A 60-year-old female presented with a 10-year history of pruritus on the chin. She did not have a family history of PXE. Her past medical history was unremarkable. The physical examination re­vealed no skin lesions, but she complained of a severe itching sensation on the chin of more than 10 years duration (Fig. 1). A punch biopsy specimen from the chin showed short, irregularly thickened, basophilic fibers in the mid-dermis (Fig. 2A). Accumulations of swollen and irregularly clumped fibers stained deep black with Verhoeffs stain (Fig. 2B). …Read the rest of this article

INTRODUCTION

Pseudoxanthoma elasticum (PXE) is a rare, in­herited, multisystemic disorder. It is characterized by progressive calcification and degeneration of elastic fibers resulting in a diagnostic pathologic picture referred to as “elastorrhexia”. Skin lesions almost always begin on the lateral portion of the neck during early childhood and remain confined to the flexural area, except in the most advanced cases, in which the trunk, face, and extremities may be involved. However, mild forms of the disorder can easily be overlooked. Skin biopsy remains the best way to diagnose PXE. To date, the majority of re­ports have focused on the typical clinical symptoms of PXE, the associated systemic abnormalities of PXE, or perforating PXE. Localized, acquired cu­taneous PXE has been proposed as a non-heritable form that is nearly identical to the heritable form. Recently, a previously undescribed cutaneous finding (mental creases) in a patient with PXE was re- ported. …Read the rest of this article

AGEP is an acute pustular eruption characterized by numerous, non-follicular pustules arising on a widespread erythematous base, which usually ap-pears first in the intertriginous areas and then spreads to other body sites. Neutrophilia and high fever are also usually present. The pustules resolve spontaneously within a few days and are followed by pinpoint desquamation. Histologically, AGEP is characterized by spongiform intraepidermal or sub- corneal pustules, dermal edema, vasculitis, periva- scular neutrophils or eosinophils, and necrotic kera- tinocytes.

AGEP is induced by systemic drugs in over 90% of cases, mainly в-lactam and macrolide anti-microbials. Calcium channel blockers, anti-malarials, and terbinafine have also been reported to cause AGEP. In addition, viral infections, mercury ex¬posure, Ginkgo biloba, and spider bites may serve as occasional causes.

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A 40-year-old man was admitted to our depart­ment with a pruritic rash of four days duration. Six days before, he had ingested lacquer chicken as a health food. Physical examination demonstrated marked generalized, symmetrical erythema with multiple overlying small pustules on the trunk, medial arms, and proximal legs (Fig. 1). The patient also had a fever and complained of severe itching and burning. He had ingested lacquer chicken a couple of times in the past, but this was the first time he had developed a skin eruption. He reported no history of psoriasis, drug ingestion, recent infection, or contact with mercury.

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INTRODUCTION

In 1980, Beylot et al first described acute gen­eralized exanthematous pustulosis (AGEP) as an acute pustular eruption on an erythematous bed, accompanied by fever and neutrophilia. A wide range of systemic drugs have been suspected of causing these reactions in more than 90% of cases. Rarely, intake of lacquer chicken can be a cause of AGEP, and Park et al reported four such cases. …Read the rest of this article

Although the mechanism linking immunosuppres- sion and PK remains unclear, there is growing evidence suggests that immunosuppression may give rise to a loss of immunosurveillance, which allows the proliferation of abnormal keratinocyte clones in PK lesions. This suggestion is supported by im- munohistochemical studies that revealed defective expressions of HLA-DR antigens by epidermal Langerhans cells in the lesions of PK. If this is the case, this could lead to the uninhibited expansion of mutant clones of epidermal keratinocytes, which corresponds with the clinical development of PK.

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Table 1 summarizes the clinical characteristics of the 9 renal transplant recipients with PK. At dermatological examinations, patient ages ranged from 38 to 67 years (mean age: 52 years). There were eight men and one woman, and no patient had previous or family history of PK. All patients had received renal allograft transplantation because of chronic renal failure. After renal transplantation, they received multi-drug regimens comprised of two or three immunosuppressive agents, namely, steroids, cyclosporine, azathioprine, mycophenolate mofetil, and/or tacrolimus. History taking revealed that all lesions developed after transplantation. Times between transplantation and lesion onset ranged from 2 to 9 years (mean: 4.1 years). The most commonly associated skin disease was verruca (4), and the others were recurrent herpes simplex infection (1), actinic keratosis (1), and cutaneous B cell lympho- ma (1).

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