Idiopathic Calcinosis Cutis Universalis

Clinical Cases 06.02.2023
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 77 years, male
Authors: Nuno M. F. Campos1, Luís M. C. Semedo1,2, Paulo Donato1,2
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Details
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AI Report

Clinical History

An elderly male patient referred for a body computed tomography (CT) after incidental findings in the chest radiography that revealed unusual cutaneous calcifications. He had no specific complaints prior or at the moment of the CT. The laboratory data showed normal serum calcium, phosphorus, parathyroid hormone, and muscle enzyme levels.

Imaging Findings

Chest radiography revealed diffuse small subcutaneous calcifications (FIG.1).

Body CT also revealed diffuse small calcifications in the skin and subcutaneous tissues, with no muscle, vessel, or visceral organ invasion (FIG.2). No other relevant findings were apparent in the examination.

The final diagnosis was calcinosis cutis universalis, confirmed by skin biopsy.

Discussion

Calcinosis cutis describes the deposition of calcium salts in the skin and subcutaneous tissue and can be divided into five main types: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis [1,2]. Dystrophic calcification is the most common cause of calcinosis cutis and is commonly seen in patients with systemic sclerosis (up to 40% of patients), dermatomyositis and systemic lupus erythematosus, but can occur in other diseases that lead to connective tissue damage [2-5].

Clinically, it may present as a more circumscribed form - calcinosis circumscripta - usually affecting the fingers and periarticular regions or a more rarer form that is more generalized and severe -  calcinosis universalis [2]. Dystrophic calcification is frequently painful, especially when the process involves areas close to joints or when ulceration is present, but it can occur gradually and be asymptomatic [2,4,5]. Unlike tumoral calcinosis, which presents as a mass-like calcification, calcinosis cutis is characterized by depositions of bands or sheets like symmetrical calcifications in skin, subcutaneous tissues and muscles [6].

The exact physiopathology remains unclear, and there are no clear guidelines of treatment; however, various treatments have been reported to be beneficial, usually with small and localized lesions being good candidates for surgical treatment, whereas more generalized disease will require medical management, namely warfarin, ceftriaxone, probenecid, diltiazem, aluminium hydroxide, colchicine or bisphosphonates [1-5].

When generalized calcinosis of the soft tissues is encountered, diagnosis as systemic sclerosis and dermatomyositis should be suspected. In the case of our patient, no underlying cause was apparent.

Differential Diagnosis List

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Final Diagnosis

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Figures

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Chest radiography (a) showing multiple diffuse small subcutaneous calcifications. In an oblique rib projection (b) the presen
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Chest radiography (a) showing multiple diffuse small subcutaneous calcifications. In an oblique rib projection (b) the presen

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Body CT scan (a, b, c) also revealed diffuse small subcutaneous calcifications (arrows) affecting every segment of the body
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Body CT scan (a, b, c) also revealed diffuse small subcutaneous calcifications (arrows) affecting every segment of the body
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Body CT scan (a, b, c) also revealed diffuse small subcutaneous calcifications (arrows) affecting every segment of the body