A four-day old Hispanic female was transferred to our
institution from an outside hospital for management
of hemolytic anemia and a rash on her abdomen. She
was born at 35 weeks to a 33-year-old G4P4 (pregnant four
times, gave birth four times) mother by urgent C-section for
preterm labor. The baby was noted to be jaundiced at birth with
an elevated direct bilirubin of 16. She additionally had thrombocytopenia
and elevated liver function enzymes. A positive
direct Coombs test and maternal antibody screen confirmed
erythroblastosis fetalis (or hemolytic disease of the newborn)
as the cause of her hemolytic anemia. Quadruple phototherapy
was initiated on the baby’s first day of life.
The patient received intravenous immunoglobulin on days 1 and
2 of life for a total of three doses. In addition, she was given a
red blood cell transfusion for anemia via umbilical catheter, receiving
furosemide before and after transfusion. On day 4 of life,
an erythematous eruption was noted on the patient’s abdomen.
The umbilical catheter was removed due to concern for infection,
antibiotics were initiated, and the patient was transferred to our
facility for management of a suspected infection. At our institution,
phototherapy was discontinued and the patient was given
an exchange transfusion. Dermatology was consulted.
On exam, the patient had a well-demarcated violaceous and
purpuric non-blanching patch on her abdomen and chest with
minimal involvement of her extremities (Figure 1). The rash was
accentuated on the central abdomen and chest corresponding to
the areas most directly exposed to phototherapy lights. There was
distinct sparing of sites previously covered by the umbilical catheter
and cardiac monitoring electrodes. There were no vesicles or
bullae present. A skin biopsy from the abdomen was performed.
Histopathology revealed extravasated erythrocytes in the
superficial dermis with no interface reaction or spongiosis
of the epidermis. There was no necrosis, vasculitis or vesiculation
(Figure 2).
Urine and plasma porphyrin levels revealed elevated plasma
coproporphyrin and protoporphyrin and elevated urine uroporphyrin
and coproporphyrin. Fecal coproporphyrin was within
reference range.
The patient’s hemolytic anemia was treated with exchange
transfusion with no phototherapy. The rash resolved over
the course of one week with supportive care and reduced ex-
posure to artificial light. There was no residual scar or milia
(Figure 3). Urine and plasma porphyrin panels were drawn
several weeks later, demonstrating slow resolution of the porphyrinemia
and porphyrinuria.
Our diagnosis in this case was transient porphyrinemia of the
newborn. Paller et al. reported a series of six similar cases in
transfused neonates receiving phototherapy.1 Five of these patients
had erythroblastosis fetalis. Porphyrins were measured
in two neonates. Plasma coproporphyrins were increased both
neonates, plasma protoporphyrins were elevated in one neonate,
and urine porphyrin levels were normal. In another case
of transient porphyrinemia in a patient with erythroblastosis
fetalis receiving phototherapy, plasma and erythrocyte porphyrins
were elevated while urine and fecal porphyrins were in the
