In a compelling expository on a rare dermatological disorder, researchers from Queen Elizabeth University Hospital, Glasgow, have shed light on a case of Nodular Primary Localised Cutaneous Amyloidosis (NPLCA). Published in the BMJ Case Reports on May 6, 2019, the case presents a valuable insight into this least understood and rarest form of cutaneous amyloidosis, which prefers the facial and acral skin. The report, which appears under the DOI: 10.1136/bcr-2018-228163, is a significant contribution to the scarce literature available on this malady.
The subject of the study is a 63-year-old Caucasian male, with a 10-year history of an asymptomatic plaque on his left cheek. The intriguing aspect of the case is the lesion’s temporal correlation with a cat scratch occurring two years before its appearance. While a causal relationship remains speculative, the case sheds light on the potential link between physical trauma and NPLCA, a subject that has lacked substantial evidence in the past.
Diagnostic Findings and Treatment Insights
A biopsy of the plaque revealed nodules of eosinophilic material in the deep dermis and subcutaneous fat, accompanied by the presence of plasma cells within the dermis. Congo red staining, a standard histopathological technique for amyloid detection, displayed the characteristic apple-green birefringence, confirming the diagnosis. Further, immunohistochemistry for serum amyloid P component indicated positivity within the eosinophilic material, and lambda light chain restriction was noted within the plasma cells, features in line with NPLCA.
The management of the patient followed a conservative approach, otherwise known as watchful waiting, owing to the lesion’s asymptomatic nature. This laid-back approach aligns with the existing management guidelines for NPLCA, which suggest that intervention is only necessary should functional or cosmetic concerns arise.
Broader Implications and Understanding of NPLCA
Only a handful of reports have indicated associations between NPLCA and trauma, focusing primarily on papular and macular forms of amyloidosis. This case, however, opens up the discourse regarding the potential for physical trauma, such as a cat scratch, to be an inciting factor for NPLCA. Previous studies cited by the authors, such as those noted by Xing and Higuchi (2002), Woollons and Black (2001), and Lee et al. (2011), have explored the pathophysiological underpinnings of localized cutaneous amyloidosis, hinting at the contribution of keratinocytes and immune dysregulation in amyloid formation.
Studies like that of Meijer et al. (2008) and Dahdah et al. (2009), also referenced in the report, highlight the association between localized cutaneous amyloidosis and autoimmune conditions, such as Sjögren’s syndrome, suggesting a broader context of immune system involvement in NPLCA. Furthermore, the investigative work by LaChance et al. (2014) into a bullous variant of NPLCA adds to the complexity of presentation and the need for a diversified diagnostic approach.
The article concludes with a discussion of the prognostic considerations and a deliberation on the rarity of progression to systemic amyloidosis from localized cutaneous forms. Following guidance from the literature, such as that highlighted by Kalajian et al. (2007), the decision to adopt a watchful waiting strategy is informed by the low rate of systemic progression and the benign nature of the lesion.
Keywords
1. Nodular Primary Localised Cutaneous Amyloidosis
2. NPLCA
3. Cutaneous amyloidosis
4. Congo red staining
5. Amyloidosis case study
References
1. Xing Y, Higuchi K. Amyloid fibril proteins. Mech Ageing Dev 2002;123:1625–36. doi: 10.1016/S0047-6374(02)00098-2.
2. Woollons A, Black MM. Nodular localized primary cutaneous amyloidosis: a long-term follow-up study. Br J Dermatol 2001;145:105–9. doi: 10.1046/j.1365-2133.2001.04291.x.
3. Meijer JM, Schonland SO, Palladini G, et al. Sjögren’s syndrome and localized nodular cutaneous amyloidosis: coincidence or a distinct clinical entity? Arthritis Rheum 2008;58:1992–9. doi: 10.1002/art.23617.
4. Lee DY, Kim YJ, Lee JY, et al. Primary localized cutaneous nodular amyloidosis following local trauma. Ann Dermatol 2011;23:515–8. doi: 10.5021/ad.2011.23.4.515.
5. Kalajian AH, Waldman M, Knable AL. Nodular primary localized cutaneous amyloidosis after trauma: a case report and discussion of the rate of progression to systemic amyloidosis. J Am Acad Dermatol 2007;57(2 Suppl):S26–S29. doi: 10.1016/j.jaad.2006.12.014.
Note: Due to constraints, the text provided is an abstract of the larger article that could incorporate all the above information. It’s an informative summary meant to give an overview of the complexity and rarity of NPLCA with references to the supporting literature. The full article would include a more detailed discussion of the patient presentation, diagnosis, management plan, and deeper exploration of the cited studies.