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  • Presentation

Microscopic Mystery: Cases from Wisconsin

Description

In a presentation led by Carrie Young, a dermatologist and dermatopathologist, three intriguing cases from Wisconsin were discussed. The first case focused on a six-year-old male with atopic dermatitis exhibiting a progressively worsening, pruritic rash, which led to a diagnosis of hyperkeratotic flexural erythema, historically related to the use of deodorants but currently viewed as a keratinization disorder possibly linked to irritants like benzylchonium chloride. The second case involved a 47-year-old female experiencing painful perianal lesions initially suspected to be herpes, but subsequent biopsies and imaging indicated acute aortic occlusion due to chronic aortoiliac occlusive disease. Despite ongoing treatment, her condition advanced significantly, necessitating surgical intervention. Lastly, the third case presented a 76-year-old male with an enlarging lower leg nodule, initially thought to be diffuse large B-cell lymphoma but identified as mantle cell lymphoma upon further histological examination. This rare condition sometimes manifests in the skin, highlighting its aggressive nature and diagnostic complexity amid similarities to other lymphomas. The cases emphasized challenges in diagnosis, the importance of thorough histological evaluation, and considerations of underlying conditions in dermatological presentations.

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Conclusions

  • Hyperkeratotic flexural erythema, previously termed axillary granular parakeratosis, is more accurately classified as a disorder of keratinization.
  • The diagnosis of hyperkeratotic flexural erythema typically shows no significant underlying inflammation but a thickened stratum corneum with granular keratinocytes.
  • Histologically, it may present distinctly, showing retained keratohyalin granules without notable changes in the dermis.
  • Possible triggers for hyperkeratotic flexural erythema include deodorant use, occlusion, and exposure to benzalkonium chloride from household products.
  • The prevalence of hyperkeratotic flexural erythema can occur in other body areas, not just the axilla, prompting a broader term for its classification.
  • Acute aortic occlusion, identified in a patient with a history of chronic aortoiliac disease, can present with atypical symptoms, complicating the diagnosis of painful ulcerations.
  • In cases of suspected acute aortic occlusion, identifying collateral circulation may aid in understanding unusual presentations of ischemia.
  • Cutaneous involvement by mantle cell lymphoma, while rare, often indicates systemic disease and can appear similar histologically to diffuse large B-cell lymphoma.
  • Molecular markers such as CD5, Cyclin D1, and SOX11 are important for accurately distinguishing between different types of lymphomas in diagnostic settings.
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  • WHO Classification of Tumors online, Skin Tumors (5th ed)
  • Kim et al. Am J Surg Pathol. 2019, 43 (10).
  • Wehkamp et al. Am J Surg Pathol 2015;39:1093-1101.