Digital pulp ulceration in an adolescent girl

Nail pathology

Dr. Emmanuelle Bourrat

Saint-Louis Hospital

The clinical case
Now it's your turn!
Co-prescription and advice

Clinical case presentation

A 12-year-old girl had been suffering for several months from acrocyanosis, which had appeared in the spring and was worsening despite the high summer temperatures. In July, she went to the emergency department with severely painful digital ulcerations on several fingers of her left hand, with early involvement of the contralateral hand.

She was pubescent, had no previous medical history and took no medications, tobacco or recreational drugs. Cardiac auscultation was normal, peripheral pulses were perceptible and symmetrical, she was afebrile and had no digestive or pulmonary complaints. The fingers were puffy, shiny and cold, with dry necrosis of the 2nd and 3rd fingers of the left hand. The patient was admitted to the hospital for therapeutic management, and a comprehensive etiological workup was carried out.

Your turn

What is your diagnosis?

Select 1 answer(s) from the following choices:

Drug-induced or toxic cause

Embolic cause

Systemic sclerosis

Primary acrocyanosis

Wrong answer!

Good answer!

Selected diagnosis

This severe acrocyanosis persisting in summer and complicated by trophic changes points to an organic cause, confirmed by nailfold capillaroscopy: inconspicuous capillaries, a few giant capillaries (megacapillaries), reduced capillary density and hemorrhages compatible with organic microangiopathy of the systemic sclerosis type. The immunological workup was also in favor of this diagnosis, with positive nuclear antibodies at 1/1600 and positive anti-Scl-70 antibodies.

The disease staging workup showed no digestive, cardiac, pulmonary or renal involvement of this systemic sclerosis.

It is therefore a case of limited cutaneous systemic sclerosis presenting with acrocyanosis and digital pulp ulcerations without a complete Raynaud’s phenomenon.

Explanation of wrong answers

A drug-induced or toxic cause should be sought as a first-line priority, though unlikely given the age of the child. There are many potent vasoconstrictor drugs (including beta-blockers), but few are likely to cause digital necrosis: bleomycin, ergotamine and vincristine. Tobacco, tetrahydrocannabinol, cocaine and crack cocaine may also be involved.
Embolic causes are uncommon at this age. Cardiac auscultation was normal, but a vascular workup (echocardiography and CT angiography) is systematically proposed (unremarkable here).
Primary “idiopathic” acrocyanosis is common in adolescent girls, especially in cases of low body mass index, which was not the case here. The persistence of symptoms during the warm season and especially the presence of trophic changes other than chilblains indicate the secondary and organic nature of this acrocyanosis.

Treatment

Due to intense, sleep-disrupting pain unrelieved by step 2 analgesics, the patient was hospitalized and treatment with intravenous iloprost for 5 days, which led to disappearance of the cyanosis and pain, and rapid healing of the ulcerations.

Message from the expert

The occurrence of digital necrosis is a diagnostic and therapeutic emergency; it may be an early sign of systemic sclerosis, even in children.

References:

^{WigleyFM,WiseRA,SeiboldJR, et al.}^{Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Ann Intern Med 1994;120(3):199-206}