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  • Presentation

Diagnostic Dilemmas in Dermatopathology: Three Challenging Cases

Description

The presentation highlighted three challenging dermatopathology cases that illustrated how difficult it can be to reach a specific diagnosis when clinical and histologic findings overlap. The first case was a 64-year-old man with chronic erythroderma and severe pruritus whose repeated biopsies initially showed only spongiotic and psoriasiform spongiotic dermatitis, raising concern for cutaneous T-cell lymphoma. After multiple biopsies, CD30 positivity, atypical epidermotropic lymphocytes, and limited blood flow abnormalities supported erythrodermic mycosis fungoides with large-cell transformation, and he improved markedly on extracorporeal photopheresis. The speaker emphasized that CTCL may require repeated biopsies, can mimic eczema or drug eruption, and that CD30 positivity has a broad differential. The second case involved a 68-year-old woman with fever, severe arthralgias, weight loss, and pink-to-violet lesions over joints. Biopsies showed palisaded granulomatous dermatitis with leukocytoclastic vasculitis and prominent neutrophils, leading to consideration of atypical Sweet syndrome, adult-onset Still disease, and ultimately reactive granulomatous dermatitis/palisaded neutrophilic granulomatous dermatitis. The discussion focused on its systemic associations with autoimmune disease, malignancy, and infection, and the need for broad workup and follow-up. The third case was a man with aggressive chronic lymphocytic leukemia who developed recurrent pruritic nodules, bullae, and episodic morbilliform eruptions. Biopsies showed eosinophilic spongiosis, subepidermal blistering, dermal eosinophilia, and possible flame figures, with negative direct immunofluorescence, favoring eosinophilic dermatosis of hematologic malignancy over bullous pemphigoid, arthropod bite reaction, or Wells syndrome. Overall, the talk stressed diagnostic uncertainty, the value of repeat sampling, and the importance of correlating pathology with clinical context.

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Conclusions

  • Chronic erythroderma with nonspecific spongiotic biopsies may still represent cutaneous T-cell lymphoma, and repeated biopsies plus clinicopathologic correlation are often necessary to reach the diagnosis.
  • Dupilumab can transiently worsen or unmask underlying cutaneous T-cell lymphoma, so apparent eczematous disease should be reconsidered when the course is atypical.
  • CD30 positivity is not specific for a single entity and can be seen in transformed mycosis fungoides, Sézary syndrome, lymphomatoid papulosis, primary cutaneous anaplastic large cell lymphoma, and even reactive conditions.
  • Pink-violet plaques overlying joints with granulomatous inflammation and leukocytoclastic vasculitis can fit reactive granulomatous dermatitis, which requires evaluation for autoimmune disease, infection, or malignancy.
  • Reactive granulomatous dermatitis can clinically and histologically overlap with granuloma annulare and may precede an underlying systemic diagnosis.
  • Recurrent tense bullae with eosinophilic spongiosis in a patient with CLL strongly suggest eosinophilic dermatosis of hematologic malignancy, though bullous pemphigoid and arthropod-related reactions remain in the differential.
  • Eosinophilic dermatosis of hematologic malignancy can present with a wide range of morphologies, from cellulitic and urticarial lesions to vesicles and bullae, and is most often associated with CLL.
  • Overall, these cases emphasize that subtle or mixed dermatopathology findings often require serial biopsies, broad differential diagnosis, and ongoing follow-up to uncover the true systemic or neoplastic process.
  • CD30 in Cutaneous Pathology. American Journal of Dermatopathology. PMID and DOI shown on slide.#10.1097/dad.0000000000002422
  • Brentuximab Vedotin for Relapsed or Refractory Sézary Syndrome. JAMA Dermatology. PMID, PMCID, and DOI shown on slide.#10.1001/jamadermatol.2020.4901
  • Underlying Systemic Diseases in Interstitial Granulomatous Dermatitis and Palisaded Neutrophilic Granulomatous Dermatitis: A Systematic Review.#10.1159/000527461
  • Case reports showing dupilumab efficacy (referenced on slide about eosinophilic dermatosis of hematologic malignancy).