Neonatal Behçet’s disease with Raynaud phenomenon

1 Case report

A male newborn of 3340 grams was vaginally delivered at 37 weeks of gestation, Apgar 9 at 1 min and 10 at 5 min, from a 21 years old secundipara mother diagnosed with Behçet disease 5 years before. She had active disease in pregnancy, with orogenital ulcerations in the first trimester, so she received treatment with colchicine plus oral prednisone for 2 weeks. At 30 weeks of gestation age, she was diagnosed with a facial herpes virus infection, recovering fully following administration of topical antiviral treatment.

Polyhydramnios was detected in routine ultrasound examinations, with no associated fetal anomalies. There were no other infection risk factors during pregnancy or delivery. Usual pregnancy serological tests, including Treponema pallidum, Rubella, Toxoplasma, Hepatitis B Virus and Human Immunodeficiency Virus (HIV) were all negative, as was Group B Streptococcus carrier screening.

At 24 hours of life, aphthous lesions appeared in the mucosa of the inferior lip and jugal mucosa (Figs. 1 and 2). The infant was in good condition, with neither respiratory distress nor hemodynamic instability and without fever or other symptoms of infection. 12 hours after his admission to the neonatal unit, vasomotor phenomena were detected in both hands, with acrocyanosis preceding a pale phase with irritability and crying, interpreted as a Raynaud’s phenomenon. He also developed located papular lesions following venous punctures (positive pathergytest).

Laboratory tests were performed, including a hemogram with normal results: Hb 19.9 g/dl, leucocytes 9140 mm3; neutrophils 51.9%, lymphocytes 31.1%, platelet count 236000 mm3. Glucose, electrolytes and renal function were in normal range, except for a mild increase in the total bilirubin level (11.4 mg/dl) with mild clinical jaundice that did not require therapy.

Microbiological testing was performed, with blood testing for HIV-1/HIV-2 (negative), Herpesvirus-1 (Ig G+), Herpesvirus-2 (negative), Cytomegalovirus (negative) and Varicella zoster virus (Ig G+); microbiological culture of mucosal lesions was negative. An ophthalmological assessment was performed showing no ocular involvement and a cerebral ultrasound ruled out associated intracranial lesions.

Once infectious causes were ruled out, mainly herpes and staphylococcus infection, oral prednisolone treatment was started (1 mg/kg/day for 5 days) in addition to symptomatic treatment with an analgesic collutory. The mucosal lesions and Raynaud’s phenomenon improved and the patient recovered fully, being discharged at 10 days of life. During follow-up (6 months) he showed no new cutaneous or mucosal manifestations.

2 Discussion

Behçet disease is an uncommon autoimmune disease (6.4 in 100,000 persons) [1]. There are no specific laboratory tests for diagnosis, so clinical diagnosis must follow the criteria of the International Study Group of Behçet Disease (recurrent oral ulceration plus two of the following: recurrent genital ulceration, eye lesions, skin lesions or positive pathergy test) [2].

An increase in outbreaks of the disease during pregnancy has been described, with an increased risk of fetal intrauterine growth restriction. No increased rate of spontaneous miscarriage, congenital anomalies or stillbirth has been described in pregnant women with Behçet disease.

Most infants born to mothers with Behçet disease remain asymptomatic. There are very few symptomatic neonatal cases reported in the literature, with mainly oral or genital lesions and other skin manifestations [2, 3].

Neonatal Behçet disease is usually transient with symptoms resolving spontaneously at 8 weeks of life, suggesting a transplacental passage of maternal antibodies as observed in neonatal lupus disease or neonatal myasthenia gravis. This hypothesis has been suggested in previous reports, by the finding of increased levels of immunoglobulins G and M, in some cases with severe ulcerations, but evidence for this assumption is still lacking.

Two cases with elevated morbidity have been described in the literature. The first case was a newborn with serious compromised airways secondary to oral scarring lesions, requiring mechanical ventilation. In this case, the outcome was good after 8 weeks and patient recovered fully withoutsequelae [4].

The second infant developed serious neurological symptoms with neonatal seizures secondary to disseminated ischemic and hemorrhagic lesions; this infant eventually died [5]. Potential life-threatening symptoms of neonatal Behçet disease (2 cases with severe respiratory or neurological manifestations) must be kept in mind by clinicians attending to infants born to mothers with Behçet disease. In some cases, it may be necessary to use a short course of systemic steroid therapy (prednisolone), always after other infectious causes have been excluded. However, evidence supporting these recommendations is still lacking due to the rarity of this disease, so the need for treatment must be considered by clinicians individually.

Disclosure statements

Parental permission for clinical case and photographs was obtained for purposes of publication in medical journals.

The authors have no financial relationships relevant to this article neither other conflicts of interest to disclose.