PMC8039294_01
(A, B) Papulo-pustular lesions on an erythematous base on the feet. (C) Scattered papules with central vesicles on the body in a newborn with chronic granulomatous disease.
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After uneventful pregnancy, a full-term neonate presented at birth with extensive papulo-pustular lesions on both hands and feet and scattered papules with central vesicles on the body ( Figure 1 ).
(A) Noncontrast CT (80kV, 48mAs, FOV 170x130mm) with coronal reconstruction using a soft tissue kernel shows a distinct bilateral axillary lymphadenopathy (arrows) and a prominent inhomogeneous thymus. (B) Sagittal T2 STIR sequence of a whole body MRI (TE 33ms, TR 3800ms, FOV 300x300mm, matrix 256x256px) shows bulky hyperintense soft tissue masses surrounding the aortocaval and mesenteric vasculature (solid arrow). The enlarged thymus features multiple jagged-edged cystic lesions (dotted arrow).
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Imaging studies revealed an enlarged thymus with multiple jagged-edged cysts ( Figure 2 ), and axillary lymph nodes as well as those located along the lateral thoracic wall, parailiacal and inguinal were also increased in size with a maximum diameter of 1.8 cm. Magnetic resonance imaging showed bulky soft tissue masses surrounding the abdominal aorta and its branches from the coeliac trunk to the external iliac artery ( Figure 2 ).
(A) H+E, 4x, Lymph node with severely disturbed architecture by a diffuse necrotizing and granulomatous inflammation. (B) H+E, 4x, Lymph node, close-up of the granulomatous inflammation with abundant multinucleated giant cells (see arrows) in a background of neutrophils and eosinophils.
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No atypical mycobacteria were detected, and CD1a and langerin expression were absent ( Figure 3 ).