Summary. We report a case of systemic phaeohyphomycosis due to Exophiala dermatitidis in a 24‐year‐old man. At the age of 17, the patient had noticed cervical swellings. On palpation at his initial examination, more than a dozen firm lymph nodes between 1 and 5 cm in diameter were found on both sides of the neck and in both axillae. Examination of biopsy specimens of lymph nodes revealed pale brown hyphae in granulomatous lesions and Exophiala dermatitidis was isolated from mycological cultures of the local tissues. The clinical course was marked by an episode of jaundice six months later, and surgery disclosed a fist‐sized inflammatory mass in the region of the head of the pancreas and the duodenum. The patient was treated by providing external biliary drainage and by the administration of antifungal agents, but two years later complained of diplopia and a slight heaviness in the head. Computed tomographic scans were made of the head, revealing multiple plum‐sized masses in the brain. The patient died a year later. In Japan, 10 cases of systemic infection by this organism (including the present one) have been reported, all in patients of up to 30 years of age, with lesions appearing in the brain, lung, liver, digestive organs and lymph nodes. The prognosis is grave. Zusammenfassung. Unser Bericht beschreibt einen Fall von systemischer Phäohyphomykose, verursacht durch Exophiala dermatitidis, bei einem 24‐jährigen Mann. Im Alter von 17 Jahren hatte dieser Patient Schwellungen am Hals festgestellt. Bei der Palpation während der ersten Unter‐suchung wurde uber ein Dutzend fester Lymph‐knoten (1–5 cm Durchmesser) an beiden Seiten des Halses und in beiden Achselhöhlen gefunden. Die Gewebsuntersuchung eines Lymphknotens zeigte hellbraune Hyphen in einer granulomatö‐sen Verletzung, und Exophiala dermatitidis wurde von einer mykologischen Kultur des lokalen Gewebes isoliert. Der klinische Verlauf war durch das Auftreten von Gelbsucht sechs Monate später gekennzeichnet, und bei einer Operation wurde eine faustgroße entzündete Masse im Bereich des Pankreaskopfes und des Zwälffingerdarms festgestellt. Der Patient wurde mit einer externen Gallengangdrainage versehen und mit Antimyko‐tika behandelt. Aber zwei Jahre später klagte er über Doppeltsehen und ein Schweregefuhl im Kopf. Der Kopf wurde mittels Computertomo‐graphie untersucht, und dabei wurden im Gehirn mehrere pflaumengroße Massen festgestellt. Der Patient starb ein Jahr spater. In Japan wurden bisher über 10 Fälle von systemischer Infektion durch diesen Organismus (den hier beschriebenen Fall eingeschlossen) berichtet. Alle traten bis zum Alter von 30 Jahren auf und waren gekenn‐zeichnet durch Läsionen im Gehirn, in Lunge, Leber, Verdauungsorganen und Lymphknoten. Die Prognose ist ernst.

We report four patients with pseudoxanthoma elasticum-like papillary dermal elastolysis (PDE). Multiple small papules on the neck, clinically resembling pseudoxanthoma elasticum, and loss of the elastic fibre network in the papillary dermis were found in each case. Immunohistochemical staining for elastin and fibrillin-1 in one patient demonstrated the disappearance of elastin and fibrillin-1 in the papillary dermis. Site-matched normal skins of the elderly showed intact elastin but a lack of fibrillin-1 in the papillary dermis. The younger normal skins revealed intact elastin and fibrillin-1. The results suggest that fibrillin-1 is absent from the papillary dermis of the normal-appearing neck skin of the elderly and that the primary defect in PDE may be in elastin rather than in fibrillin-1.

Actinic elastosis is characterized by an accumulation of elastotic material in the upper dermis and is considered to be a manifestation of ultraviolet-induced skin aging. To compare the structural components of the elastotic material in actinic elastosis with those in normal skin, skin specimens were stained with antibodies raised against various elastin-related proteins. Elastotic materials exhibited a strong reaction to the antibodies for elastin, microfibril-associated glycoprotein-1 (MAGP-1), MAGP-4, matrix metalloproteinase 1 (MMP-1), MMP-2 and MMP-3, but a diminished reaction to anti-MMP-9 antibody. Fibroblast cell lines from the upper dermis of affected and unaffected skin were established, and the mRNA levels of MMPs were determined. MMP-1 and -2 mRNA levels were found to be elevated approximately twofold in the fibroblasts from actinic elastosis. Since MMP-1 and -2 are considered to be major enzymes involved in the degradation of matrix components, the accumulation of elastotic materials in actinic elastosis may be related to the degradation process.

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