Acta Dermato-Venereologica 98-9CompleteContent | Page 18

900 SHORT COMMUNICATION Methotrexate for the Treatment of Recalcitrant Primary Localized Cutaneous Amyloidosis: A Case Series Carolyn M. STULL 1 , Hong Liang TEY 2,3 and Gil YOSIPOVITCH 4 1 Department of Dermatology, Rush University Medical Center, Chicago, IL, 2 National Skin Centre, 3 Lee Kong Chian School of Medicine, Singapore, and 4 Department of Dermatology and Itch Center, University of Miami Miller School of Medicine, 1600 NW 10 th Ave, Rosenstiel Medical Science Bldg., Miami, FL 33136, USA. E-mail: [email protected] Accepted Jun 14, 2018; Epub ahead of print Jun 25, 2018 10-point numeric rating scale (NRS). He failed to respond to multiple prior treatments including high potency cor- ticosteroids with occlusion, combined topical ketamine 10% and amitriptyline 5%, and UV phototherapy. He experienced minimal relief of pruritus with doxepin 25 mg daily, gabapentin 1,200 mg daily, and use of compounded topical mometasone 0.075%, menthol 2% and pramoxine 2%. Dermatological exam revealed symmetrical, dome- shaped papules coalescing into plaques on his forearms and pretibial surfaces (Fig. 1). Histology revealed col- lections of homogeneous, eosinophilic material with artifactual cracks in the papillary dermis, with plump, stellate-shaped spindle cells. Clinical and histopathologic findings were diagnostic of PLCA. Due to the recalcitrant nature of this patient’s disease, treatment was commenced with methotrexate 15 mg weekly and folic acid. Within 4 weeks, the patient experienced resolution of plaques and papules (Fig. 1), and his pruritus severity decreased from 8 to 3. He tolerated the medication well and did not experience any adverse effects. After 4 months of CASE REPORTS use, the patient felt he no longer needed MTX and chose to discontinue it. He subsequently began to experience Case 1. A 57-year-old Chinese man presented with a recurrence of pruritic papules on his forearms and shins. 30-year history of pruritic papules and plaques on his The patient was restarted on MTX and responded well. forearms and shins. He rated his itch severity as 8 on a Case 2. A 71-year-old Chinese man pre- sented with a 30-year history of pruritic papules on his calves and shins. He rated his itch severity as 10/10. He failed to respond to numerous treatments including systemic antihistamines, high potency topical cor- ticosteroids, intralesional triamcinolone, tacrolimus ointment, topical imiquimod and PUVA. Dermatological exam revealed ex- tensive keratotic brown papules on bilateral shins and calves. Histology showed eosi- nophilic and amorphous amyloid deposits in the upper dermis that were enhanced by Congo Red staining and exhibited apple- green birefringence on polarised microscopy. The stratum corneum was hyperkeratotic and the epidermis showed mild acanthosis with hypergranulosis. These findings were consistent with the diagnosis of PLCA. He was started on methotrexate 10 mg weekly with folic acid. At a 2-month follow-up visit Fig. 1. Dome-shaped papules coalescing into plaques on the left shin at initial presentation which resolved by the 4-week follow-up visit. the patient reported complete resolution of Primary localized cutaneous amyloidosis (PLCA) is cha- racterized by deposition of keratinocyte-derived amyloid within the papillary dermis. PLCA often presents with intensely pruritic, waxy papules that coalesce into pla- ques on the extensor surfaces of the extremities. PLCA is most common in Asians and South Americans, and has been associated with atopic dermatitis (1–3). PLCA poses a therapeutic challenge. Various treatment methods have been employed, but evidence in the form of randomized controlled trials is lacking. Topical therapies for PLCA include high potency steroids, calcipotriene, and menthol (4). Systemic therapies include acitretin, cyclophosphamide and cyclosporine. Additionally, the use of phototherapy and laser therapy has been reported (5). Despite the array of available treatments, PLCA of- ten exhibits limited response, with frequent recurrence. Herein, we describe two cases of PLCA successfully treated with methotrexate (MTX). doi: 10.2340/00015555-2991 Acta Derm Venereol 2018; 98: 900–901 This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2018 Acta Dermato-Venereologica.