Thursday, April 25th, 9pm EST
Article discussed: Erythema elevatum diutinum a rare and poorly understood
cutaneous vasculitis: A single institution experience
Authors: Luis A. Sardiña, George Jour, Melissa P. Piliang, Wilma F. Bergfeld
Summary prepared by: Juanita Duran, MD; Pathology resident, PGY-3 (@JDuranMD)
Journal Club summary:
- EED is a rare and chronic vasculitis of unknown etiology with variable clinical presentation.
- The initiation of the disease is believed to occur via activation of cytokines, especially interleukin 8.
- Its pathogenesis remains unknown, but a leading theory postulates an immune complex-mediated vasculitis (type III hypersensitivity) as the underlying phenomenom (idiopathic or related to immune diseases).
- Reported associated causes: infections (viral and bacterial), rheumatologic diseases, and hematologic neoplasms.
- Typically presents as persistent red, raised nodules, and plaques distributed symmetrically on acral surfaces.
- Mostly involves extensor surfaces of the extremities as a spectrum of lesions.
- Classic histologic features (although non-specific) are those of leukocytoclastic vasculitis involving the papillary and mid-dermal vessels.
- Aging and more chronic lesions demonstrate onion skin-like fibrosis surrounding the vessels.
- Differential diagnoses: leukocytoclastic vasculitis, granuloma faciale, and Sweet syndrome.
- Retrospective analysis of five cases of EED from a single institution retrieved over a period of 27 years (1989-2016).
- Criteria applied for inclusion/diagnosis:
- Lack of papillary dermal edema and microabscesses with negative direct immunofluorescence (DIF) for Immunoglobulin A deposits arguing against dermatitis herpetiformis.
- Chronic presentation, and lack of fever arguing against an acute Sweet’s neutrophilic dermatosis.
- Lack of small vessel deposits as well as a negative serological workup ruling out etiologies, such as mixed cryoglobulinemia and paraproteinemia.
- Negative direct immunofluorescence except for positive granular vascular deposits of Immunoglobulin M and/or C3.
Salient histopathologic findings are depicted in the image below:
- EED is an uncommon but treatable skin condition
- Various clinical and histologic mimickers exist, histologic ddx includes: bacillary angiomatosis, dermatofibroma, Sweet syndrome, pyoderma gangrenosum, granuloma annulare, granuloma faciale and Kaposi sarcoma.
- Often associated with HIV, Hepatitis B, E.coli and Streptococcal antigens
- In this series, women are more often affected than men.
- One patient was asymptomatic, thus the importance of considering this diagnosis in asymptomatic patients.
- Up to 40% of patients present with joint pain and arthritis.
- Can show overlapping characteristics with granulomatous dermatoses and mixed connective tissue disease.
- Associated to neoplasms (benign and malignant solid tumors)?
- Clinicopathologic correlation is vital to render a diagnosis due to its heterogeneous presentation.
Highlights from the #dermpathJC session:
Thank you so much for attending and for reading this summary. We are so excited to plan yet another journal club for you next month. Stay tuned.