Unusual case of deep scleroderma in a patient with chronic kidney disease in Puerto Rico

It was a 51-year-old woman with bilateral painful plaques.

Deep localized scleroderma. The lesions had extended proximally to the patients thighs, further limiting mobility, during follow-up evaluation 6 months later. Photo: Case Report.

Morphea or localized scleroderma (LS) is a condition that causes lesions, also affects 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 among the seven LS variants, accounting for less than 5% of cases. Typically, it affects pressure areas such as the hips and waist, according to the literature. This connective tissue disease has a worldwide prevalence of 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 typically affects weight-bearing areas of the body such as the hips

and waist.

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

It was a 51-year-old woman who went to the dermatology service due to bilateral painful plaques that she presented in the lower part of the leg for two months.

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

He also reported having been under reported treatment with erythropoietin (also called “epo” or Epogen) used to prevent or treat anemia) but denied exposure to gadolinium (Gd), which can cause allergic reactions.

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

The patient had received Ultraviolet-A (UVA) and phototherapy at another clinic for a period of two months without improvement. At follow-up six months later, the lesions had progressed proximally to the patient’s thighs, limiting her 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.

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 distinguish LD from nephrogenic systemic fibrosis (NSF) – a fibrosing disorder that affects patients with impaired renal function – since both conditions can present fibrosis in the underlying skin and joints, but have treatments and prognosis different.

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

Access the report here.

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