Nodular Cutaneous Amyloidosis in a Female patient

Location

Virtual

Presentation Date

26-10-2020 10:00 AM

End Date

30-10-2020 3:00 PM

Description

This Submission is a case report focusing on the condition known as nodular cutaneous amyloidosis (NCA). NCA is an extremely rare condition which is characterized by an asymptomatic, pruritic, or possibly painful plaque presenting anywhere on a patient’s body. The rarity of the condition and its similarities with other amyloidoses makes it a formidable diagnostic challenge.

This patient is a 59-year-old black female who presented to clinic for an initial evaluation of a lesion on her back. The lesion had been present for multiple years and was occasionally pruritic. The patient also noted that the lesion had grown albeit very slowly since she first noticed it. There had been not prior treatment of the lesion. She also denied any history of keloids or other bleeding, ulcerating, or non-healing lesions. She does not wear sunscreen or protective clothing daily and attests to a history of high sun exposure and sunburns. On physical exam the patient features a hyperpigmented, reticulated plaque on the right upper back with surrounding reticulated hyperpigmentation. Punch Biopsy of this lesion revealed interstitial deposition of amorphous pink hyaline material as well as perivascular plasma cells. Following these findings, the patient received a serum and urine protein electrophoresis to differentiate an isolated cutaneous amyloidosis from a cutaneous manifestation of a systemic amyloidosis. Both tests returned normal and a diagnosis of nodular cutaneous amyloidosis was made. Upon subsequent clinic visits the patient denied any systemic symptoms, visual changes, neurologic dysfunction, or new cutaneous lesions. She elected not to have her plaque removed surgically.

This case presents the occurrence of the rare condition NCA and provides a brief review of the literature and guidelines pertaining to the disease. The rare nature of NCA is what makes it such an important condition to document whenever present. In the first 43 years since the condition’s initial discovery only 50 total cases were documented. One literature review of the condition conducted in 2008 identified less than 70 total cases of NCA overall. By identifying patients with NCA and following them long term we can add to our knowledge of this rare condition and progress towards a better understanding of NCA.

Comments

Mentor: Alexandra Bourgeois (LSUHSC Department of Dermatology)

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Oct 26th, 10:00 AM Oct 30th, 3:00 PM

Nodular Cutaneous Amyloidosis in a Female patient

Virtual

This Submission is a case report focusing on the condition known as nodular cutaneous amyloidosis (NCA). NCA is an extremely rare condition which is characterized by an asymptomatic, pruritic, or possibly painful plaque presenting anywhere on a patient’s body. The rarity of the condition and its similarities with other amyloidoses makes it a formidable diagnostic challenge.

This patient is a 59-year-old black female who presented to clinic for an initial evaluation of a lesion on her back. The lesion had been present for multiple years and was occasionally pruritic. The patient also noted that the lesion had grown albeit very slowly since she first noticed it. There had been not prior treatment of the lesion. She also denied any history of keloids or other bleeding, ulcerating, or non-healing lesions. She does not wear sunscreen or protective clothing daily and attests to a history of high sun exposure and sunburns. On physical exam the patient features a hyperpigmented, reticulated plaque on the right upper back with surrounding reticulated hyperpigmentation. Punch Biopsy of this lesion revealed interstitial deposition of amorphous pink hyaline material as well as perivascular plasma cells. Following these findings, the patient received a serum and urine protein electrophoresis to differentiate an isolated cutaneous amyloidosis from a cutaneous manifestation of a systemic amyloidosis. Both tests returned normal and a diagnosis of nodular cutaneous amyloidosis was made. Upon subsequent clinic visits the patient denied any systemic symptoms, visual changes, neurologic dysfunction, or new cutaneous lesions. She elected not to have her plaque removed surgically.

This case presents the occurrence of the rare condition NCA and provides a brief review of the literature and guidelines pertaining to the disease. The rare nature of NCA is what makes it such an important condition to document whenever present. In the first 43 years since the condition’s initial discovery only 50 total cases were documented. One literature review of the condition conducted in 2008 identified less than 70 total cases of NCA overall. By identifying patients with NCA and following them long term we can add to our knowledge of this rare condition and progress towards a better understanding of NCA.