Kyphoscoliosis type of ehlers-danlos syndrome in a family

Ehlers-Danlos syndrome [EDS] is a generalized disorder of connective tissue characterized by hyperextensibility of skin, hypermobility of joints, and fragility of skin and blood vessels. According to new nomenclature EDS is classified in six major types. We are reporting kyphoscoliosis type of Ehlers-Danlos syndrome in two siblings

case report of chronic sclerosing panniculitis

Sclerosing panniculitis is a fibrotic process that usually occurs on the legs, commonly in women older than 40. The principal features are indurated woody plaques with erythema, edema, telangiectasia, and hyperpigmentation. Although the exact pathogenesis is uncertain, it is thought to occur as a result of ischemic changes. We present a 28-year-old married female who had a 10-year history of painful sclerotic plaques, repeated ulceration and healing with fibrosis of the both lower legs and abdomen. Venogram and Doppler investigations were normal. Skin biopsy from the edge of the ulcer demonstrated the feature of chronic sclerosing panniculitis. Satisfactory improvement was found with methotrexate 7.5mg weekly for 4 months. No recurrence was noted within 1 year follow up