Angiosarcoma is a relatively rare soft tissue tumour. It usually occurs in the head and neck, and especially in the scalp, in elderly people. Its presentation varies from a small plaque to multifocal nodules. The treatment depends on the extent of the disease. Most cases are treated with wide excision with reconstruction. Radiotherapy and chemotherapy are advocated in the recurrent or extensive lesions with regional or distant metastasis. Other modalities such as immunomodulation have been tried. A case of a 55-year-old female patient with a bleeding scalp lesion is presented. Initially thought to be a pyogenic granuloma, on excisional biopsy it was diagnosed as angiosarcoma with microscopic involvement of the margins. Wide excision with reconstruction using a local rotation flap was done at a second stage. The patient was not given postoperative radiotherapy or chemotherapy. There has been no recurrence for two years.
Background:Kyrle's disease is a rare variant of primary perforating dermatosis. Its occurrence in a familial setting, especially in children, is extremely uncommon. Similar appearing skin lesions have been described in adults, secondary to metabolic disorders, infective agents as well as exposure to chemicals. We present a rare case of this genodermatosis in two siblings.Materials and Methods:Two siblings of a non-consanguineous marriage came with generalized discrete papular lesions with a central keratotic plug. All biochemical and serological investigations were within normal limits. Serial sections of the biopsy revealed typical epidermal invaginations filled with parakeratotic debris and perforation into the dermis with accompanying granulomatous reaction.Results and Conclusions:A careful history, detailed routine investigations and serial sections of the skin biopsy are required to demonstrate the typical morphology and stages of evolution of Kyrle's disease. This helps to differentiate the rare primary Kyrle's disease from other primary and secondary keratotic lesions. Due to the familial occurrence, screening of relatives of an index case is recommended.
Angiosarcoma is a relatively rare soft tissue tumour. It usually occurs in the head and neck, and especially in the scalp, in elderly people. Its presentation varies from a small plaque to multifocal nodules. The treatment depends on the extent of the disease. Most cases are treated with wide excision with reconstruction. Radiotherapy and chemotherapy are advocated in the recurrent or extensive lesions with regional or distant metastasis. Other modalities such as immunomodulation have been tried. A case of a 55-year-old female patient with a bleeding scalp lesion is presented. Initially thought to be a pyogenic granuloma, on excisional biopsy it was diagnosed as angiosarcoma with microscopic involvement of the margins. Wide excision with reconstruction using a local rotation flap was done at a second stage. The patient was not given postoperative radiotherapy or chemotherapy. There has been no recurrence for two years.
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