Acute abdomen due to anaphylactic intestinal edema associated with systematic mastocytosis: a case report

Abstract

Background: Among various anaphylactic conditions resulting in acute abdomen, mast cell activation disorders, although rare, are included in the differential diagnosis.

Case presentation: This report describes a 63-year-old Caucasian man who was brought to the emergency room with sudden onset abdominal pain, vomiting, and diarrhea, with breathing difficulty, and with facial swelling after quarrelling with an acquaintance. Computed tomography showed edematous and swollen intestines, consistent with splenomegaly. Physical findings included maculopapular cutaneous mastocytosis. He also had a long history of repeated episodes of anaphylaxis requiring occasional epinephrine auto-injector administration; however, the precise cause of anaphylaxis was previously undetermined. Blood tests showed high serum concentrations of soluble IL-2R and tryptase, suggesting mast cell-related disease. Subsequent biopsies of his bone marrow and cutaneous rash confirmed the diagnosis of systemic mastocytosis (SM).

Conclusion: SM was diagnosed in a patient with acute abdomen who visited the emergency room.

Keywords: Acute abdomen; Anaphylaxis; Intestinal edema; Systemic mastocytosis.

Fig. 1

Abdominal CT of the patient showing A an edematous and swollen small intestine (arrows), B splenomegaly, and C maculopapular cutaneous mastocytosis on the back. D Bone density CT showing osteosclerosis of the pelvic bones, with partial punched out osteopenia of the spine (arrowheads)

Fig. 2

Bone marrow biopsy specimen showing marked infiltration by mast cells. A May-Giemsa stain, B CD117 stain, C CD25 stain, and D CD2 stain (original magnification, × 100)

Fig. 3

Biopsy specimen of a cutaneous rash on the abdomen, showing infiltration of the dermis by mast cells. A Hematoxylin and eosin stain, B CD117 stain, C CD25 stain, and D CD2 stain (original magnification, × 100)