An adult patient with Kabuki syndrome presenting with Henoch-Schönlein purpura complicated with pulmonary hemorrhage.
We present a case of a 33-year-old woman with Kabuki syndrome (KS) presenting with Henoch-Schönlein purpura (HSP). She was admitted to our hospital with a brain abscess in the lateral ventricle and meningitis. She had been diagnosed with KS. Skin eruptions had appeared on her lower extremities, with arthralgia, cough, and hemoptysis. She suddenly developed pulmonary hemorrhage and respiratory failure. We intubated her trachea and started mechanical ventilation in the intensive care unit (ICU). Skin biopsy revealed leukocytoclastic vasculitis with granular depositions of immunoglobulin A (IgA) in dermal vessel walls, and she was diagnosed as having HSP. Supportive management and prednisolone at 20 mg x day(-1) cured the pulmonary hemorrhage and respiratory failure. On ICU day 27, she was weaned from mechanical ventilation. Pulmonary hemorrhage as a complication of HSP is rare and sometimes fatal. KS is often associated with an increased incidence of infection and congenital heart disease. Susceptibility to infection and pulmonary hypertension due to congenital heart disease in this patient may have led to the development of the pulmonary hemorrhage. Supportive care and steroid therapy appeared to be beneficial in the treatment of this patient with HSP with pulmonary hemorrhage.
Oto
Jun
J
Department of Emergency and Critical Care Medicine, Tokushima University Hospital, 3-18-15 Kuramoto-cho, Tokushima, 770-8503, Japan.
Mano
Akiko
A
Nakataki
Emiko
E
Yamaguchi
Harutaka
H
Inui
Daisuke
D
Imanaka
Hideaki
H
Nishimura
Masaji
M
D000015:Abnormalities, Multiple / Q000473:pathology*; D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001922:Brain Abscess / Q000150:complications / Q000188:drug therapy; D002097:C-Reactive Protein / Q000378:metabolism; D005260:Female; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D011695:IgA Vasculitis / Q000473:pathology*; D007070:Immunoglobulin A / Q000378:metabolism; D008171:Lung Diseases / Q000209:etiology*; D011239:Prednisolone / Q000627:therapeutic use; D012121:Respiration, Artificial; D012129:Respiratory Function Tests; D012131:Respiratory Insufficiency / Q000209:etiology; D012867:Skin / Q000473:pathology; D013577:Syndrome; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications / Q000473:pathology
D000900:Anti-Bacterial Agents; D000893:Anti-Inflammatory Agents; D007070:Immunoglobulin A; D002097:C-Reactive Protein; D011239:Prednisolone