ISSN:
1365-4632
Source:
Blackwell Publishing Journal Backfiles 1879-2005
Topics:
Medicine
Notes:
A 28-year-old woman was seen for pruritic lesions on both hands and wrists which had been present since the age of 10 years. Both palms showed symmetric, diffuse hyperkeratosis extending over both wrists and the dorsal aspects of both hands with well-demarcated, erythematous, lichenified plaques (〈link href="#f1 #f2"〉Figs 1 and 2). The borders of the plaques were pigmented and studded with papules (〈link href="#f2"〉Fig. 2). The skin lesions were not related to sun exposure. No similar lesions were found elsewhere on the body. The hair, nails, and dental development were normal. A skin biopsy was taken for histopathologic and ultrastructural studies under the clinical impression of pityriasis rubra pilaris.〈figure xml:id="f1"〉1〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2031:IJD_2031_f1"/〉Hyperkeratosis of both palms and well-demarcated, erythematous, lichenified plaques on the wrists〈figure xml:id="f2"〉2〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2031:IJD_2031_f2"/〉The borders of the plaques are pigmented with a few papulesMicroscopically, homogeneous perivascular deposits of hyaline-like material were seen in the papillary and reticular dermis, accompanied by mononuclear cells (〈link href="#f3 #f4"〉Figs 3 and 4). The overlying epidermis was acanthotic with hyperkeratosis (〈link href="#f3"〉Fig. 3). The hyaline deposits were positive for periodic acid–Schiff (PAS) and oil-red O stains (〈link href="#f5 #f6"〉Figs 5 and 6). Congo red stain was negative. Electron microscopic examination showed abundant amorphous material intermingled with short collagen fibrils in the dermis and around the blood vessels (〈link href="#f7 #f8"〉Figs 7 and 8).〈figure xml:id="f3"〉3〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2031:IJD_2031_f3"/〉The dermal vessels are surrounded by hyalinized material and mononuclear cells. The overlying epidermis is acanthotic (hematoxylin and eosin; original magnification, × 100)〈figure xml:id="f4"〉4〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2031:IJD_2031_f4"/〉Higher magnification to show the homogeneous perivascular deposits of hyaline-like material, accompanied by mononuclear cells (hematoxylin and eosin; original magnification, × 250)〈figure xml:id="f5"〉5〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2031:IJD_2031_f5"/〉The hyaline deposits are positive for periodic acid–Schiff stain (original magnification, × 250)〈figure xml:id="f6"〉6〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2031:IJD_2031_f6"/〉The hyaline deposits also stain with oil-red O stain (original magnification, × 100)〈figure xml:id="f7"〉7〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2031:IJD_2031_f7"/〉Ultrastructurally, abundant amorphous deposits are intermingled with short collagen fibrils in the dermis (original magnification, × 4000)〈figure xml:id="f8"〉8〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2031:IJD_2031_f8"/〉A blood vessel is surrounded by concentric rings of amorphous material (original magnification, × 4000)Further examination revealed that the patient could protrude her tongue freely. The oral mucosa and vocal cords were smooth. There was no sign of systemic amyloidosis. The patient's complete blood cell count, biochemical tests, and protein electrophoresis were all within normal limits. Antinuclear antibody was negative. Results of porphyrin screening tests were negative for uroporphyrins and coproporphyrins in the urine and feces. The possibility of porphyria was therefore excluded. Both neurologic and ophthalmic examinations were negative. No intracranial calcification was found on a skull X-ray examination. The patient was treated with topical steroid and urea. She was lost to follow-up.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1111/j.1365-4632.2005.02031.x
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