PMC3362225_01
Clinical appearance of the patient at the initial visit in 2006. a Diffuse pigmentation of his face and generalized hypotrichosis. b Hyperplastic and papillomatous changes of the oral cavity. c, d Dirty keratosis of the palms and soles.
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F1
Hyperplastic and papillomatous changes of the lips and oral cavity accompanied by impaired taste sensation were also noted (fig. 1a-d).
Histological findings. a, c Histological features of biopsy specimen from posterior neck (a, magnification x40) and larynx (c, magnification x200). Slight hyperkeratosis and acanthosis were seen undulating with dermal papillomatosis. b Upper endoscopy feature. A diffuse papillomatosis extending to the esophagus, where little normal mucosa was left. Carcinomatous changes such as ulcers or nodules were not observed.
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F2
The biopsy specimens taken from skin of the posterior neck and mucosa of the pharynx and larynx revealed slight hyperkeratosis and acanthosis undulating with dermal papillomatosis (fig. 2a, c). Upper endoscopy showed a diffuse papillomatosis extending from the mucosa of the larynx to the esophagogastric junction, where little normal mucosa was left (fig. 2b).
Papillomatosis of the lip (a, b) and polyp of the larynx (c, d; arrow) before (a, c) and after (b, d) CsA therapy.
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F3
CsA 150 mg (2.5 mg/kg) was initiated, which gradually resolved the papillomatosis of the lip, keratosis of the umbilicus, papillomatous polyp of the larynx, and generalized hypotrichosis (fig. 3a-d). ANA titers decreased from 1/1,280 to 1/160 and anti-DNA antibody from 60.4 to 24 IU/ml. CsA was discontinued after 8 months because new small nodules in the lung, which could have been malignant, were detected by CT.