{"id":4174,"date":"2026-07-23T17:57:02","date_gmt":"2026-07-23T17:57:02","guid":{"rendered":"https:\/\/edmpackz.com\/?p=4174"},"modified":"2026-07-23T17:57:02","modified_gmt":"2026-07-23T17:57:02","slug":"leukocytoclastic-vasculitis-with-koebner-phenomenon-associated-with-ehlers-danlos-syndrome","status":"publish","type":"post","link":"https:\/\/edmpackz.com\/?p=4174","title":{"rendered":"Leukocytoclastic vasculitis with Koebner phenomenon associated with Ehlers Danlos syndrome \u00a0"},"content":{"rendered":"
\n
\n
<\/div>\n<\/div>\n<\/div>\n
\n
\n

Key words<\/h2>\n
Ehlers Danlos<\/div>\n
Koebner<\/div>\n
leukocytoclastic vasculitis<\/div>\n<\/div>\n
\n

Abbreviation used<\/h2>\n
LCV<\/p>\n
leukocytoclastic vasculitis<\/div>\n<\/div>\n<\/div>\n<\/div>\n
\n
\n
\n

Introduction<\/h2>\n
We present an unusual case of\u00a0leukocytoclastic vasculitis<\/a>\u00a0(LCV) demonstrating the\u00a0Koebner phenomenon<\/a>. A patient with\u00a0Ehlers Danlos syndrome<\/a>\u00a0had\u00a0cutaneous lesions<\/a>\u00a0exclusively on skin of the lower legs that was traumatized by shaving. Biopsy of the lesions found LCV.\u00a0Vascular fragility<\/a>\u00a0caused by Ehlers Danlos may have contributed to Koebnerization after mild trauma from shaving.<\/div>\n<\/section>\n
\n

Case report<\/h2>\n
A 51-year-old woman with a history of\u00a0Ehlers Danlos syndrome<\/a>, hypermobile type (diagnosed by a medical geneticist using the 2017 international classification criteria of generalized\u00a0joint hypermobility<\/a>, systemic manifestations of generalized\u00a0connective tissue disorder<\/a>, positive family history, multiple musculoskeletal complications, and absence of unusual\u00a0skin fragility<\/a>\u00a0or other connective tissue disorders),\u00a0postural orthostatic tachycardia syndrome<\/a>, and\u00a0fibromyalgia<\/a>\u00a0presented to the\u00a0dermatology<\/a>\u00a0clinic for evaluation of an asymptomatic eruption on her lower legs that developed several hours after shaving. This eruption occurred twice in 4\u00a0months, each time preceded by shaving her lower legs. She did not shave her legs between episodes.<\/div>\n
She had fatigue, weight gain, and symptoms of\u00a0Raynaud disease<\/a>. Her medications included\u00a0ivabradine<\/a>, tramadol,\u00a0metaxalone<\/a>,\u00a0levothyroxine<\/a>, and\u00a0ranitidine<\/a>. Her ivabradine dose was increased around the time the rashes began; otherwise, there were no recent medication changes or vaccinations. She denied use of nonsteroidal anti-inflammatory drugs. She denied recent infections, sick contacts, recent travel,\u00a0hematuria<\/a>, abdominal pain,\u00a0photosensitivity<\/a>, or\u00a0oral ulcers<\/a>. She was up to date on age-appropriate\u00a0malignancy<\/a>\u00a0screening. Family history was significant for a sister with Ehlers Danlos syndrome who had biopsy-confirmed vasculitis.<\/div>\n
\n
Physical examination found\u00a0palpable purpura<\/a>\u00a0in a linear configuration on the lower legs (Fig 1<\/span><\/span><\/a>,\u00a0Fig 2<\/span><\/span><\/a>,\u00a0Fig 3<\/span><\/span><\/a>). Biopsy of one of the lesions found a mostly superficial perivascular lymphohistiocytic\u00a0inflammatory infiltrate<\/a>\u00a0with conspicuous nuclear debris, extravasated erythrocytes, and intravascular\u00a0fibrin deposition<\/a>\u00a0(Fig 4<\/span><\/span><\/a>). Periodic acid\u2013Schiff and\u00a0Gram stains<\/a>\u00a0were negative for bacterial and fungal organisms. Direct\u00a0immunofluorescence<\/a>\u00a0showed focal granular C3 deposition within papillary dermal vessels. No IgA deposition was visualized. A diagnosis of\u00a0leukocytoclastic vasculitis<\/a>\u00a0was rendered.<\/div>\n
\"\"<\/p>\n
    \n
  1. Download:\u00a0Download high-res image (181KB)<\/span><\/span><\/span><\/a><\/li>\n
  2. Download:\u00a0Download full-size image<\/span><\/span><\/span><\/a><\/li>\n<\/ol>\n

    Fig 1<\/span>.\u00a0Palpable purpura on lower extremities that developed several hours after shaving the area in November 2018.<\/p>\n<\/figure>\n

    \"\"<\/p>\n
      \n
    1. Download:\u00a0Download high-res image (91KB)<\/span><\/span><\/span><\/a><\/li>\n
    2. Download:\u00a0Download full-size image<\/span><\/span><\/span><\/a><\/li>\n<\/ol>\n

      Fig 2<\/span>.\u00a0Palpable purpura on left lower extremity that developed several hours after shaving the area in February 2019.<\/p>\n<\/figure>\n

      \"\"<\/p>\n
        \n
      1. Download:\u00a0Download high-res image (223KB)<\/span><\/span><\/span><\/a><\/li>\n
      2. Download:\u00a0Download full-size image<\/span><\/span><\/span><\/a><\/li>\n<\/ol>\n

        Fig 3<\/span>.\u00a0Palpable purpura on lower extremities 2\u00a0days after onset in February 2019.<\/p>\n<\/figure>\n

        \"\"<\/p>\n
          \n
        1. Download:\u00a0Download high-res image (145KB)<\/span><\/span><\/span><\/a><\/li>\n
        2. Download:\u00a0Download full-size image<\/span><\/span><\/span><\/a><\/li>\n<\/ol>\n

          Fig 4<\/span>.\u00a0Histopathologic findings. Conspicuous leukocytoclasis, nuclear debris, and intravascular fibrin deposition supports a diagnosis of leukocytoclastic vasculitis. Sparse eosinophils are scattered. Extravasated erythrocytes were seen in other sections. Periodic acid\u2013Schiff and Gram stains (not shown) were negative for bacterial and fungal organisms. (Hematoxylin-eosin stain; original magnification: \u00d74.)<\/p>\n<\/figure>\n<\/div>\n

          Laboratory values were negative or within normal limits for the following: comprehensive metabolic profile, hepatitis B, hepatitis C, HIV,\u00a0Epstein Barr virus<\/a>,\u00a0antinuclear antibody<\/a>, antineutrophil cytoplasmic antibody, anti\u2013Sj\u00f6gren-syndrome\u2013related antigen A\/anti-Ro antibody, anti\u2013Sj\u00f6gren-syndrome\u2013related antigen B\/anti-La antibody, antiribonucleoprotein antibody, anti-Smith antibody, anti\u2013double-stranded DNA antibody,\u00a0complement C3<\/a>,\u00a0serum protein<\/a>\u00a0electrophoresis<\/a>, urine\u00a0protein electrophoresis<\/a>,\u00a0urinalysis<\/a>,\u00a0cryoglobulins<\/a>, thyroid-stimulating hormone, and\u00a0vitamin D.<\/a>\u00a0The following laboratory values were abnormal:\u00a0erythrocyte sedimentation rate<\/a>\u00a0(high, 60\u00a0mm\/h), C-reactive protein (high, 36.4\u00a0mg\/L),\u00a0complement C4<\/a>\u00a0(high, 40.6\u00a0mg\/dL), and total complement (high, 222\u00a0mg\/dL). Complete blood count showed a slightly elevated white blood cell count (11.8\u00a0\u00d7\u00a0109<\/sup>\/L) but otherwise was within normal limits.<\/div>\n<\/section>\n
          \n

          Discussion<\/h2>\n
          Koebner phenomenon<\/a>, also known as\u00a0isomorphic response<\/em>, describes the development of an existing\u00a0dermatosis<\/a>\u00a0in previously normal skin following trauma. This phenomenon is commonly observed in\u00a0psoriasis<\/a>,\u00a0vitiligo<\/a>,\u00a0lichen planus<\/a>, and\u00a0discoid lupus<\/a>.1<\/span><\/span><\/a>,\u00a02<\/span><\/span><\/a>\u00a0The Koebner phenomenon is rarely observed in LCV1<\/sup><\/span><\/span><\/a>\u00a0but has been described at sites of scars, excoriations, and constrictive clothing, most commonly in Henoch-Schonlein purpura.1<\/span><\/span><\/a>,\u00a03<\/span><\/span><\/a>,\u00a04<\/span><\/span><\/a>,\u00a05<\/span><\/span><\/a>,\u00a06<\/span><\/span><\/a>,\u00a07<\/span><\/span><\/a>\u00a0The development of LCV in the pattern of razor strokes after shaving is another demonstration of the Koebner phenomenon.<\/div>\n
          Fragility of blood vessels and perivascular connective tissues, with consequent easy bruising and bleeding, is a feature of all types of Ehlers Danlos.8<\/sup><\/span><\/span><\/a>\u00a0Immune complexes<\/a>\u00a0are more likely to localize in blood vessels that have sustained microvascular injury,9<\/sup><\/span><\/span><\/a>\u00a0as may be caused by razor strokes in individuals with underlying\u00a0vascular fragility<\/a>. Additionally,\u00a0histamine release<\/a>\u00a0triggered by razor strokes may cause\u00a0venous dilatation<\/a>, contributing to stasis and\u00a0immune complex deposition<\/a>.1<\/sup><\/span><\/span><\/a><\/div>\n
          This patient’s LCV was thought to be idiopathic or possibly triggered by the recent increase in her\u00a0ivabradine<\/a>\u00a0dose, given the slightly increased number of\u00a0eosinophils<\/a>\u00a0noted in the biopsy. In light of her family history significant for a sister who also has Ehlers Danlos and biopsy-proven vasculitis, we feel that EDS may predispose individuals to LCV through a mechanism of increased vascular fragility. In this case, vascular fragility in the setting of EDS may have contributed to the Koebnerization of LCV to skin traumatized by razor strokes.<\/div>\n<\/section>\n<\/div>\n
          We acknowledge Dr David H. Chu, MD, PhD, and Dr Elina M. Shustef, DO, of Scripps Clinic Medical Group for performing and interpreting the biopsy, respectively.<\/div>\n<\/div>\n
          <\/div>\n
          \n
          \n

          References<\/h2>\n
          \n