PubMed · 14215150
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R BONDI, L AMBROSI. 1963. [ANGIOBLASTIC MALIGNANCY].. https://pubmed.ncbi.nlm.nih.gov/14215150/
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Infantile systemic hyaloinosis is a rare, progressive, and fatal disease that is inherited in an autosomal recessive fashion. We describe 2 patients in whom thickened skin; small nodules of the perianal region, face, and neck; joint contractures; growth failure; diarrhea; and frequent infections developed within the first few weeks of life. Both patients died before 2 years of age.
BACKGROUND: Perianal hidradenitis suppurativa is a chronic recurrent inflammatory, suppurating, and fistulizing disease of apocrine glands, adjacent anal canal skin, and soft tissues. The standard treatment used for extensive cases is a staged surgical procedure allowing the wound to heal by secondary intention or the delayed use of skin grafts. CASE PRESENTATION: A long-standing case, disabling for the patient, with extensive involvement of the buttock region, treated in one stage, which for reconstruction required the use of sliding plasties and free skin grafts, is reported. RESULTS: The outcome was satisfactory. Primary closure after wide excision using plastic-surgery techniques may help us resolve complex situations and obtain good results and a rapid recovery. CONCLUSION: The method of closure with a combination of skin flaps and skin graft in one stage can be considered a valid surgical option for a group of patients with extensive perianal hidradenitis.
Uterine leiomyomas (fibroids) are very common lesions with well-described imaging features. We present an unusual case of a fibroid which presented in the buttock with atypical clinical and imaging features in a woman who had had a previous hysterectomy. Despite extensive imaging and biopsies, the diagnosis was not suspected until pathological evaluation of the resected mass. The differential diagnosis for such a lesion, and the imaging and pathological features of fibroids, including atypical and parasitic leiomyomas, are discussed.