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Secondary cutaneous amyloidosis in disseminated superficial porokeratosis: a case report
1Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.
Abstract:
Disseminated superficial porokeratosis (DSP) is a rare cause of secondary cutaneous amyloidosis. An 83-year-old male patient showed an increase in both size and number of DSP lesions after contracting pulmonary tuberculosis. The DSP lesions of the patient consisted of numerous annular eruptions on both sun-exposed and sun-protected areas, which occurred over a period of 20 years. Multiple skin biopsies were taken from normal or lesional/sun-exposed or sun-protected skin samples. Histopathologic examination included routine H&E stains, Congo red stains, thioflavin-T stains and anticytokeratin antibodies (AE1, AE3). And the results were as follows; 1) Positive staining with Congo red and thioflavin-T indicated an amyloid nature for the deposits, 2) confinement of the amyloid deposition just below the lesional epidermis (while sparing the neighboring uninvolved or distant normal skin) indicated some role of the lesional epidermis, and 3) positive staining with AE3 further indicated an epidermal origin-type II epithelial keratin-of the amyloid. We present a case of DSP with a local amyloid deposit, characterized by association of positive familial background, severe pruritus and pulmonary tuberculosis.
Insights
Disseminated superficial porokeratosis (DSP) can lead to secondary cutaneous amyloidosis. This case highlights DSP
Area of Science:
- Dermatology
- Pathology
- Oncology
Background:
- Disseminated superficial porokeratosis (DSP) is a rare keratinization disorder.
- Secondary cutaneous amyloidosis is an uncommon complication of various dermatoses.
- The relationship between DSP and amyloidosis requires further investigation.
Observation:
- An 83-year-old male with a 20-year history of DSP experienced lesion exacerbation after pulmonary tuberculosis.
- Lesions were widespread, annular, and present on both sun-exposed and sun-protected skin.
- Histopathology revealed amyloid deposits beneath the lesional epidermis.
Findings:
- Congo red and thioflavin-T staining confirmed amyloid nature of deposits.
- Amyloid deposition was localized to the epidermis of DSP lesions.
- AE3 antibody staining indicated an epidermal origin (Type II keratin) for the amyloid.
Implications:
- This case suggests DSP can be a precursor to localized cutaneous amyloidosis.
- Pulmonary tuberculosis may trigger or exacerbate DSP and associated amyloidosis.
- Understanding the epidermal origin of amyloid in DSP could inform future therapeutic strategies.
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