A healthy 8-year-old girl of Nigerian origin, presented in January 2014 with a 2 month history of progressive pain and swelling of the right 2nd, 3rd and 4th toe. There was no preceding trauma or illness. Those toes were swollen, tender and cold to touch, with bluish skin discoloration (Figure 1).
She had normal peripheral pulses. Her inflammatory markers were normal, as was haemoglobin electrophoresis. A Doppler ultrasound study ruled out vascular obstruction. X ray showed acro-osteolysis of the toes involved (Default 2). MRI revealed bone oedema changes in the affected phalanges (MRI fat saturation image Figure 3).
She improved without any intervention in summer, which alongside the typical radiographic appearance, confirmed the diagnosis of Phalangeal Microgeodic Disease.
Repeat MRI in September showed almost complete resolution.
However the following November her toes became itchy, and by January 2015 they were swollen, painful and red again.
Phalangeal Microgeodic Disease is rare, with approximately 20 cases reported since the original description by Maroteaux in 1970. It usually affects the hands, whereas our case had foot involvement which is even rarer.
The aetiology is not clear, but cold exposure has been suggested as cases usually present in winter. Radiologic features typically show osteosclerosis with multiple small spots of osteolysis in the middle or proximal phalanx. MRI reveals evidence of bone marrow oedema.
Awareness of the diagnosis is important to prevent children being subjected to invasive investigations such as biopsy or inappropriate treatment such as antibiotics.
Disclosure: The authors declared no competing interests.
27 - 30 Jun 2015