PO-05. Severe urticaria pigmentosa and osteoblastoma in a child: A case report

Summary

Introduction

Mastocytosis is a clonal disease of the mast cells. Frequent symptoms include rash and pruritus with some patients describing flushes. Here we present a case of severe urticaria pigmentosa and osteoblastoma in a child.

Clinical description

The boy was born following a full-term pregnancy. Just following the delivery, the urticarial rash with blistering was noted (Fig. 1). After birth, histamine crisis happened regularly once to twice a day and usually presented with sudden skin erythema, severe pruritus, blistering (Fig. 2.), abdominal pain, extreme excitation with crying up occasionally followed by fainting. Its duration varied from several minutes to 1 hour. There was a single episode of respiratory arrest lasting for 10-15 seconds. The episodes were spontaneous and sometimes were triggered by warm bath, agitated state and eating. Mastocytosis was diagnosed histologically by identifying excess of mast cells in dermal layer, mutation of C-KIT D816V, elevation of mast cell tryptase up to 17.3 mcg/l (normal upper limit, 11.4). The child received antihistamines, and short courses of betamethasone therapy. Six months later, there was a continuous alleviation of the symptoms: flushing and abdominal pain became less frequent, pruritus and rash became milder along with reduced medication requirements. At 2 years and 8 months, pain and dismobility were noted in the left shoulder joint. When the child was 4.5 y.o., osteoblastoma was diagnosed. The tumor was removed. Now the boy is 10 y.o. He attends school. There is still mild pruritus and residual skin pigmentation (Fig. 3). Antihistamine medications are taken on demand. No relapse of osteoblastoma.

Conclusion

A favorable prognosis for mastocytosis and osteoblastoma was documented in the observed case.

Keywords

Mastocytosis, urticaria pigmentosa, histamine crisis.