Pneumatosis cystoides intestinalis with systemic sclerosis, limited type resulting in a poor prognosis.
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.
Hamada
Manabu
M
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.
Kayashima
Miki
M
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.
Morai
Yoichi
Y
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.
Masuda
Teiichi Chi
TC
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.
Urabe
Kazunori
K
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.
Furue
Masutaka
M
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.
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
D005343:Fibrinolytic Agents; D000527:Alprostadil