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June 2010

Pale Macules, Crusted Papules, and Breathlessness in a Child—Quiz Case

Author Affiliations


Arch Dermatol. 2010;146(6):667-672. doi:10.1001/archdermatol.2010.118-a

A 15-month-old girl was referred for an asymptomatic eruption noted during her admission and failure to thrive and respiratory distress. Clinical examination revealed widespread, macular, confetti-like hypopigmentation affecting the trunk and extremities. A congenital melanocytic nevus was noted on the lower back, below which lay a cluster of scaly, erythematous, crusted papules (Figure 1). Further questioning revealed that a widespread, vesicopustular eruption had been noted at birth, clearing over a 2-month period to leave pale macules. Baseline blood test results were unremarkable. Chest radiography and high resolution computed tomography revealed diffuse multicystic lung disease. Immunohistochemical analysis of bronchioalveolar lavage aspirate revealed numerous (>40%) macrophages expressing CD1a. A punch biopsy specimen was obtained from one of the crusted papules on the lower back (Figure 2 and Figure 3).

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