|Year : 2019 | Volume
| Issue : 2 | Page : 104-106
Dermoscopy of localized cutaneous nodular amyloidosis resembling granulomatous disorders
Bhavesh Sonagara, Hita Mehta, Prachi Gajjar
Department of Dermatology, Government Medical College and Sir T Hospital, Bhavnagar, Gujarat, India
|Date of Web Publication||28-Nov-2019|
Dr. Hita Mehta
201, Golden Arc Apartment, Atabhai Chowk, Bhavnagar, Gujarat
Source of Support: None, Conflict of Interest: None
Cutaneous amyloidosis refers to a group of skin diseases, which is characterized by the deposition of amyloid material in the skin. Among them, Localized cutaneous nodular amyloidosis (LCNA) is the rarest type of cutaneous amyloidosis. A 38-year-old male presented to dermatology OPD with a single soft nodular lesion on left cheek. On basis of clinical and dermoscopic examination we thought it as granulomatous disease but histopathologically confirmed diagnosis of LCNA. Systemic amyloidosis is excluded on clinical as well as laboratory bases. The patient was treated with topical tacrolimus with slight improvement and followed up for 1 year and no systemic abnormalities were found.
Keywords: Dermoscopy, nodular amyloidosis, yellow-orange background
|How to cite this article:|
Sonagara B, Mehta H, Gajjar P. Dermoscopy of localized cutaneous nodular amyloidosis resembling granulomatous disorders. Indian J Dermatopathol Diagn Dermatol 2019;6:104-6
|How to cite this URL:|
Sonagara B, Mehta H, Gajjar P. Dermoscopy of localized cutaneous nodular amyloidosis resembling granulomatous disorders. Indian J Dermatopathol Diagn Dermatol [serial online] 2019 [cited 2020 Jun 1];6:104-6. Available from: http://www.ijdpdd.com/text.asp?2019/6/2/104/271949
| Introduction|| |
Primary localized cutaneous amyloidosis is a metabolic disease of the skin that is characterized by extracellular deposition of amyloid proteins in the skin, without evidence of systemic involvement. Primary cutaneous amyloidosis is subdivided into macular, papular, and nodular types. Among these subcategories, localized cutaneous nodular amyloidosis (LCNA) is the rarest type, which presents as a single or rarely multiple nodules of varying size. Dermoscopy is a novel, noninvasive technique in dermatology practice which aids in clinical diagnosis. Here, in this case, we observed dermoscopic findings of LCNA mimicking granulomatous diseases, but histopathologically, it was confirmed as nodular amyloidosis.
| Case Report|| |
A 38-year-old male patient presented with asymptomatic soft, elevated growth on the left cheek, which gradually increased in size over 3 years. The patient mentioned that initially, the lesion was reddish in color which later on turned to brownish-orange color. There was no specific history suggestive of systemic involvement. We carried out a detailed general, dermatological, and systemic examination of the patient after written informed consent. Local examination showed a 2 cm × 2.5 cm sized, well-demarcated, brownish-orange colored, soft waxy nodule on the malar area of the left cheek [Figure 1]. Dermoscopy illustrated yellow-to-orange background with red linear and curved vessels. There were multiple tiny uniform sized, regularly arranged white dots and few structureless white areas [Figure 2]. On the basis of clinical and dermoscopic findings, sarcoidosis and lupus vulgaris were kept as differentials. Histopathology demonstrated patchy nodular deposits of uniformly pink-stained amorphous material within the deep dermis and subcutaneous tissue [Figure 3]. Congo red staining showed apple-green birefringence under polarized light, which was consistent with nodular amyloidosis [Figure 4]. Laboratory tests including complete blood count, renal and liver function test, viral marker, rheumatoid arthritis factor, echocardiogram, chest X-ray, ultrasonography of the abdomen, and pulmonary function test were normal. Specific test such as serum protein electrophoresis and urine Bence Jones protein were negative which excluded systemic amyloidosis. The patient was treated with topical tacrolimus.
|Figure 1: Single soft waxy brownish-orange color nodule on the left face|
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|Figure 2: Dermoscopic (polarized noncontact) image showing yellow to orange homogeneous background (star) with linear red lines (black arrow), multiple uniformed sized tiny white dots (circle), and few structureless white areas (triangle)|
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|Figure 3: Histopathology demonstrated patchy nodular deposits of uniformly pink-stained amorphous material within the deep dermis and subcutaneous tissue (H and E, ×10)|
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|Figure 4: Characteristic apple-green birefringence under polarized light (Congo-red staining)|
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| Discussion|| |
Amyloidosis is characterized by the deposition of amyloid in tissues. Deposits can only be limited in the skin without any evidence of systemic involvement, namely primary localized cutaneous amyloidosis, or can be systemic and involve multiple organs and tissues, namely primary or secondary systemic amyloidosis. Nodular amyloidosis is the rarest type of primary localized cutaneous amyloidosis. It has somewhat distinct characteristics when compared to the other types of cutaneous amyloidosis. It clinically may mimic sarcoidosis, necrobiosis lipoidica, lupus vulgaris, and leprosy which are difficult to differentiate only on a clinical basis.
Dermoscopy is a novel noninvasive diagnostic technique, which permits a detailed view of structures within the skin which are usually invisible to unaided eye. There is a paucity of literature on dermoscopic findings of nodular amyloidosis. A single study by Rongioletti et al. revealed central orange-yellowish homogeneous background with elongated and serpentine telangiectasia. However, these similar dermoscopic findings (yellowish-orange background and red vessels) were observed in granulomatous conditions such as sarcoidosis, lupus vulgaris, necrobiosis lipoidica, and granuloma annulare by Errichetti and Stinco, Chauhan et al., and many other authors.,,
On searching available literature, we could not find a correlation between dermoscopy and histopathology of nodular amyloidosis. However, it is our opinion that yellowish-orange background in dermoscopy may be correlated with deposits of uniformly, pink-stained, amorphous material within the deep dermis and subcutaneous tissue on histopathology. Multiple, tiny uniform sized, white dots in our observation can probably be explained by the presence of eccrine gland openings.
It has been reported that cases of localized nodular amyloidosis have 7%–50% tendency to progress to systemic amyloidosis., We followed the patient for 1 year to exclude any systemic involvement. Different treatment options described in the literature are topical steroids, topical tacrolimus, dermabrasion, intralesional methotrexate injections, and surgical shaving of lesions by knife and lasers., As lesion was on the face and having little telangiectasia, so in order to avoid topical corticosteroids we prescribed topical tacrolimus, after which mild improvement in terms of lightening of color, was evident clinically and dermoscopically [Figure 2] within a period of 6 months. Laser is, nowadays, a better option but carries risk of hemostasis during intervention as described in the literature.
Yellowish-orange background with linear vessels is the dermoscopic finding of many granulomatous diseases, but nodular amyloidosis should also be considered as a differential diagnosis and need to be confirmed histopathologically. Dermoscopy may be helpful in diagnosis as well as therapeutic monitoring. Our study adds little evidence in scarcity of such literature. More number of studies are needed in future for a detailed comparison of clinical, dermoscopic, and histopathological features of nodular amyloidosis.
Declaration of patient consent
It is to certify that we 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 initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]