A 26-year-old male presented with salmon colored annular lesions extending from the dorsum of his feet proximal into his lower extremities bilaterally. He originally thought he had eczema.
The patient had no pertinent past medical history or allergies, and no current medications. The patient had not traveled recently and had no recent history of infection. The lesions were mildly pruritic and he had not changed any of his detergents, soaps or anything else in his environment. He did not relate this rash as appearing after any excessive sun exposure recently. He denied fever, chills and joint pain.
The exam revealed reddish, pink-colored annular plaques that varied in size and began at the dorsum of the foot and continuing up the leg below the knee. The lesions did not blanch upon pressure. The lesions were not scaly. No lesions were present on the trunk or the back. There were no vesicles, target lesions or ulcers. Toenails and fingernails showed no signs of dystrophy. His hallux lateral nail border was erythematous and edematous, but this paronychia began months earlier than the current skin rash. There were no scalp lesions. We obtained a biopsy of one of the newest presenting lesions.
1. What is the most likely diagnosis?
2. What is your differential diagnosis?
3. What are the characteristic lesions in this disease?
4. What is the treatment?
1. The most likely diagnosis is a form of cutaneous small vessel vasculitis, which is also known as cutaneous leukocytoclastic vasculitis (LCV).
2. Differential diagnoses include: systemic lupus erythematosus, drug reaction, Henoch-Schönlein purpura, purpura from other hemorrhagic causes (thrombocytopenia) and other vessel-based inflammatory conditions such as Sweet’s syndrome and urticarial vasculitis.
3. The characteristic lesion in this disease is classically described as palpable purpura that range in size from a few millimeters to a few centimeters. This lesion does not blanch upon pressure. These lesions usually begin on the lower extremity or on areas of dependency. There might be constitutional signs and symptoms (fever, myalgia, arthralgia) that accompany the local symptoms of pruritus and burning, but generally the skin is solely involved. Urticarial lesions that last greater than 24 hours may also be present.
4. Treatment varies from oral nonsteroidal anti-inflammatory drugs (NSAIDs), systemic steroids and oral antihistamines depending on the severity of the presentation. If one identifies a drug reaction, connective tissue disorder or infection as the basis for this, then consider treatment of the triggering cause or discontinuation of the drug.
This patient has cutaneous leukocytoclastic vasculitis. Biopsy results showed neutrophilic perivascular infiltrate and extravasation of red blood cells. The LCV can be associated with drug reactions, infections, malignancies and systemic inflammatory conditions. However, LCV can present idiopathically with no correlation to any systemic disorder.1 This presentation is generally termed as primary cutaneous small vessel vasculitis (PCSVV) and has no extracutaneous involvement.2
The most common cutaneous presentation begins as a crop of palpable purpura or non-blanchable lesions.3 It primarily occurs on the “dependent” areas of the body such as feet, legs and buttocks. The initial crop of lesions may resolve in a month with residual pigmentation. However, while present on the skin, they may cause pruritus, burning and stinging. The lesions can be exacerbated by sun exposure, continued pressure on the affected areas and exercise.4 Otherwise, the prognosis is good since primary cutaneous small vessel vasculitis generally is a single episode.
In addition to the skin involvement, constitutional symptoms may be present but generally primary cutaneous small vessel vasculitis does not extend beyond the skin. If further extracutaneous involvement is visible, one should consider systemic and/or connective tissue disease. Even though a biopsy in this case suggested cutaneous leukocytoclastic vasculitis, primary cutaneous small vessel vasculitis is generally a diagnosis of exclusion. It is important to perform a detailed history and physical exam, and rule out various systemic and connective tissue disorders that may present similarly clinically.
Systemic lupus erythematosus and other connective tissue disorders. The annular nature of these lesions occurring in a conceivably sun exposed area (lower legs) allows for systemic lupus erythematosus as a differential diagnosis. If the classic characteristic butterfly facial rash or atrophic scarring occurs following the initial skin rash on the sun exposed areas, one should strongly consider systemic lupus erythematosus and discoid lupus erythematosus, and conduct a skin biopsy. Also complete a thorough history and physical.
Drug reaction. Drug reactions may present with palpable purpura and target-like lesions. Fixed drug eruptions in which the skin reaction occurs in the same anatomical area that receives the drug may also cause annular pigmented lesions.
Henoch-Schönlein purpura. Typically occurring in children and young adults, Henoch-Schönlein is characterized by palpable purpura, arthralgia and abdominal pain.5 It commonly occurs on the buttocks and the lower extremities where primary cutaneous small vessel vasculitis is generally found.
The skin biopsy in this case supported the clinical diagnosis and the history and physical exam helped to rule out connective tissue disease. The patient did not undergo a separate biopsy for direct immunofluorescence evaluation at this time. The patient also underwent further testing, such as complete blood count (CBC) with differential, erythrocyte sedimentation rate (ESR), urinalysis, liver function tests and hepatitis serology.
The aforementioned patient underwent treatment with systemic corticosteroids (a long prednisone taper) and is still undergoing treatment for this condition. However, if the aforementioned patient had an underlying systemic disorder or a suspected drug reaction, one would have addressed that prior to prescribing prednisone for the skin manifestation.
Although primary cutaneous small vessel vasculitis is generally a self-limiting entity, conservative methods such as oral NSAIDs, antihistamines and leg elevation can be options. Research has also described a bland diet in assisting to decrease the development of palpable purpura.6 In addition to oral prednisone, dapsone (Aczone®, Allergan), colchicine (Colcrys®, AR Scientific) and cyclosporine have shown some success in decreasing the symptoms.6
The primary cutaneous small vessel vasculitis may represent an evolving systemic disorder. If and when the patient’s skin symptoms clear, it is important to monitor the patient for both recurrence of the skin rash and any other clinical changes.
Dr. Vlahovic is an Associate Professor at the Temple University School of Podiatric Medicine. She is a Fellow of the American Professional Wound Care Association and is board certified by the American Board of Podiatric Surgery.
Dr. Vlahovic pens a bimonthly blog for Podiatry Today. For more info, visit http://www.podiatrytoday.com/blogs/tracey-vlahovic-dpm  .
Editor’s note: For a related article, see “How To Treat Granuloma Annulare” in the December 2004 issue of Podiatry Today or visit the archives at www.podiatrytoday.com  .
1. Gyselbrecht L, De Keyser F, Ongenae K. Etiological factors and underlying conditions in patients with leukocytoclastic vasculitis. Clin Exp Rheum 1996; 14(6):665–668.
2. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37(2):187–192.
3. Ekenstam E, Callen JP. Cutaneous leukocytoclastic vasculitis: clinical and laboratory features of 82 patients seen in private practice. Arch Dermatol 1984; 120: 484–489.
4. Martinez-Taboada VM, Blanco R, Garcia-Fuentes M. Clinical features and outcome of 95 patients with hypersensitivity vasculitis. Am J Med 1997; 120(2):186–191.
5. Ponce de Souza E, Usatine RP. Palpable purpura and a visible sock line. J Fam Pract. 2005; 54(6):520-3.
6. Russell JP, Gibson LE. Primary cutaneous small vessel vasculitis: approach to diagnosis and treatment. Int J Dermatol. 2006; 45(1):3-13.