Photo Challenge

Scattered Red-Brown, Centrally Violaceous, Blanching Papules on an Infant

Author and Disclosure Information

A 2-week-old infant girl was transferred to a specialty pediatric hospital where dermatology was consulted for evaluation of a diffuse eruption triggered by cold that was similar to an eruption present at birth. She was born at 31 weeks and 2 days’ gestation at an outside hospital via caesarean delivery. Early delivery was prompted by superimposed pre-eclampsia with severe hypertension after administration of antenatal steroids. At birth, the infant was cyanotic and apneic and had a documented skin eruption, according to the medical record. She had thrombocytopenia, elevated C-reactive protein, and an elevated temperature without fever. Extensive septic workup, including blood, urine, and cerebrospinal fluid cultures; herpes simplex virus and cytomegalovirus screening; and Toxoplasma polymerase chain reaction were negative. Magnetic resonance imaging of the brain revealed no evidence of intracranial congenital infection. Ampicillinsulbactam was initiated for presumed culture-negative sepsis. On day 2 of hospitalization, she developed conjunctival icterus, hepatomegaly, and jaundice. Direct hyperbilirubinemia; anemia; and elevated triglycerides, ferritin, and ammonia all were present. Coagulation studies were normal. Subsequent workup, including abdominal ultrasonography and hepatobiliary iminodiacetic acid scan, was concerning for biliary atresia. Despite appropriate treatment, her condition did not improve and she was transferred. Repeat abdominal ultrasonography on day 24 of life confirmed hepatomegaly but did not demonstrate other findings of biliary atresia. At the current presentation, physical examination revealed many scattered, redbrown and centrally violaceous, blanching papules measuring a few millimeters involving the trunk, arms, buttocks, and legs. A punch biopsy was obtained.

Scattered red-brown, centrally violaceous, blanching papules on an infant

What's your diagnosis?

chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome

familial cold autoinflammatory syndrome (FCAS)

Muckle-Wells syndrome (MWS)

neonatal-onset multisystem inflammatory disorder (NOMID)

tumor necrosis factor receptor–associated periodic fever (TRAPS)

Next Article:

A 7-month-old male presents with pustules and inflamed papules on the scalp and extremities

Related Articles

  • Photo Challenge

    Retiform Purpura on the Lower Legs

    A 58-year-old man presented with a petechial and purpuric rash limited to the lower extremities. He reported that the rash had been present for...

  • Photo Challenge

    Symmetric Palmoplantar Papules With a Keratotic Border

    A 67-year-old man presented to our office with a rash on the hands, feet, and periungual skin that began with wartlike growths many years prior...