Raynaud's with Digital Ulcerations in a Smoker

The patient is a 63-year-old waitress who presents for evaluation of painful fingertip ulcerations that have been present for ten months. She has been seen by a number of physicians including the Wound Care Center. Nothing has helped.  She has had Raynaud’s Disease for 7 – 8 years.  She has been a heavy smoker for decades and has cut down from two packs per day to 1/2 ppd a month or so ago.

Medications: Nifedipine for her fingertip ulcers without any help.  She’s also taking lisinopril and carvedilol for hypertension. Her ulcerations were treated with Bactrim and oral clindamycin without effect.

The Examination shows a pleasant articulate woman. She has markedly erythematous hands that appear somewhat puffy. There are two purulent subungual digital ulcers.

Clinical Photos: (1/16/17)
The erythema was quite dramatic.  I should have taken my associate's hand next to the patient's hand for comparison.

IMPRESSION:

Raynaud’s disease with ulcerations. Should we consider progression to scleroderma?  Could this be Buerger’s Diasease?. Her history of smoking may be significant.

Lab: ANA 1:2560 Centromere, RF 25, C4 low 94, C3, nl.  Low titer antibeta 2GP Igm. Negative ACA and LAC, negative ENA, dsDNA, CCP
Xray: resorption of distal L small finger

Note:
Raynaud's occurs in two main types:

• Primary Raynaud's is the most common form of the disorder and is not connected to an underlying disease or related medical problem. It is also called Raynaud’s phenomenon.
• Secondary Raynaud's is also called Raynaud's phenomenon. This form is caused by an underlying, or related, problem. Secondary Raynaud's is less common than the primary form, but it tends to be a more serious disorder. Symptoms of secondary Raynaud's often first appear at later ages — around 40 — while people with the primary form often see symptoms earlier.

PLAN: I had a long discussion with the patient. We talked about the need to stop smoking and she promised to do so. A prescription for Bactroban ointment was given and she will be seen back in one week.

With her permission, she is being presented here on VGRD.

Follow-up:
The digital ulceration of the left fifth finger has progressed to distal reabsorption with bone protrusion.  X-rays and orthopedic consultation is pending.

Comments of Richard Sontheimer, M.D.
These are extended and useful remarks that begin with: "This patient’s digital ulceration and the puffiness of her fingers together argue strongly in favor of systemic sclerosis. The fingertip ulceration in your patient would be highly atypical for isolated benign Raynaud's disease.  And, Buerger's disease would not typically produce the sausage-like digital swelling,"  Please read the entire link as it is very helpful.
Also see Reference # 3 below.

Reference:

1. Ann Dermatol Venereol. 2013 Aug-Sep;140(8-9):549-54. doi: 10.1016/j.annder.2013.02.010. Epub 2013 Apr 11.
[Minimal work-up for Raynaud syndrome: a consensus report. Microcirculation working group of the Société française de médecine vasculaire]. [in French]

Pistorius MA1, Carpentier PH; le groupe de travail « Microcirculation » de la Société française de médecine vasculaire.

Abstract: About ten to fifteen percent of the French population suffer from Raynaud's phenomenon. Most of the time, it is considered as primary Raynaud's phenomenon, without underlying disease. The aim of this expert consensus from the "microcirculation group" for the French Society of Vascular Medicine and the French Society for Microcirculation, was to define clinical guidelines in patients consulting for Raynaud's phenomenon. The recommended minimal screening includes clinical examination, nailfold capillaroscopy and antinuclear antibodies. In particular, the aim of this screening is to identify patients with a significant risk for scleroderma, who would need a careful follow up.
  

2. A portable dermatoscope for easy, rapid examination of periungual nailfold capillary changes in patients with systemic sclerosis.
Muroi E, et. al. Rheumatol Int. 2011 Dec;31(12):1601-6. doi: 10.1007/s00296-010-1532-0. Epub 2010 May 27.

Abstract: excerpt “The presence of two or more enlarged capillaries in one or more fingers showed 83.1% sensitivity and 100% specificity for SSc. Among patients with SSc with anti-topoisomerase I antibody, the disease duration correlated negatively with the dermatoscopic number of enlarged capillaries and hemorrhages. Dermatoscope allows the easy and rapid identification of capillary nailfold morphological changes in SSc and should be routinely used for diagnosing SSc.”
 

3. Proximal nailfold microhemorrhage events are manifested as distal cuticular (eponychial) hemosiderin-containing deposits (CEHD) (syn. Maricq sign) and can aid in the diagnosis of dermatomyositis and systemic sclerosis.
McBride JD, Sontheimer RD.  Dermatol Online J. 2016 Feb 17;22  Free Full Text Online

CONCLUSIONS AND RELEVANCE:

Cuticular (eponychial) hemosoderin-containing deposits (CEHD) are in fact hemosiderin-containing deposits that can reflect the active inflammatory phase of microvascular injury occuring in autoimmune disorders such as DM and SSc. CEHD can be visualized and documented at the bedside with tools commonly available to any dermatologist (portable dermatoscope and compact digital camera).