4 results match your criteria: "Elastosis Perforans Serpiginosum"
A Case of Noonan Syndrome and Kyrle's Disease: Coincidence or Causality?
Acta Dermatovenerol Croat
March 2024
Marco Brusasco, MD, University of Parma, Via Gramsci 14, Parma, Italy;
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.
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.
Clinical practice guide for the treatment of perforating dermatosis.
J Dermatol
December 2020
Department of Dermatology, Juntendo University Urayasu Hospital, Urayasu, Japan.
Article Synopsis
- Perforating dermatoses are a diverse group of skin diseases characterized by the skin's expulsion of various materials, including collagen, elastic fibers, and keratin.
- Four main types are identified: acquired reactive perforating collagenosis, elastosis perforans serpiginosa, Kyrle's disease, and perforating folliculitis, each differing in the material eliminated through the skin.
- An updated Japanese treatment guideline for these conditions was created based on a comprehensive review of literature from 1989 to 2019, emphasizing consensus among experts despite the limited scope of existing studies.