Mastocytosis in pregnancy.
[cutaneous mastocytosis]
Mastocytosis is a rare disorder characterized by abnormal accumulation of mast cells in various organs. Clinical complaints include pruritus, cutaneous flushing, dyspepsia, and episodes of anaphylaxis, and are usually the result of local and systemic mast cell mediator release. The triggers include a variety of factors including drugs, exercise, stress, anxiety, and temperature extremes.A 26-year-old primigravida at 40 weeks' gestation with urticaria pigmentosa presented to our hospital. She was diagnosed with cutaneous mastocytosis based on pathologic examination of her skin biopsy. There were no complications during pregnancy, except for cutaneous manifestations with pruritus and premature uterine contractions at 27 weeks' gestation. After admission, antihistamine agents were administered during labor to treat the above symptoms, and antibiotic agents were given for prophylaxis of chorioamnionitis. Labor pain was successfully managed with warm showers, frequent position changes and massage, and therefore, epidural analgesia was not carried out. After 6 hours of labor, the patient gave birth to a healthy female infant via normal spontaneous vaginal delivery with right mediolateral episiotomy. Neither local anesthetic agents nor antibiotic agents caused any reaction. The postpartum period was uneventful.Pregnant women with mastocytosis should be treated symptomatically and should avoid factors that may exacerbate symptoms of disease. Clinicians should be aware of preterm labor during pregnancy. As a preventive measure, resuscitation equipment should be available during the labor, delivery and postpartum period to treat unanticipated hypotension and shock.
