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.

Keywords: morphea; parry-romberg syndrome; progressive hemi-facial atrophy.

Figure 1:. Clinical image demonstrating the atrophic, and hypo pigmented lower cheek/jaw

Figure 2:. Hematoxalin and eosin Histology image showing atrophy of the epidermis, dermis, and thickening of the collagen with little inflammation