Unexpected post-mortem diagnosis of systemic sclerosis presenting as pneumatosis intestinalis: Revised diagnostic criteria and medicolegal implications

• We present a case of systemic sclerosis diagnosed after death of unknown cause.
• We propose applying clinical criteria to autopsy diagnosis of this disease.
• We review additional histopathologic findings specific to this disease.
• We explore pre- and post mortem medicolegal implications of this disease.

Systemic sclerosis is a challenging diagnosis for clinicians and pathologists alike due to its protean manifestations and often insidious onset, particularly in cases without significant titres of auto-antibodies. Herein we present a case of a female in her sixties who died rapidly following a clinical diagnosis of pneumatosis intestinalis and respiratory failure of unclear etiology. Recently revised clinical diagnostic criteria were applied to the clinical history and postmortem findings to reach an unexpected diagnosis of systemic sclerosis. The diagnosis of systemic sclerosis at autopsy has important medicolegal implications largely related to premature death due to delayed treatment or poor post-operative outcome. Moreover, familial clustering of this disease underscores the importance of maintaining a high index of suspicion in the postmortem setting.