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Koebnerization in Henoch-Schönlein purpura: How does it line up with prognosis?


DII small banner By Warren R. Heymann, MD
Nov. 20, 2017


  koebnerization.jpg
Urticarial hemorrhagic lesions of Henoch-Schönlein purpura in characteristic distribution. Note Koebner phenomenon in lines of scratch.
Credit: JAAD
Heinrich Koebner is eponymically known for having first described the phenomenon of developing an existing dermatosis in previously normal skin following external or internal trauma. The prototype of the Koebner phenomenon, (aka isomorphic response) is observed in psoriasis, although it has been described in many dermatoses, including leukocytoclastic vasculitis. (1)

Leukocytoclastic vasculitis (LCV) is a reactive process, caused by the deposition of circulating immune complexes, predominantly in capillaries and post-capillary venules of the superficial plexus, leading to inflammation and necrosis. Drugs, infections, autoimmune diseases, and malignancies may be causes of LCV. Of these, drugs are the most common offending agents. An example of the Koebner phenomenon (KP) in LCV is the case of a 66 year-old woman with vasculitis due to cefuroxime who developed lesions on a skin graft donor site. (2)

The reverse Koebner phenomenon (RKP), defined as the non-appearance or disappearance of lesions has also been described in vasculitis. RKP has only been reported rarely, being first described in patients with psoriasis and vitiligo. In patients with LCV demonstrating the RKP, lesions may not appear under bandages, presumably because the mechanical pressure leads to diminished blood flow in the small vessels of dermis; immune complexes fail to deposit in adequate concentration, and the immune response remains unelicited. (3)

The KP has been reported in children and adults with Henoch-Schönlein purpura (HSP, IgA LCV). Examples include the case of a 5-year-old boy who presented with complaints of a skin rash, arthralgia, and abdominal pain. On examination, he had a palpable purpuric rash mostly involving the extremities. He also displayed linear bands of palpable purpura on the arms and legs (KP), which developed at the site where a thread was tied for religious reasons. Histopathologic examination of skin lesions revealed leukocytoclastic vasculitis with immunoglobulin A deposits on immunofluorescence. (4) Nischal et al presented the case of a 42-year-old male presented with red rash of two days’ duration over both legs, preceded by low-grade fever and mild abdominal pain. The patient wore a pair of new socks that had tight elastic in its upper part. The following day, horizontal strips of red raised bands at the site of pressure from socks’ elastic band, which enlarged into plaques, were observed. A biopsy from the band-like purpuric plaque revealed LCV consistent with the diagnosis of HSP. (5) [I did not see a mention of direct immunofluorescence in this paper].
 
Milani et al examined the prevalence and characteristics of linear nonblanching skin lesions by performing a prospective case series of 31 consecutive children with HSP (3.0 to 12.0 years of age, median age, 6.2 years).  Among the 31 children, 8 (26%) had linear lesions on the legs, groin, waistline, wrists, or forearms. Patients with or without linear lesions did not differ significantly with respect to sex, age, and cutaneous, abdominal, articular, or renal involvement. This study demonstrated that the KP may be more common in patients with HSP than previously recognized. The authors concluding statement was: “Patients with symptoms suggestive of this vasculitis should be evaluated for the presence of nonblanching, palpable lesions with a linear pattern.” (6)
 
Other than advising patients to avoid constrictive clothing or jewelry, I’m not sure why HSP patients need to be evaluated for such lesions. An astute dermatologist will recognize the KP when it occurs. Regardless, Milani et al offer an important contribution to the literature noting that the KP in patients with HSP may be more common than previously appreciated, and that its presence does not appear to influence systemic manifestations or prognosis.

1. De D, et al. Isomorphic response of Koebner in a patient with cutaneous small vessel vasculitis. J Eur Acad Dermatol 2008; 22: 1125-6.
2. Montero I, et al. Cefuroxime-induced cutaneous pustular leukocytoclastic vasculitis with Koebner phenomenon on the donor area of a skin graft. Int J Dermatol. 2015; 54: 1338-9.
3. Yadav S, et al. Reverse Koebner phenomenon in leukocytoclastic vasculitis. Indian J Dermatol 2011; 56: 598-9.
4. Jindal AK, et al. Koebner phenomenon in a young boy with Henoch-Schönlein purpura. J Clin Rheumatol 2017; 23: 290.
5. Nischal KC, et al. Koebner’s phenomenon in Henoch Schoenlein purpura and role of pressure in its causation. Dermatol Online J 2007; 13: 26.
6. Milani GP, et al. Prevalence and characteristics of nonblanching, palpable skin lesions with a linear pattern in children with Henoch-Schönlein syndrome. JAMA Dermatol 2017; 153: 1170-3.

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