Hidradenitis Suppurativa in a Patient with Smith-Magenis Syndrome: A Case Report.

ABSTRACT

Hidradenitis suppurativa (HS) is an inflammatory disorder characterized by recurrent painful nodules and draining sinus tracts with subsequent scarring. HS has been associated with several dermatologic syndromes including the follicular occlusion tetrad, PASH (pyoderma gangrenosum, acne, and suppurativa hidradenitis), PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, and suppurativa hidradenitis), and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndromes. However, aside from the well-established association between HS and Down Syndrome, it has rarely been reported in association with other multisystem genetic diseases. We report a case of HS in a 22-year-old man with Smith-Magenis syndrome (SMS), a developmental disorder caused by a chromosomal 17 microdeletion. Obesity and a tendency toward follicular occlusion are features of SMS that may have contributed to the development of HS in our patient. Interestingly, the patient also had several other follicular-based skin diseases including erythromelanosis follicularis faciei, keratosis pilaris, and acne keloidalis nuchae. Dermatologic findings in SMS have been characterized in the literature in the past, but to our knowledge, this is the first case of HS reported in a patient with SMS. We also provide a brief literature review of cases of HS occurring in patients with other multisystem genetic diseases.