Multiple Pilomatricomas Associated with Myotonic Muscular Dystrophy in Brother and Sister / 대한피부과학회지

Pilomatricoma is a tumor with differentiation toward hair cortex cells. There have been a few cases of multiple familial pilomatricomas in association with myotonic muscular dystrophy. We report two members of the family(brother and sister) with association of multiple pilomatricomas and myotonic muscular dystrophy. Multiple familial pilomatricomas may be a cutaneous manifestation of the myotonic syndrome.

Low-dose UVA1 Phototherapy for Localized Scleroderma / 대한피부과학회지

Localized scleroderma(LS) is sclerosis of the skin characterized by one or multiple circumscribed ivory-white, indurated, sometimes confluent plaques. It has been reported that LS might result from the unbalance between synthesis and degradation of collagen in the dermis. Recently, treatment of LS with long wave UVA1, which can induce mRNA of matrix metalloproteinase-1 from dermal fibroblast and can cause apoptosis of infiltrating T lymphocytes, showed promising results. In this case, a 14-year-old girl had a 6 month history of linear, brown colored, indurated plaque on her left thigh compatible with LS histopathologically. UVA1(2.4-10.8J/cm2) was irradiated to the skin lesion at each visit, a total of 68 times for 18 months with the cumulative dose of 533J/cm2 UVA1. Her fibrotic skin lesion was resolved during treatment, but became hardened with cessation of phototherapy. She remains disease free for 11 months. We report a case of LS with improvement with low-dose UVA1 phototherapy.