Shock in an Infant with Bullous Mastocytosis

Abstract

A 6-month-old infant had bullous lesions on his posterior neck, upper trunk, and extremities for two months prior to admission for fever and shock. He had an elevated white blood cell count with left shift and normal platelet count, but abnormal coagulation studies. He was treated with intravenous antibiotics, crystalloids, fresh-frozen plasma, and pressor agents. A histamine H2 receptor antagonist was started for guaiac-positive nasogastric tube drainage. The patient recovered after four days of treatment. A skin biopsy confirmed mastocytosis. A week later the child passed grossly bloody stools with blood clots. No source of gastrointestinal bleeding was identified by extensive work-up. Blood histamine level measured one day before gastrointestinal bleeding was 16,400 pg/ml (normal 263 +/- 202 pg/ml). The bleeding resolved spontaneously. The patient was maintained on cimetidine. Results of a subsequent bone scan were normal. Shock or gastrointestinal bleeding associated with unusual skin lesions should alert the pediatrician to the possibility of mastocytosis.

Recurring Staphylococcal Scalded Skin Syndrome‐like Bullous Mastocytosis: The Utility of Cytodiagnosis and the Rapid Regression with Steroids

“…Systemic involvement is more frequent in this variant than in others and some lethal outcomes have been reported (6). During extensive skin blistering, complications like shock (6,7), ulceration, systemic infection, and metabolic imbalance due to fluid loss may be noted (8). Orkin et al (5) described two subsets of bullous mastocytosis: the neonatal form with a poor prognosis due to a high risk for extracutaneous involvement, severe complications, and early death; and the form with delayed onset, having a good prognosis and no significant extracutaneous involvement.…”

Diffuse Cutaneous Mastocytosis Mimicking Staphylococcal Scalded‐Skin Syndrome: Report of Three Cases

“…In three children with DCM and extensive blistering the disease was misinterpreted as SSSS. Extensive neonatal blistering has been reported sporadically in DCM (6,7). When blisters are present, the Tzanck smear can provide important information by revealing mast cells (8).…”

Diffuse cutaneous mastocytosis with novel somatic KIT mutation K509I and association with tuberous sclerosis

“…As most cutaneous mastocytosis cases are not associated with significant morbidity or mortality, it is under‐recognized that the DCM type of cutaneous mastocytosis is associated with fatal complications . DCM is characterized by diffuse dense infiltrates of MCs in the dermis, and this high MC burden can result in significant amounts of MC mediator release leading to systemic reactions that can be severe and even fatal . Given the risk for life‐threatening complications, it is important to consider DCM in pediatric patients with a concerning skin examination (skin with “peau d'orange” appearance, hyper‐pigmentation, positive Darier's sign).…”

“…1,4 DCM is characterized by diffuse dense infiltrates of MCs in the dermis, and this high MC burden can result in significant amounts of MC mediator release leading to systemic reactions that can be severe and even fatal. [5][6][7][8] Given the risk for life-threatening complications, it is important to consider | 1835 OTANI eT Al. DCM in pediatric patients with a concerning skin examination (skin with "peau d'orange" appearance, hyper-pigmentation, positive Darier's sign).…”