Atypical Presentation of Congenital Insensitivity to Pain With Anhidrosis Leading to Diagnostic Odyssey.

Background: Congenital insensitivity to pain with anhidrosis (CIPA) (OMIM 256800) is a rare autosomal-recessive condition, also known as hereditary sensory and autonomic neuropathy type IV (HSAN-IV). The most commonly reported features include anhidrosis, intellectual disability, self-mutilation, febrile episodes, impaired temperature perception, recurrent infections and/or autonomic nervous system impairment. Major joint destruction and joint deformity known as Charcot (neuropathic) joints are also seen in CIPA patients attributed to insensitivity to joint pain.

IgG4-related disease presenting as a solitary paratesticular fibrous pseudotumor.

Paratesticular fibrous pseudotumors (PFP) have long been described in the literature. Since the recognition of IgG4-related disease (IgG4-RD) as a distinct clinical entity, cases of PFP with similar pathological characteristics have been described. A case of IgG4-RD presenting as an isolated PFP is presented.