Frontal linear scleroderma (also known as en coup de sabre or morphea en coup de sabre) is a type of linear scleroderma characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal scalp. Multiple lesions of en coup de sabre may coexist in a single patient, with one report suggesting that the lesions followed Blaschkos lines. It gets its name from the perceived similarity to a sabre wound. Atrophoderma of Pasini and Pierini (also known as "Dyschromic and atrophic variation of scleroderma," "Morphea plana atrophica," "Sclérodermie atrophique demblée") is a disease characterized by large lesions with a sharp peripheral border dropping into a depression with no outpouching, which, on biopsy, elastin is normal, while collagen may be thickened. Atrophoderma of Pasini and Pierini affects less than 200,000 Americans and is classified as a rare disease by http://rarediseases.info.nih.gov. The disease results in round or oval patches of hyper-pigmented skin. The darkened skin patches may sometimes have a bluish or purplish hue when they first appear and are often smooth to the touch and hairless. Treatment
Throughout the years, many different treatments have been tried for morphea including topical, intra-lesional, and systemic corticosteroids. Antimalarials such as hydroxychloroquine or chloroquine have been used. Other immunomodulators such as methotrexate, topical tacrolimus, and penicillamine have been tried. Children and teenagers with active morphea (linear scleroderma, generalised morphea and mixed morphea: linear and circumscribed) may experience greater improvement of disease activity or damage with oral methotrexate plus prednisone than with placebo plus prednisone.
Signs and symptoms
Uvietis, Glaucoma, and Hyphema are the classic sins of UGH syndrome, but the term is often used when one, two, or all three signs are present in the presence of any IOL causing mechanical irritation of the iris or angle structures. Blurred vision, transient vision loss, eye pain, photophobia, erythropsia (objects appear red) are the main symptoms. Diagnosis
The diagnosis of UGH Syndrome is mainly based on patient history and eye examination. Patient will have history of cataract surgery with intraocular lens implantation. Slit-lamp examination may reveal hyphaema, aqueous cells and flare, iris neovascularization, mispositioned IOL, iris-lens contact, iris transillumination defects etc. Variations
UGH Plus and IPUGH (Incomplete Posterior UGH) are the variations of UGH syndrome. IPUGH is defined as bleeding into the posterior chamber with/ without glaucoma and no uveitis. UGH Plus is defined as a UGH syndrome plus a vitreous hemorrhage and occurs more frequently with anterior chamber IOLs but can occur with any IOLs. Treatment
Initially, topical and systemic medication to control inflammation and raised IOP is appropriate, but the definitive treatment is an IOL reposition or exchange. Topical corticosteroids may be used to control anterior inflammation. Raised IOP can be lowered using topical and systemic anti-glaucoma medications such as prostaglandin analogs, beta-adrenergic antagonists, alpha-adrenergic agonists, and carbonic anhydrase inhibitors etc. History
The UGH Syndrome was originally described by Ellingson in 1978 and classically included uveitis, glaucoma, and hyphaema in the setting of an anterior chamber IOL. == References ==