Clinical resolution of generalized lichen amyloidosis with dupilumab: a new alternative therapy.

Lichen amyloidosis is a subtype of primary localized cutaneous amyloidosis characterized by deposition of amyloid protein in the skin without visceral involvement. Although it is usually limited to localized areas of the body, it rarely can present in a generalized fashion and is severely pruritic. The limited form is treated with skin directed therapies such as topical or intralesional corticosteroids or topical tacrolimus but the generalized type is more difficult to treat. We present a patient with generalized primary cutaneous lichen amyloidosis successfully treated with dupilumab.

An intensely pruritic pebbled presentation.

Lichen amyloidosis (LA) is a form of primary localized cutaneous amyloidosis (PLCA) characterized by bilateral intensely itchy domed scaly hyperkeratotic papules. Lichen amyloidosis is rare and affects men more than women. It is uncommonly seen in the western world but more prevalent in Asia. These papules most typically affect the shins and occasionally the arms and torso. Lichen amyloidosis has been reported in association with autoimmune disorders and after prolonged exfoliation and friction of affected skin. We present a 40-year-old woman with LA. In LA, the characteristic histological finding is apple-green birefringence of Congo red-stained preparations observed under polarized light. However, this is not always strongly positive, as in our patient. Other findings may include eosinophilia, periodic acid-Schiff positivity, staining with thioflavin T, and metachromasia after staining with crystal violet or methyl violet. Treatment of LA is difficult and complete clinical remission is seldom achieved. Recent trials revealed beneficial outcomes with topical calcipotriol, phototherapy, acitretin, cyclophosphamide, and laser treatments. A combination of acitretin, antihistamines, topical steroids, and hydrocolloid dressings have been beneficial in our patient with LA.

Successful treatment of lichen amyloidosis accompanied by atopic dermatitis by fractional CO2 laser.

Lichen amyloidosis (LA) is a subtype of primary cutaneous amyloidosis that is presented as persistent, multiple-grouped hyperkeratotic papules usually located on the shins, back, forearms or thighs. The treatment of LA has been considered to be difficult, and of the various methods available, recent studies have reported the efficacy of fractional CO2 lasers. LA may be accompanied by atopic dermatitis (AD) in which the treatment options may be more limited. Herein, we report three cases of LA accompanied by AD treated by a fractional CO2 laser.

A Case of Acute Pulmonary Embolism Associated with Dysplasminogenemia

The incidence of pulmonary embolism (PE) rises markedly with age, and only a few cases have been reported in younger adults. Thrombophilia has been reported as one of the predisposing factors for PE in younger adults. Here we report an extraordinary case of PE complicated with dysplasminogenemia, a rare genetic disorder resulting in hypercoagulability, in a young male. An 18-yr-old male visited an emergency room in the United States complaining chest discomfort. He was diagnosed as PE with deep vein thrombosis without apparent risk factors. Anticoagulation therapy with warfarin had been initiated and discontinued after 6 months of treatment. After returning to Korea he was tested for thrombophilia which revealed decreased activity of plasminogen and subsequent analysis of PLG gene showed heterozygous Ala620Thr mutation. He was diagnosed with PE complicated with dysplasminogenemia. Life-long anticoagulation therapy was initiated. He is currently under follow-up without clinical events for 2 yr.