Nijmegen breakage syndrome (NBS) is a chromosomal breakage disorder with characteristic physical features, chromosomal instability, and combined immunodeficiency. It is closely related to other chromosomal breakage disorders like ataxia telangiectasia. Noninfectious granulomatous inflammation refractory to treatment is a relatively common feature in ataxia telangiectasia. Herein we report a patient with NBS who developed chronic refractory necrotizing granulomatous ulcerations and review the pathophysiology of NBS and noninfectious granulomas in primary immunodeficiency syndromes.

Cutaneous lymphadenoma is known to occur over a broad age range, from 14 to 72 years of age. We report the unique clinical and histologic presentation of a cutaneous lymphadenoma from the suprapubic abdomen of a neonate which may represent a novel subtype, glandular congenital lymphadenoma. Cutaneous lymphadenoma is a rare tumor with a distinct histologic triad of epithelial nodules, dense fibrous stroma, and intense intranodular lymphocytic infiltrate. Typically, it is a slow growing, skin colored papule, nodule, or plaque, clinically resembling a basal cell carcinoma and often occurring in the head and neck region or lower extremities.