A skin biopsy specimen was obtained from

A skin biopsy specimen was obtained from her neck. The histopathologic evaluation revealed that mildly hyperkeratotic epidermis and amorphous eosinophilic masses were deposited in the papillary GSK503 (Figure 2). There were no alternations in the reticular dermis. Congo red staining was positive for eosinophilic materials (Figure 3).
Diagnosis
Discussion Primary cutaneous amyloidosis (PCA), a pruritic skin disorder, is the deposition of amyloid in the papillary dermis without any systemic involvement. It is more commonly seen in South America and Southeast Asia compared with North America and Europe. It is also more common in Chinese compared with Malays or Indians. The differential diagnoses include dyschromatosis universalis hereditaria, xeroderma pigmentosum, amyloidosis cutis dyschromica (ACD), and poikilodermatous amyloidosis. The deposition of amyloid in the papillary dermis is not observed in the first two diseases. ACD, which was described first by Morishima in 1970, is characterized by the following features: (1) stippled, reticular hyperpigmentation with hypopigmented spots without papulation almost over the whole body, (2) no or little itch, (3) onset before puberty, and (4) small foci of amyloids close beneath the epidermis. Familial ACD has been reported in Taiwan. Our case presented with mottled skin lesions, like ACD, but they were limited to the face and neck, and onset occurred after puberty. Poikilodermatous amyloidosis is another rare type of PCA and is associated with short stature, blisters, photosensitivity, and palmoplantar keratoderma. The present case was of normal stature, and we did not observe any dermatologic abnormality except for the hyperpigmentation on her neck and face. The main treatment strategy is to reduce the irritation and friction of the skin. Sedative antihistamine and topical steroids are beneficial for pruritus relief. Administration of retinoid may also be effective for both the pruritus and hyperkeratotic papules. In addition, 532-nm and 1064-nm Q-switched Nd:YAG laser therapy may reduce pigmentation in macular amyloidosis patches. The patient received oral isotretinoin for 5 months, but neither obvious clinical improvement nor progression of the pigmentation was observed.
Case report A 46-year-old man was admitted to a community hospital with fever and rapidly progressing, painful, ulcerative skin lesions. He was transferred to our hospital for further examination. According to his medical history, he had been diagnosed with psoriasis, but this was not confirmed by a skin biopsy. The generalized pruritic plaques on his trunk and extremities had waxed and waned for several years but had exacerbated during the previous year. Skin examination on admission showed diffuse redness with periorbital, paranasal, and perioral sparing with multiple coalescent annular plaques on the trunk and extremities. Some of these lesions were indurated, others were crusted. Ulcerative plaques were present on the dorsum of the patient\'s right hand and soles (Figures 1A and 2A). A few inguinal lymph nodes were palpable on each side. The histopathological examination of specimens obtained from his left elbow, left thigh, and an inguinal lymph node revealed dense infiltrations of atypical medium-sized lymphocytes with nuclear pleomorphism. Eosinophils were identified in the dermis (Figure 3) of the specimens from the elbow and thigh and in the perifollicular zones of the lymph node. Immunohistochemical staining showed that these infiltrates were positive for CD2, CD3, CD4, CD5, CD8, and CD30 and negative for CD10, CD20, CD21, CD23, Epithelial Membrane Antigen (EMA), and Anaplastic Lymphoma Kinase (ALK). In situ hybridization for Epstein-Barr virus-encoded RNA (EBER) produced negative findings. Other significant laboratory data included the following: white cell count, 23,410 cells/μL (normal: 3500–9100/μL); band form, 35% (normal: 0–3%); total Immunoglobulin E (IgE), 574 IU/mL (normal for adults: <87 IU/mL); LDH, 461 U/L (normal: 98–192 U/L); ferritin, 387.3 ng/mL (normal for men: 23.9–336.2 ng/mL). The results of the bone marrow biopsy were normal. Abdominal computed tomography (CT) and bone scan revealed the lack of involvement in the internal organs. Human T cell lymphotropic virus I (HTLV)-related lesions were suspected; therefore, serological tests were performed. Enzyme-linked immunosorbent assay (ELISA) screening test revealed positivity to anti-HTLV I/II, and reactivity to HTLV type I (HTLV-I) was confirmed by Western blot analysis.