title: Pneumatosis cystoides intestinalis with systemic sclerosis, limited type resulting in a poor prognosis.
abstract: Pneumatosis cystoides intestinalis (PCI) is a rare disease characterized by the presence of multilocular intramural clusters of gas in the alimentary tract and has been considered to have a favorable response to conservative treatment. We describe the first case of limited type of systemic sclerosis (SSc) with PCI. A 74-year-old Japanese woman presented with a 4-month history of an unhealed cutaneous ulcer on the right third finger, along with sclerodactyly of bilateral hands. Proximal skin sclerosis was absent. The patient reported acute abdominal pain, and a diagnosis of PCI was established on plain radiography. The patient died of multiple organ failure 5 months after the development of PCI. PCI is rarely complicated with SSc, and all cases previously reported were associated with diffuse SSc. Because PCI is one of the poor prognostic factors of SSc, we should recognize the presence of this condition even in patients with limited cutaneous involvement.
authors.affiliation: Division of Dermatology, Kitakyushu Municipal Medical Center, Kitakyushu, and Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.; Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.
authors.forename: Manabu
authors.initials: M
authors.lastname: Hamada
authors.affiliation: Division of Dermatology, Kitakyushu Municipal Medical Center, Kitakyushu, and Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.; Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.
authors.forename: Miki
authors.initials: M
authors.lastname: Kayashima
authors.affiliation: Division of Dermatology, Kitakyushu Municipal Medical Center, Kitakyushu, and Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.; Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Morai
authors.affiliation: Division of Dermatology, Kitakyushu Municipal Medical Center, Kitakyushu, and Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.; Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.
authors.forename: Teiichi Chi
authors.initials: TC
authors.lastname: Masuda
authors.affiliation: Division of Dermatology, Kitakyushu Municipal Medical Center, Kitakyushu, and Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.; Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.
authors.forename: Kazunori
authors.initials: K
authors.lastname: Urabe
authors.affiliation: Division of Dermatology, Kitakyushu Municipal Medical Center, Kitakyushu, and Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.; Department of Dermatology, Graduate School of Medical Sciences, Kyushu, University, Fukuoka, Japan.
authors.forename: Masutaka
authors.initials: M
authors.lastname: Furue
mesh_terms: D000368:Aged; D000527:Alprostadil / Q000008:administration & dosage; D044466:Asian People; D017809:Fatal Outcome; D005260:Female; D005343:Fibrinolytic Agents / Q000008:administration & dosage; D006801:Humans; D007564:Japan; D009102:Multiple Organ Failure / Q000209:etiology; D011006:Pneumatosis Cystoides Intestinalis / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D011859:Radiography; D012595:Scleroderma, Systemic / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology
chemical_list: D005343:Fibrinolytic Agents; D000527:Alprostadil