A Newborn With Cutaneous Lesions and Hematochezia

Salvatore E. Mignano, DO; Hemant Pal, MD


June 12, 2017

Multifocal Lymphangioendotheliomatosis With Thrombocytopenia

On the 13th day of life, esophagogastroduodenoscopy (EGD) was done to investigate the patient's hematochezia. EGD showed red-violet papular lesions with streaks of old blood in the stomach (Figure 2).

Figure 2. Esophagogastroduodenoscopy demonstrating lesions similar to the ones seen on the patient's skin. Multiple small, red-violet, ovoid maculopapular lesions are seen in the gastric mucosa in a background of streaky old blood. No ulceration or active bleeding was seen.

No biopsy samples of the gastrointestinal lesions were taken to avoid further bleeding. Instead, punch biopsies of the left knee papular lesions were done. The biopsies showed dermally based dilated vessels filled with intraluminal papillary projections, comprising bland, hobnailed endothelial cells. No cellular atypia was noted. These findings were correlated with the clinical data, and MLT was diagnosed (Figure 3a and 3b).

Figure 3a. Lesions in multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT), which present in the dermis. The lesions comprise dilated vessels filled with complex, intraluminal papillary projections. b. High-power view of the vascular spaces in MLT, revealing that the intraluminal projections are cytologically bland, with minimal atypia.


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