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Acquired reactive perforating dermatosis (ARPD) is characterized by its onset after the age of 18 years, umbilicated papules or nodules with a central keratotic plug, and the presence of necrotic collagen tissue within an epithelial crater. ARPD is strongly associated with systemic diseases such as diabetes mellitus (DM) and chronic renal failure, which may contribute to ARPD through factors including microcirculatory disturbances and the deposition of metabolic byproducts, including advanced glycation end-products and calcium. Here, we report a case of ARPD that improved following DM treatment and catheter-based interventions for peripheral artery disease (PAD).

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Article Synopsis
  • A 39-year-old woman with Noonan Syndrome and a RAF1 mutation presented with itchy lesions on her limbs that developed two months prior, characterized by multiple dome-shaped papules.
  • Blood tests showed no abnormalities, but a skin biopsy revealed features consistent with Kyrle's disease, a type of perforating dermatosis.
  • The patient was treated with narrowband UVB phototherapy, resulting in complete resolution of symptoms despite leaving residual atrophic scars.
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A Case of Noonan Syndrome and Kyrle Disease: Casualty or Causality?

Acta Dermatovenerol Croat

December 2023

Marco Brusasco, MD, Dermatology Section Department of Medicine and Surgery, University of Parma, Via Gramsci, 14, 43126 Parma, Italy;

Article Synopsis
  • A 39-year-old woman with Noonan Syndrome and RAF1 mutation presented with itchy skin lesions on her limbs, which were diagnosed as Kyrle disease (KD) after a detailed examination.
  • KD is characterized by dome-shaped papules with a central keratotic plug and is often linked to systemic conditions such as chronic renal failure and diabetes, although this patient did not have these issues.
  • Treatment involved narrowband UVB phototherapy, leading to complete resolution of symptoms, which highlights the importance of managing underlying health conditions in skin diseases like KD.
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