A case of generalized lichen amyloidosis treated with dupilumab: the first case reported in China / 中华皮肤科杂志

To report a case of generalized lichen amyloidosis successfully treated with dupilumab in China. A 70-year-old male patient presented with extensive itchy papules on the trunk and extremities for 23 years. Skin examination revealed diffuse millet-to mung bean-sized hemispherical brown plaques on the trunk, right anterior shank, and extensor aspect of both upper arms, with a hard texture on palpation. No abnormalities were observed in the blood eosinophil count or serum IgE level. Histopathological examination of the skin lesion on the lower limb showed epidermal hyperkeratosis and homogeneous red-stained lumpy materials in the papillary dermis. Immunohistochemical study showed positive staining with Congo red. The pruritus numerical rating scale score was 10 points. The diagnosis of generalized lichen amyloidosis was confirmed. The patient received subcutaneous injection of dupilumab at an initial dose of 600 mg, followed by an every-2-week regimen at a dose of 300 mg. At week 2 after the start of treatment, pruritus was markedly relieved; at week 14, the skin lesions began to subside markedly; at week 18, the skin lesions on the chest and abdomen nearly completely subsided, and lesions on the lower back and limbs markedly regressed. No obvious adverse reactions were observed.

Epidermolysis bullosa pruriginosa: A case with a novel mutation and co-existent lichen amyloidosus.

Epidermolysis bullosa pruriginosa is a rare variant of dystrophic epidermolysis bullosa characterized by severely pruritic and cicatricial lesions localized to the extensor extremities. We report a Singaporean Chinese male with epidermolysis bullosa pruriginosa with an underlying novel mutation in the COL7A1 gene. A heterozygous acceptor splice site mutation IVS67‑1G>T probably led to in‑frame skipping of exon 68 (36‑basepairs), resulting in a loss of 12 amino acids. Among his three children, only the youngest son, who had bilateral big toenail thickening, possessed the same mutation. His skin biopsy one decade ago revealed association of focal amyloidosis; a recent skin biopsy showed more established features of lichen amyloidosis. It is debatable whether the cutaneous amyloidosis was a secondary or primary phenomenon. Our report highlights that the diagnosis of epidermolysis bullosa pruriginosa may be obscured when cutaneous amyloidosis is coexistent.

A Case of Lichen Amyloidosis on the Auricular Concha and a Review of the Literature / 대한피부과학회지

Lichen amyloidosis is composed of firm, yellowish to brownish papules, usually found on the pretibial area. Amyloidosis of the auricular concha is a rare variant of primary cutaneous amyloidosis, presenting papules or plaques localized to the ear. Histopathologically, it is characterized by hyperkeratosis, acanthosis and deposition of eosinophilic materials in the papillary dermis similar to the presentation of lichen amyloidosis. Herein, We report a case of lichen amyloidosis occurring on the auricular concha of a 48-year-old Korean woman, presented with a 3-years history of asymptomatic monomorphous papules on her both ears.

A Case of Lichen Amyloidosis Improved by Topical Salicylic Acid and Topical Corticosteroid / 대한피부과학회지

Lichen amyloidosis (LA) is a chronic pruritic skin disorder characterized by the deposition of amyloid in the papillary dermis. A variety of therapeutic regimens for LA have been described. However, many of these have been shown to have limited effect. We report the successful, long-lasting resolution of LA and marked improvement of pruritus in one patient who was treated with the combination of topical corticosteroid and salicylic acid 25% ointment. We suggest this combination therapy as an efficacious treatment for LA.

A Case of Membranous Lipodystrophy Observed in Lichen Amyloidosis

Membranous lipodystrophy is characterized by the presence of microcysts lined by amorphous, eosinophilic material with an arabesque appearance. We experienced a case of a 72-year-old man who had dark brownish, pruritic papules on the arms, legs, and back. Histopathologic examination of a biopsied lesion showed homogeneous, eosinophilic material in the papillary dermis, as well as membranous lipodystrophy. We report a case of membranous lipodystrophy observed in lichen amyloidosis.

A Case of Lichen Amyloidosis / 대한피부과학회지

The primary cutaneous amyloidoses are divided into lichen, macular and nodular amyloidosis. Lichen amyloidosis is a chronic pruritic skin disorder characterized by deposition of amyloid material in previously normal skin, without evidence of visceral involvement, and presents as small, discrete, brownish gray papules. Prolonged friction, genetics, viral infection and the environment have been proposed as factors inducing lichen amyloidosis, but the precise pathogenesis of lichen amyloidosis is yet to be determined. We report herein a lichen amyloidosis occurring on the back of a 26-year-old woman who presented with 10-years history of chronic pruritic skin lesion.

Two Cases of Lichen Amyloidosis Associated With Atopic Dermatitis / 대한피부과학회지

Lichen amyloidosis is a chronic intensely pruritic skin disorder characterized histologi-cally by the deposition of amyloid in the papillary dermis. Lichen amyloidosis has been reported in association with several pruritic skin disorders including atopic dermatitis, lichen planus, and mycosis fungoides. It is hypothesized that the scratching in pruritic skin diseases is the most important step in forming amyloid deposit. We report two cases of lichen amyloidosis occuring in two patients with atopic dermatitis.

Two Cases of Lichen Amyloidosis Stained Negatively for Antikeratin Antibodies / 대한피부과학회지

Localized primary cutaneous amyloidosis consists of three types: nodular, macular, and lichenoid amyloidosis. Lichen amyloidosis is characterized by the appearance of paroxysmally itchy lichenoid papules on the shins. Although the origin of the amyloid is controversial and can not provide any definite answer, some of amyloid substances can be derived from epidermal cells and others from degenerative collagen fibers. We suggested the origin of amyloid in our cases as epidermal keratinocyte and collagen degenerations. Because degeneration of basal keratinocyte is observed on electron microscopy and degeneration of collagen is in H&E and Masson's trichrome stain although amyloid materials are negatively stained against pankeratin antibody and collagen antibody. The skin lesion was improved after triamcinolone intralesional injections which reduce collagen synthesis. We report two cases of lichen amyloidosis which stained negatively for antikeratin antibodies and improved with triamcinolone intralesional injection.

A Case of Lichen Amyloidosis Associated with Pdoriasis / 대한피부과학회지

Psoriasis is merely recognized as a disorder associated with pruritus. But in some cases, psoriasis does induce pruritus which could alter the daily lives of the people affected. Pruritus in psoriasis has been reported to be influenced by various external and internal factors and as a consequence, aggravation of psoriasis is possible by scratching due to the well-known Koebners phenomenen. Lichen amyloidosis, on the other hand, appears as multiple papules with intense pruritus. Recently, there are some evidence emphasizing pruritus as a main factor in forming amyloid deposits. We report a case of 65-year-old lady who was suffering psoriasis for 25 years with associated pruritic papular lesions on both of her thighs, knees and ankles. The diagnosis of Lichen amyloidosis was confirmed by Periodic Acid Schiff and Dylon stain. We discuss the role of pruritus in lichen amyloidosis and psoriasis and speculate that pruritus induced by lichen amyloidosis may induce a vicious cycle in aggravating psoriasis.