Idiopathic Systemic Capillary Leak Syndrome: Cutaneous Involvement Can Be Misleading

Abstract

Background: Systemic capillary leak syndrome (SCLS) is a severe disorder characterized by unexplained rapid transfer of considerable volumes of plasma from the intravascular to the extravascular compartment. For some cases of SCLS, no aetiology is evident and these cases are reported as idiopathic (ISCLS). Objectives: To describe the cutaneous findings in 3 patients with ISCLS. Results: Cutaneous involvement consisted in sclerosis, livedo, purpura and photodistributed maculopapular erythematous rash. Dermal mucinosis was proven by biopsy in 1 patient. No underlying disease was diagnosed during follow-up. Conclusion: The above-mentioned cutaneous findings can be present during acute attacks of ISCLS. They seem specifically related to the ISCLS and not indicative of an underlying disease.

Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome)

“…For example, perivascular mononuclear infiltrates have been detected in only a few cases of SCLS, whereas most skin biopsies done in these patients have been reported as normal at the light microscopic level. [27][28][29][30][31][32][33] These studies are consistent with our finding that soluble serum factors present in SCLS sera induce endothelial hyperpermeability without a requirement for accessory immune cells (eg, leukocytes) to elicit an inflammatory vasculitis. The absence of substantial acute inflammation also makes involvement of traditional inflammatory mediators of vascular leakage such as TNF-␣, platelet activating factor, thrombin, histamine, and IL-8 unlikely.…”

Idiopathic Systemic Capillary Leak Syndrome(SCLS): Case Report and Systematic Review of Cases Reported in the Last 16 years

“…Ch a r a c t e r i s t i c s o (12,13). There was accompanying generalized edema in 37 cases with weight gain reported in 10 cases ranging from 2-29 kg (6,9,(14)(15)(16)(17). Some reports described the edema to be non pitting (4,14).…”

“…There was accompanying generalized edema in 37 cases with weight gain reported in 10 cases ranging from 2-29 kg (6,9,(14)(15)(16)(17). Some reports described the edema to be non pitting (4,14). Accompanying effusions/tissue edema included bilateral pleural effusions (9,15), pericardial effusion/tamponade (9,10,18,19), cerebral edema (20), epiglottic edema (10) and even macular edema leading to blindness (21).…”

“…Accompanying effusions/tissue edema included bilateral pleural effusions (9,15), pericardial effusion/tamponade (9,10,18,19), cerebral edema (20), epiglottic edema (10) and even macular edema leading to blindness (21). Compartment syndromes or rhabdomyolysis (reflecting leakage into muscles) occurred in 12 cases, some even requiring fasciotomy (4,10,14,18,(22)(23)(24)(25)(26). Pulmonary edema was reported in 8 cases, all in the recovery phase after intravenous fluid replenishment (6,10,13,15,21,24,25,27).…”

Síndrome de extravasamento capilar sistêmico

“…10 Cappilary leak occurs in infections (sepsis, dengue shock syndrome), hereditary angioedema caused by deficiency of C1 esterase, systemic mastocytosis, chemotherapy, malignancy and hemophagocytic syndrome and these should be discarged against new cases of shock with laboratory evidence of plasma extravasation. To characterize an idiopathic form it should also be discarded Carbon monoxide poisoning, maternal status of postpartum and pustular psoriasis.…”