JPRAS Open 6 (2015) 25e30. Contents lists available at ScienceDirect. JPRAS Open. journal homepage:

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1 JPRAS Open 6 (2015) 25e30 Contents lists available at ScienceDirect JPRAS Open journal homepage: jpras-open Case report Treatment of rare nodular amyloidosis on the nose: A case report C.R. Schipper a, *, A.J.M. Cornelissen a, b, C.F.M. Welters b, M.M. Hoogbergen a a Department of Plastic and Reconstructive Surgery, Catharina Hospital, Eindhoven, The Netherlands b Department of Plastic and Reconstructive Surgery, St. Anna Hospital, Geldrop, The Netherlands article info abstract Article history: Received 26 July 2015 Accepted 29 July 2015 Available online 12 September 2015 Keywords: Nodular cutaneous amyloidosis NLCA Treatment Autoimmune disease Introduction: Cutaneous amyloidosis refers to a rare group of skin disease, which is characterized by the deposition of amyloid material. Nodular localized cutaneous amyloidosis (NLCA) is the rarest type of localized cutaneous amyloidosis. A 54-year-old woman presented to the clinic with two lesions on the nose. We describe the examinations that led to the diagnosis of NLCA and the treatment given to her. Methods: PubMed was used to find literature about the treatment of nodular amyloidosis. After carefully weighing different treatment options and consulting colleagues, the shaving techniques were considered the best treatment option for this patient. Results: The shaving technique gave excellent results for this patient in treating the nodular localized cutaneous amyloidosis. Conclusions: The shaving technique is a good treatment option for patients with nodular localized cutaneous amyloidosis The Authors. Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons. This is an open access article under the CC BY-NC-ND license ( creativecommons.org/licenses/by-nc-nd/4.0/). Introduction Cutaneous amyloidosis refers to a rare group of skin disease, which is characterized by the deposition of amyloid material. Nodular localized cutaneous amyloidosis (NLCA) is the rarest type of * Corresponding author. Catharina Hospital, Michelangelolaan 2, 5623 EJ Eindhoven, The Netherlands. Tel.: þ31 (0) ; fax: þ31 (0) (PCH, St Anna). address: carlijn.schipper@catharinaziekenhuis.nl (C.R. Schipper) / 2015 The Authors. Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons. This is an open access article under the CC BY-NC-ND license (

2 26 C.R. Schipper et al. / JPRAS Open 6 (2015) 25e30 localized cutaneous amyloidosis. Since the first report of NLCA in 1950 by Gottron, approximately 60 cases have been reported in the North American, European and Asian literature. Numerous treatments have been introduced in an attempt to improve the appearance of NLCA lesions, including shaving, excision and split-skin grafting, 1 dermabrasion, 2 carbon dioxide laser, 3 topical and intralesional corticosteroids, cryotherapy and pulsed dye laser. 4 In this case report, we present a woman with a rare form of NLCA and the treatment given to her. Case report A 54-year-old woman presented to the clinic with two lesions on the nose: one lesion on the right side of the nose bridge and one on the left nostril, 1.5 cm and 0.7 cm, respectively (Figure 1). Further examination of the lesions showed that they were skin-coloured to purpuric, waxy and eminent. The patient had noticed the tumours for the first time a few years earlier. Her history revealed psoriasis and breast cancer, and she smoked 15 cigarettes a day. Her family history for systemic diseases was negative. Background Amyloid Amyloid is a combined term for abnormal, insoluble, fibrillar-formed, extracellular proteins that share some important characteristics. The diagnosis of amyloid is established by biopsy. All amyloids are eosinophilic, and they turn apple green when viewed under polarized lighting after staining with Congo red. 5 The classification of amyloidosis can be based on the type of the precursor protein or on the clinical classification. 6 The clinical classification is an older classification based on the clinical presentation of the patient, and it categorizes amyloidosis as primary, secondary, familial and isolated amyloidosis. The first three mentioned are usually, but not always, systemic diseases. Isolated Figure 1. Detailed picture of the two marked lesions on the nose.

3 C.R. Schipper et al. / JPRAS Open 6 (2015) 25e30 27 amyloidosis is restricted to a single organ. Since the 1970s, the classification was based on the type of protein. The common clinical amyloid entities are AL, AA, ATTR and Ab2M types. Both classifications are still used. Cutaneous amyloidosis Cutaneous deposits of amyloid can be discerned in 29e40% of the cases with primary systemic amyloidosis, but they are very rare in the other systemic types. If the cutaneous amyloidosis appears not to be a symptom of a systemic amyloidosis, it is called a primary localized cutaneous amyloidosis. There are three types of primary localized cutaneous amyloidosis: nodular, macular and lichen amyloidosis. Nodular amyloidosis is the rarest type. 7 The main symptom of macular and lichen amyloidosis is pruritus, and it is rarely seen in the nodular form (Table 1). Nodular localized cutaneous amyloidosis NLCA is most often benign and limited to the skin. Cases developing into a systemic disease are described in series with small numbers of patients with rates varying from 7% to nearly 50%. 8 The mean age of onset has been reported to be 55 years, with a range from 33 to 86. The cause of the disease is still unknown, but there seems to be an association between NLCA and autoimmune disorders, such as CREST syndrome, rheumatoid arthritis, primary biliary cirrhosis and systemic lupus erythematosus. 9 In previous case reports, 25% of the patients had Sj ogren syndrome. 10 Systemic amyloidosis It is important to rule out systemic amyloidosis in a patient with NLCA, because systemic amyloidosis is a progressive, incurable, metabolic disease in which amyloid deposits in one or more organs tend to cause failure. The most common systemic amyloidosis is the AA form of amyloidosis; it was formerly termed secondary amyloidosis. The diagnosis of systemic amyloidosis can be made based on clinical features, studying the amyloid protein types and imaging tests. Treatment options The treatment for NLCA is still experimental. As mentioned, several techniques have been used in an attempt to treat NLCA in the best possible way. Unfortunately, none of these treatments totally Table 1 Types and characteristics of cutaneous amyloidosis. Type of cutaneous amyloidosis Type of amyloid composition Mostly affected location on the body Appearance of lesion Can be a sign of systemic amyloidosis Nodular AL amyloid Symmetrical noduli on ears, joint cavities, face, trunk, limbs, genital area Pink to brown red noduli Macular AK amyloid Back and shoulders Hyperpigmented skin with an unsharp margin Lichen AK amyloid Pretibial Multiple pink to brown hard hyperkeratotic papules Yes No No

4 28 C.R. Schipper et al. / JPRAS Open 6 (2015) 25e30 eradicate the amyloid depositions due to the persistence of amyloid in the reticular dermis. Therefore, there is always a possibility of recurrence. Results Before treatment, the patient was referred to a dermatologist. A 3-mm biopsy of the lesion on the right side of the nose was performed. Microscopic examination has shown a homogeneous eosinophilic stroma of the dermis with locally resembling colloid bodies. The stroma discoloured red under Congo red staining and apple green when viewed with polarized light. This is typical for amyloid deposits. In this case, an internist excluded a systemic disease. Her family history for systemic diseases was negative. This makes hereditary amyloidosis improbable, as it is an autosomal dominant disease, and age of onset is typically between 5 and 15 years. The pathology report of the biopsy showed a non-aa type of amyloidosis, which excluded secondary amyloidosis. In order to exclude primary systemic amyloidosis, additional examinations were done: chest radiography, abdominal ultrasonography, urine and blood tests. These tests were normal, and they showed no indications for a systemic component of amyloidosis. After exclusion of systemic amyloidosis, the diagnosis of NLCA was made. After a literature search and careful considerations consulting nine plastic surgeons at our monthly outpatient consulting hour, the two lesions were shaved with a 15-blade knife and equalized by dermabrasion. Haemostasis was well achieved. At 24 months follow-up, there were no signs of recurrence or systemic disease (Figures 2 and 3). Figure 2. Patient 1 week after treatment.

5 C.R. Schipper et al. / JPRAS Open 6 (2015) 25e30 29 Figure 3. Patient 6 months after treatment. Discussion In this case, systemic amyloidosis was excluded. In some cases, unfortunately, a local amyloidosis has the tendency to progress to a systemic disease in at least 7%, and rates up to 50% have also been reported. Because of this wide interval, the necessity for follow-up remains to be established. The cause of NLCA is unknown, but it is associated with autoimmune disorders. Our patient has a history of psoriasis. To our knowledge, this association has never been described in the literature. Different treatment options were considered. Because of the size and location of the noduli, primary excision and closure was not an option without leaving massive scars. Laser therapy was also considered, but it was not used in view of the thickness of the noduli. Moreover, haemostasis problems during a laser intervention were described in a previous case report with a nasal lesion. Dermabrasion was performed. Conclusion When a nodular skin lesion is present in a patient with an autoimmune disorder, an amyloid deposition should be considered. If a biopsy confirms amyloid deposition, it is necessary to exclude a systemic cause. If the diagnosis of NLCA is established, follow-up of the patient after treatment should be performed. Treatment of NLCA is still experimental, and recurrences have been mentioned. In this experience, the shaving technique is a good treatment option for patients with NLCA. Conflicts of interest None. Funding None.

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