Calciphylaxis is an uncommon and complex syndrome characterized by the occlusion of micro-vessels within the subcutaneous tissue, resulting in painful ischemic skin ulcers. Its prognosis is poor, and the disease is associated with a high mortality rate. This rare condition usually affects patients with chronic kidney disease, but it may also occur in patients with normal kidney function. The pathogenesis of calciphylaxis is unlikely to be linked to vascular calcifications, which are frequently seen in the general population. Therapeutic guidelines are still lacking, whereas recent studies have improved the understanding of the condition’s physiopathology, enabling us to expect encouraging therapeutic approaches. Through a clinical case, this paper highlights the condition’s diagnostic difficulties and discusses its physiopathology, as well as treatment procedures.
Many conditions may cause painful edema of one or more extremities. Eosinophilic fasciitis (EF), also called Shulman syndrome, is an uncommon one, evolving into sclerodermoid cutaneous infiltration, associated with peripheral blood eosinophilia. EF is a fibrotic disease of muscle fascia and skin, whose etiology remains unclear. Several pathophysiological mechanisms have nevertheless been proposed, the major component being an imbalance between extracellular matrix production and degradation. Visceral involvement is uncommon. EF may be associated with blood disorders, which determine its prognosis. The diagnosis is confirmed by elliptical full-thickness incisional biopsy of skin and subcutaneous tissues down to the muscle surface. EF is most likely underdiagnosed due to its rarity and its similarities with other conditions, such as systemic sclerosis. The typical first-line treatment is systemic corticosteroid therapy tapered over several months. Cortico-resistant and severe-onset forms require adding another immunomodulatory agent. In this case report, we describe a typical presentation of EF, highlighting the diagnostic difficulties posed by this uncommon pathology. Based on a literature review, we also recall its underlying pathophysiological mechanisms, along with the diagnostic and therapeutic steps.