What's Your Diagnosis?

A slightly premature newborn with an unusual-appearing arm

A 36-week gestation male newborn is delivered by emergency cesarean section because of decreased fetal movements. Pregnancy was also complicated by the mother having a positive group B streptococcal screen and taking Zoloft for depression.

James H. Brien
Figures 1 to 3: Left arm having a necrotic appearance from the mid-forearm to the hand, including some of the fingers.

Source: James H. Brien, DO

The mother, who is healthy with one other child, was given a dose of IV penicillin, but there was no time for a second dose, as recommended by protocol. Therefore, a blood culture was obtained on the baby, and empiric treatment was begun with ampicillin plus gentamicin. There were no other prenatal complications. The baby was vigorous with Apgar scores of 8 and 9. However, upon delivery, it was noted that the baby’s left arm looked unusual.

Figures 4 and 5: Appearance of the area of involvement did not progress over 48 hours.

Except for his left arm and hand, his examination revealed a perfectly normal-appearing, near-term baby, who weighed 2.6 kg, with normal vital signs at the birth hospital. Because of the unusual appearance of his left arm, he was transferred to the children’s hospital, where he was noted to have a fever (temperature of 101.5°F) upon arrival, with all normal vital signs thereafter. His exam was confirmed to be positive only for the left arm having a necrotic appearance from the mid-forearm to the hand, including some of the fingers, with dark, purplish skin with an irregular, sharp line of demarcation (Figures 1 to 3). As noted, there appeared to be some redundant skin with the appearance of an empty or flaccid bullous lesion, along with some necrotic ulcerations and erosion on the anterior aspect.

Lab tests include the blood culture noted, plus another blood culture upon arrival with the fever. These cultures were negative, as well as a negative Gram stain and culture of some fluid aspirated from under the “blister” skin. A complete blood count (CBC), metabolic profile and coagulation studies were normal. A vascular ultrasound study of the left arm was also normal, and the appearance of the area of involvement did not progress over the next 48 hours (Figures 4 and 5).

What’s your diagnosis?

A. Zoloft (sertraline, Pfizer) toxicity

B. In utero vascular “accident”

C. Sepsis with disseminated intravascular coagulation (DIC)

D. Epidermolysis bullosa (EB)

Case Discussion

Although absolute proof was lacking, we believed the answer to be B, a vascular clot or compression in utero. Even though the baby had a single fever noted, the blood cultures were negative, and he was normal in all other respects. Additionally, DIC was not supported by lab results, and the area of necrosis never progressed as one would expect in sepsis with DIC (Figure 6, a child with group A streptococcal sepsis). Certain immunosuppressed patients can develop necrotic lesions called ecthyma gangrenosum (Figure 7, a patient with cancer), caused by septic emboli, usually due to Pseudomonas aeruginosa sepsis.

After a few days of observation for changes, the antimicrobial agents were discontinued. In retrospect, the fever noted on arrival was likely due to overwarming in transport.

Figure 6: Child with group A streptococcal sepsis.
Figure 7: Ecthyma gangrenosum in a patient with cancer.

Over the next 6 weeks of follow-up on anticoagulation therapy with Lovenox (enoxaparin, Sanofi-Aventis), the arm healed and returned to a normal appearance. Function was never affected. With a normal follow-up vascular study, the anticoagulation therapy was discontinued, and with 11 months of follow-up, no other problems have developed.

Sertraline has not been associated with teratogenic effects or coagulation abnormalities. However, it does raise the question.

Lastly, EB is a hereditary spectrum of disorders that affects the ability of the dermis and epidermis to remain “anchored.” The more severe form may be suspected at birth because of the immediate blistering damage that occurs as a result of birth trauma. Ongoing injury with blistering would continue with handling, making EB very unlikely in this case. DermNetNZ.org is an excellent online site for reading a review of EB, written by Vanessa Ngan, which includes several good pictures.

Disclosure: Brien reports no relevant financial disclosures.

A 36-week gestation male newborn is delivered by emergency cesarean section because of decreased fetal movements. Pregnancy was also complicated by the mother having a positive group B streptococcal screen and taking Zoloft for depression.

James H. Brien
Figures 1 to 3: Left arm having a necrotic appearance from the mid-forearm to the hand, including some of the fingers.

Source: James H. Brien, DO

The mother, who is healthy with one other child, was given a dose of IV penicillin, but there was no time for a second dose, as recommended by protocol. Therefore, a blood culture was obtained on the baby, and empiric treatment was begun with ampicillin plus gentamicin. There were no other prenatal complications. The baby was vigorous with Apgar scores of 8 and 9. However, upon delivery, it was noted that the baby’s left arm looked unusual.

Figures 4 and 5: Appearance of the area of involvement did not progress over 48 hours.

Except for his left arm and hand, his examination revealed a perfectly normal-appearing, near-term baby, who weighed 2.6 kg, with normal vital signs at the birth hospital. Because of the unusual appearance of his left arm, he was transferred to the children’s hospital, where he was noted to have a fever (temperature of 101.5°F) upon arrival, with all normal vital signs thereafter. His exam was confirmed to be positive only for the left arm having a necrotic appearance from the mid-forearm to the hand, including some of the fingers, with dark, purplish skin with an irregular, sharp line of demarcation (Figures 1 to 3). As noted, there appeared to be some redundant skin with the appearance of an empty or flaccid bullous lesion, along with some necrotic ulcerations and erosion on the anterior aspect.

Lab tests include the blood culture noted, plus another blood culture upon arrival with the fever. These cultures were negative, as well as a negative Gram stain and culture of some fluid aspirated from under the “blister” skin. A complete blood count (CBC), metabolic profile and coagulation studies were normal. A vascular ultrasound study of the left arm was also normal, and the appearance of the area of involvement did not progress over the next 48 hours (Figures 4 and 5).

What’s your diagnosis?

A. Zoloft (sertraline, Pfizer) toxicity

B. In utero vascular “accident”

C. Sepsis with disseminated intravascular coagulation (DIC)

D. Epidermolysis bullosa (EB)

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Case Discussion

Although absolute proof was lacking, we believed the answer to be B, a vascular clot or compression in utero. Even though the baby had a single fever noted, the blood cultures were negative, and he was normal in all other respects. Additionally, DIC was not supported by lab results, and the area of necrosis never progressed as one would expect in sepsis with DIC (Figure 6, a child with group A streptococcal sepsis). Certain immunosuppressed patients can develop necrotic lesions called ecthyma gangrenosum (Figure 7, a patient with cancer), caused by septic emboli, usually due to Pseudomonas aeruginosa sepsis.

After a few days of observation for changes, the antimicrobial agents were discontinued. In retrospect, the fever noted on arrival was likely due to overwarming in transport.

Figure 6: Child with group A streptococcal sepsis.
Figure 7: Ecthyma gangrenosum in a patient with cancer.

Over the next 6 weeks of follow-up on anticoagulation therapy with Lovenox (enoxaparin, Sanofi-Aventis), the arm healed and returned to a normal appearance. Function was never affected. With a normal follow-up vascular study, the anticoagulation therapy was discontinued, and with 11 months of follow-up, no other problems have developed.

Sertraline has not been associated with teratogenic effects or coagulation abnormalities. However, it does raise the question.

Lastly, EB is a hereditary spectrum of disorders that affects the ability of the dermis and epidermis to remain “anchored.” The more severe form may be suspected at birth because of the immediate blistering damage that occurs as a result of birth trauma. Ongoing injury with blistering would continue with handling, making EB very unlikely in this case. DermNetNZ.org is an excellent online site for reading a review of EB, written by Vanessa Ngan, which includes several good pictures.

Disclosure: Brien reports no relevant financial disclosures.