September 1975 The Journal of P E D I A T R I C S
443
Simultaneous vasculitis in a mother and newborn infant John J. Miller Ill, M.D., Ph.D., and James F. Fries, M.D.,
P E R I P H E R A L GANGRENE in the n e w b o r n i n f a n t is unusual, a l t h o u g h it has m a n y potential causes including sepsis, dehydration, p r i m a r y a n d secondary effects o f trauma, e m b o l i z a t i o n f r o m catheters, a n d vascUlitis. 1, 2 In a n i n f a n t with n e o n a t a l g a n g r e n e seen at Stanford, the basic pathologic process b e c a m e clear w h e n vasculitis was recognized in the mother. CASE REPORT
A 28-year-old gravida 3, para 2, Caucasian woman developed fever, nausea, and muscle pain at four and one-half months of gestation. The symptoms waned but were followed by arthi-itis in the wrists, the distal interphalangeal joints of her hands, and the joints of both thumbs. She was given a combination of aspirin and meprobamate (Equagesic, Wyeth Laboratories, Philadelphia, Pa.) to take daily until delivery. After a normal labor and parturition, painful nodules gradually appeared on her shoulders, arms, buttocks, and thighs, each of which lasted for one to two weeks. Three months postpartum a biopsy of skin overlying a nodule showed a perivascular infiltrate (Fig. 1). Immunologic studies done at this time are presented in Table I. The recurrent nodules and arthralgias resolved spontaneously six months postpartum. Her male infant weighed 3,320 gm. and appeared well at birth. Livido reticularis and extreme acrocyanosis were seen at Day 5. By eight days the central portion of the upper lip had become deep blue. At ten days of age an electrocardiogram was normal. The white blood cell count was 20,000/ram 3 with 36% polymorphonuclear cells, 40% band forms, 27% lymphocytes, 16% monocytes, and 15% eosinophils. At age 13 days the fingers and upper lip became black (Fig. 2). The physical examination was normal except for the gangrenous areas, generalized livido reticularis, and cyanotic feet. Depressed ST segments were noted in an electrocardiogram in precordial leads. White blood count was 45,000/mm 3 with 43% polymorphonuclear cells, 25% lympho-
From the Children's Hospital at Stanford, Department of Pediatrics, and The Department of Medicine, Stanford University School of Medicine.
Stanford, Calif.
Table I. I m m u n o l o g i c studies in the m o t h e r a n d child
Mother Serum concentration (mg/dl) IgG 1,200 (8001,800)* IgA 125 (90-450) IgM 91 (70-280) C3 87 (80-120) Serum titers Direct Coombs 0 VDRL 0 Cryoglobulins Trace Antinuclear antibodies 0 Anti-DNA Hemagglutinins 0 Precipitins Trace Free DNA 0 Anti-ENA 0 Latex fixation 0 Lupu s erythematosus prep 0 Rubella antibody < V8 Australia antigen < % Skin test PPD 0
Child (2 wk of age)
720 (6001,200" 53 (3-20) 78 (15-60) 137 (80-120)
0
443
Simultaneous vasculitis in a mother and newborn infant John J. Miller Ill, M.D., Ph.D., and James F. Fries, M.D.,
P E R I P H E R A L GANGRENE in the n e w b o r n i n f a n t is unusual, a l t h o u g h it has m a n y potential causes including sepsis, dehydration, p r i m a r y a n d secondary effects o f trauma, e m b o l i z a t i o n f r o m catheters, a n d vascUlitis. 1, 2 In a n i n f a n t with n e o n a t a l g a n g r e n e seen at Stanford, the basic pathologic process b e c a m e clear w h e n vasculitis was recognized in the mother. CASE REPORT
A 28-year-old gravida 3, para 2, Caucasian woman developed fever, nausea, and muscle pain at four and one-half months of gestation. The symptoms waned but were followed by arthi-itis in the wrists, the distal interphalangeal joints of her hands, and the joints of both thumbs. She was given a combination of aspirin and meprobamate (Equagesic, Wyeth Laboratories, Philadelphia, Pa.) to take daily until delivery. After a normal labor and parturition, painful nodules gradually appeared on her shoulders, arms, buttocks, and thighs, each of which lasted for one to two weeks. Three months postpartum a biopsy of skin overlying a nodule showed a perivascular infiltrate (Fig. 1). Immunologic studies done at this time are presented in Table I. The recurrent nodules and arthralgias resolved spontaneously six months postpartum. Her male infant weighed 3,320 gm. and appeared well at birth. Livido reticularis and extreme acrocyanosis were seen at Day 5. By eight days the central portion of the upper lip had become deep blue. At ten days of age an electrocardiogram was normal. The white blood cell count was 20,000/ram 3 with 36% polymorphonuclear cells, 40% band forms, 27% lymphocytes, 16% monocytes, and 15% eosinophils. At age 13 days the fingers and upper lip became black (Fig. 2). The physical examination was normal except for the gangrenous areas, generalized livido reticularis, and cyanotic feet. Depressed ST segments were noted in an electrocardiogram in precordial leads. White blood count was 45,000/mm 3 with 43% polymorphonuclear cells, 25% lympho-
From the Children's Hospital at Stanford, Department of Pediatrics, and The Department of Medicine, Stanford University School of Medicine.
Stanford, Calif.
Table I. I m m u n o l o g i c studies in the m o t h e r a n d child
Mother Serum concentration (mg/dl) IgG 1,200 (8001,800)* IgA 125 (90-450) IgM 91 (70-280) C3 87 (80-120) Serum titers Direct Coombs 0 VDRL 0 Cryoglobulins Trace Antinuclear antibodies 0 Anti-DNA Hemagglutinins 0 Precipitins Trace Free DNA 0 Anti-ENA 0 Latex fixation 0 Lupu s erythematosus prep 0 Rubella antibody < V8 Australia antigen < % Skin test PPD 0
Child (2 wk of age)
720 (6001,200" 53 (3-20) 78 (15-60) 137 (80-120)
0
