Indian Dermatology Online Journal

THROUGH THE LENS
Year
: 2019  |  Volume : 10  |  Issue : 2  |  Page : 204--205

Primary systemic amyloidosis mimicking rhinophyma


Ankita Agrawal, Meena Makhecha, Kinjal Rambhia 
 Department of Dermatology, HBT Medical College and Cooper Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Ankita Agrawal
D-14, Gokul Apartment City Centre Gwalior, Madhya Pradesh - 474 001
India




How to cite this article:
Agrawal A, Makhecha M, Rambhia K. Primary systemic amyloidosis mimicking rhinophyma.Indian Dermatol Online J 2019;10:204-205


How to cite this URL:
Agrawal A, Makhecha M, Rambhia K. Primary systemic amyloidosis mimicking rhinophyma. Indian Dermatol Online J [serial online] 2019 [cited 2021 Dec 7 ];10:204-205
Available from: https://www.idoj.in/text.asp?2019/10/2/204/254287


Full Text



A 55-year-old male presented with asymptomatic nodular growth over nose since 6 months. On examination it was ~5 cm, well-defined, waxy thick brownish plaque extending from left ala of nose to tip of nose with skin overlying showed ridging and dilated pilosebaceous openings [Figure 1]a. On further follow-up, we noticed periorbital ecchymosis, which was not present earlier, few waxy papules and nodules over scalp, lateral aspect of lower eyelids, purpura over neck and toe, finger and toe nails showing dystrophy, striations, and anonychia [Figure 1]b, and tongue showing macroglossia. Histopathology showed eosinophilic deposits in the dermis [Figure 2]a, which on special staining with Congo red showed apple green birefringence of fibrils under polarized light [Figure 2]b. On further investigation, 24 h urine protein levels were raised 793.80 mg/24 h and albumin and gamma band were present on electrophoresis. Therefore, final diagnosis of primary systemic amyloidosis was made.{Figure 1}{Figure 2}

Amyloidosis is a disease caused by extracellular deposition of abnormal insoluble protein fibrils with antiparallel β-pleated sheet. Amyloidosis is classified into systemic amyloidosis and primary cutaneous amyloidosis. The latter is more common than former. Systemic amyloidosis is classified as primary systemic amyloidosis, secondary systemic amyloidosis, hemodialysis associated amyloidosis, and familial form of amyloidosis. Primary cutaneous amyloidosis is classically divided into macular amyloidosis, lichen amyloidosis, and nodular amyloidosis. Primary systemic amyloidosis has various clinical presentations. Cutaneous finding appears early compared to systemic involvement in 40–50% of patients. Deposition of amyloid in dermis gives rise to waxy papules, nodules, and plaques. In our case, deposition of amyloid locally over nose gave rise to rhinophyma-like presentation. On going through the literature, we found head and neck amyloidosis is rare and usually of localized type.[1] Case reports of primary localized nodular amyloidosis over nose are present, but there are no case report of primary systemic amyloidosis.[2] Trachyonychia secondary to amyloid deposits around blood vessels and in the dermis of the nail bed has been reported, as present in our patient.[3] This case illustrates that rhinophyma as a presenting feature is mentioned for localized amyloidosis but not for systemic amyloidosis. It is unusual to think of amyloidosis when patient presents with rhinophyma-like growth in Indian skin, where periorbital ecchymoses may not always be noticed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

We acknowledged our support from Department of Pathology, HBT medical college.

References

1Chin SC, Fatterpeckar G, Kao CH, Chen CY, Som PM. Amyloidosis concurrently involving the sinonasal cavities and larynx. AJNR Am J Neuroradiol 2004;25:636-8.
2Nakayama T, Otori N, Komori M, Takayanagi H, Moriyama H. Primary localized amyloidosis of the nose. Auris Nasus Larynx 2012;39:107-9.
3Derrick EK, Price ML. Primary systemic amyloid with nail dystrophy. J R Soc Med 1995;88:290P-1P.