Wednesday, August 10, 2022

An unusual case of deep scleroderma in a patient with chronic kidney disease in Puerto Rico.

This was a 51-year-old woman with bilateral painful bandages.

Deep localized scleroderma. During follow-up evaluation 6 months later, the lesions further limited mobility of the patients to the thighs. Photo: Case report.

Morphea or localized scleroderma (LS) is a condition that causes lesions, also affecting the dermis, fatty tissue, underlying muscle and bone, and belongs to a group of diseases that are grouped under the term “scleroderma”. which means hardening of the skin.

It is the least common of the seven LS variants, accounting for less than 5% of cases. According to the literature it usually affects pressure areas such as the hips and groin. The worldwide prevalence of this connective tissue disease is 0.4–2.7 per 100,000, with a female:male ratio of 3:1. Although it can affect all races, LD is more prevalent in whites and usually affects areas of body weight such as the hips.

and waist.

According to the island’s doctors, in Puerto Rico The first case reported in their opinion was a bilateral deep LE in the lower extremities of a 51-year-old Puerto Rican woman with chronic kidney disease (CKD).

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This was a 51-year-old woman who went to a dermatological service because of bilateral painful plaques that she presented with for two months on her lower leg.

The patient also had orange peel discoloration of the skin, edema and leucoderma. Her history included stage 5 CKD on peritoneal dialysis, hypertension, type 2 diabetes mellitus and psoriasis.

They also reported use with erythropoietin (also known as “Epo” or Epogen) to prevent or treat anemia, but ruled out exposure to gadolinium (Gd), which can cause allergic reactions. can.

Laboratory tests were positive for ANA, elevated erythrocytes, and biopsy revealed thickening of the dermis with collagen bundles and some perivascular lymphocytes.

The patient had received ultraviolet-A (UVA) and phototherapy in another clinic for a period of two months with no improvement. At follow-up six months later, the lesions had reached the patient’s thighs, limiting mobility. After a careful review of the literature, we determined that the best treatment plan was methotrexate 7.5 mg once a week and prednisone 20 mg once a day.

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Two days after taking her first dose, methotrexate was discontinued due to adverse events. The patient was subsequently hospitalized for CKD progression.

In patients with CKD, it is important to differentiate LD from nephrogenic systemic fibrosis (NSF) – a fibrosing disorder that affects patients with impaired renal function – as both conditions can present fibrosis in the skin and underlying joints, but treatment and The prognosis is different.

“Our case is unique because of Puerto Rican ethnicity, history of CKD, and injuries to the patient’s lower extremities in particular. In addition, we present clinical and histopathological differences between LD and NSF”, the case concluded. part of reads.

Access the report here.

Nation World News Desk
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