plaque-type, linear, deep, guttate, and nodular. In this paper, the authors report a case report of a rare subtype of linear morphea called Parry Romberg syndrome, also known as progressive hemi-facial atrophy (PHA). PHA usually involves at least one branch of the trigeminal nerve unilaterally. The authors will emphasize the importance of a multidisciplinary approach to diagnose and treat this disorder while also considering the multiple theories surrounding its pathophysiology.

Disturbances of the stomatognathic system and possibilities of its correction in patients with craniofacial morphea

“…The inability to close the lips freely increases the likelihood of diseases of the oral mucosa. The inability to close the edges of the lips also impairs the swallowing process [16,[20][21][22][23][24][25][26][27]. Structural deficits in the course of PHA translate into functional impairments in chewing, swallowing, speech and breathing.…”

“…The structure of tooth crowns usually does not differ from the norm. On the other hand, there are anomalies within the tooth roots, manifested by their distortion and shortening [16,20,[23][24][25][26][27]. Trigeminal neuralgia manifests itself in the form of acute pain (rarely dull and continuous) lasting from several seconds to several minutes, triggered during chewing, brushing teeth, washing the head [28] (Figure 1).…”