HealthDecember 31, 2020

UpToDate Clinical Story: A Closer Look at Scleroderma with Generalized Morphea

"Over the last thirty years or so, in the face of the rapidly developing scientific and technological progress that characterizes modern medicine, a progressive process of the specialization of medical skills has become the norm. At the same time, with the lengthening of the average life expectancy, there has often been a simultaneous increase in patients affected by several chronic illnesses."

Hence, there is an ever increasing need, within the scope of acute hospital care, to be able to rely on internal medicine departments capable of caring for patients with "polypathology" and those who are "elderly and fragile" or, more simply, patients whose condition cannot be treated in just one specialist department."

This statement is taken from the mission of the Busto Arsizio Hospital's Medicine II Department headed by Dr. Guido Bonoldi. Bonoldi recently used UpToDate with a challenging patient case at the Internal Medicine Clinic.

The clinical case

"I examined a female patient in her mid-eighties in our clinic. She had been referred to us by her GP because of skin abnormalities that were not easily identifiable. (Her GP noted that he had never seen anything similar and was not sure where to begin. In such cases, an internist is consulted). The patient's history included a hysterectomy and surgery to correct vaginal vault prolapse; a myocardial infarction in 2007 treated by angioplasty plus a stent implant in the anterior descending artery; followed by a coronary artery bypass graft; phlebitis in February of this year, treated using LMWH; and another episode of phlebitis resolved spontaneously.

Dr. Guido Bonoldi UpToDate enables physicians to practice medicine while relying on the experience and expertise of more than 6,000 peer specialists...

— Dr. Guido Bonoldi, Head of Medicine II, Busto Arsizio Hospital

The patient had been complaining of a burning and itching sensation, especially on the trunk. Initially, this was localized in the inguinal area, and then extended to the rest of the trunk. There was also cutaneous hyperpigmentation that presented in the same area. The patient had already had blood tests done, which showed no abnormality except for hypergammaglobulinemia.

The patient's GP sent her to us with a diagnosis of migratory thrombophlebitis and a suspected paraneoplastic syndrome.

Upon physical examination, she presented cutaneous sclerosis of the trunk along two lines or bands. One band was just below the breasts, the other along the abdomen and lower inguinal area. Apart from the sclerosis, there was also evident cutaneous hyperpigmentation. The physical examination revealed no other abnormalities.

First, I ordered an abdominal ultrasound, which excluded any findings that might suggest productive processes at a level of the organs being examined.

The diagnosis

"While writing up the patient's report I searched UpToDate for the Italian term for scleroderma (sclerodermia). I then consulted the topic "Overview and classification of scleroderma disorders." In this topic, there is an image entitled, "Generalized morphea on the trunk" (Fig. 1) which corresponded perfectly with the appearance of the patient's disorder, which I had photographed (Fig. 2).

Generalized morphea trunk

Generalized Morphea Trunk - Figure 1

Generalized Morphea Trunk - Figure 2

This was a generalized morphea of the trunk. The condition is one of several manifestations of cutaneous scleroderma. Morphea differs from systemic sclerosis because the latter is a disease with cutaneous manifestations that can also involve several organs.

In the report, I included as a conclusive evaluation, "Suspected scleroderma with generalized morphea on the trunk." I prescribed "additional examinations (chest x-ray and blood tests) to exclude possible systemic involvement."


Since these exams showed no systemic involvement, my initial diagnosis was confirmed. At that point, it was necessary to make a decision about what treatment to adopt.

If UpToDate did not exist, someone would have to invent it.
— Dr. Guido Bonoldi

In this instance, the second topic, "Treatment of morphea (localized scleroderma) in adults" was of great assistance. The topic contained a very useful algorithm called "Treatment algorithm for morphea" that suggested phototherapy as the first choice for treatment.

I then referred the patient to a colleague for a dermatological evaluation which indicated phototherapy together with, in this case, a low-dose steroid therapy.

Dr. Bonoldi concludes, "UpToDate enables physicians to practice medicine while relying on the experience and expertise of more than 6,000 peer specialists, who work for us with the objective of providing the right information at the right time. This reinforces our abilities and enables us to make the best clinical decision. If UpToDate did not exist, someone would have to invent it."

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1. Isaac T, Zheng J, Jha A. Use of UpToDate and outcomes in US hospitals. J Hosp Med. 2011 Nov 16. doi: 10.1002/jhm.944.