An Unusual Case of Deep Localized Scleroderma in a Patient with Chronic Kidney Disease

Localized scleroderma (LS) is a rare fibrosing disorder of skin and underlying tissues. Although it can affect all races, it has a higher prevalence in whites. Deep LS is the least common among seven LS variants, representing less than 5% of cases, and typically affects areas of pressure such as the...

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Published inPuerto Rico health sciences journal Vol. 41; no. 1; p. 45
Main Authors Rodriguez-Baisi, Katinna E, Santaliz-Ruiz Iv, Luis E, Diaz-Martinez, Angel J, Pagan, Angel D, Sánchez, Nestor P, Villa, Jaime
Format Journal Article
LanguageEnglish
Published Puerto Rico Universidad de Puerto Rico, Recinto de Ciencias Medicas 01.03.2022
Subjects
Contrast Media
Disease Progression
Female
Gadolinium
Humans
Male
Middle Aged
Nephrogenic Fibrosing Dermopathy - etiology
Nephrogenic Fibrosing Dermopathy - pathology
Nephrogenic Fibrosing Dermopathy - therapy
Renal Insufficiency, Chronic - complications
Scleroderma, Localized - complications
Skin Diseases
Puerto Rico
Kidney disease
Morphea
Localized scleroderma
Gadolinium
Nephrogenic systemic fibrosis
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Summary:Localized scleroderma (LS) is a rare fibrosing disorder of skin and underlying tissues. Although it can affect all races, it has a higher prevalence in whites. Deep LS is the least common among seven LS variants, representing less than 5% of cases, and typically affects areas of pressure such as the hips and waist. We report a unique clinical case of bilateral lower extremity deep LS in a 51-year-old Puerto Rican woman with chronic kidney disease (CKD). In patients with CKD, it is important to distinguish LS from nephrogenic systemic fibrosis (NSF). Both can present with skin fibrosis and contractures over joints yet have significantly differing treatment approaches and prognosis. Our case report is unique due to the patient's Puerto Rican ethnicity, CKD history, and isolated anterior lower extremity involvement. In this report, we highlight key clinical and histopathological findings of LS, and how they differ from that of NSF.
ISSN:0738-0658
2373-6011