Serpiginous Purpuric Eruption of the Leg

October 2017 | Volume 16 | Issue 10 | Case Report | 1036 | Copyright © 2017

Kristin Totoraitis BS,a Cynthia M. Magro MD,b Adam Friedman MDc

aOakland University William Beaumont School of Medicine, Rochester, Michigan bDepartment of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY cDepartment of Dermatology, The George Washington Medical Faculty Associates, Washington, DC

Abstract

A previously healthy 68-year-old male presented with a rash on his right lower leg. The lesions had spread along the leg since onset 9 days prior, and the patient reported localized soreness and pruritus. He denied systemic symptoms including fever, fatigue, myalgia, joint pain, and recent illness. Physical examination revealed a serpiginous, purpuric eruption on the anterior and posterior right thigh and lower leg. A 4mm punch biopsy from the right lower leg revealed a Th2 dominant process reflective of a type IV delayed hypersensitivity reaction. Superficial and deep angiocentric and eccrinotropic lymphocytic infiltrate and tissue eosinophilia were noted. Degranulated eosinophils forming ‘flame figures’ were also identified with accompanying mural edema and red blood cell extravasation. Further evaluation revealed an elevated antistreptolysin O antibody, though the remainder of the work up was unremarkable. Clinicohistopathologic correlation supported the diagnosis of Blaschkolinear purpuric Wells’ Syndrome. This case highlights a unique presentation of a rare inflammatory dermatosis, and serves as a reminder that given Wells’ can be associated with underlying malignancy, an age appropriate work up, based on patient history and presentation, may be warranted.

J Drugs Dermatol. 2017;16(10):1036-1038.

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CASE REPORT

A previously healthy man in his 60s presented to the dermatology clinic with a chief complaint of rash on his right lower leg for 9 days duration. The patient admitted to the lesions spreading along the leg since onset, as well as soreness and pruritus at the site. On review of systems, the patient denied any systemic symptoms including fever, fatigue, myalgia, joint pain, or recent illness. Physical examination revealed a serpiginous, purpuric eruption on the anterior and posterior right thigh and lower leg (Figures 1, 2). A punch biopsy specimen from a lesion on the right posterior lower leg was obtained (Figures 3, 4).The biopsy specimen revealed a Th2 dominant process reflective of a type IV delayed hypersensitivity reaction. Superficial and deep angiocentric and eccrinotropic lymphocytic infiltrate and tissue eosinophilia were noted, likely contributing to vascular compromise (Figure 3, 4). Degranulated eosinophils forming ‘flame figures’ were also identified. There was accompanying mural edema and red blood cell extravasation. Clinicohistopathologic correlation supported the diagnosis of Blaschkolinear purpuric Wells’.The patient was treated with clobetasol cream twice daily. Further evaluation revealed an elevated antistreptolysin O antibody, though the remainder of the work up was unremarkable, including complete blood count, complete metabolic panel, anti-nuclear antibody, C-reactive protein, lactate dehydrogenase, rapid plasma regain, thyroid stimulating hormone, Lyme antibodies, hepatitis panel, and protein electrophoresis. He experienced gradual improvement of the eruption over the following weeks with subsequent resolution within 8 weeks.

DISCUSSION

Wells’ Syndrome, also known as eosinophilic cellulitis, is a rare inflammatory dermatosis that typically presents as acute erythematous plaques. Lesions rarely occur on the face, and patients can present at any age.1,2 The clinical course can be divided into two stages: first with erythematous plaques that can have associated itching, burning, or local tenderness.3 Less common findings include blisters, bullae, urticaria, papules, and nodules.3,4 Our patient’s unique purpuric presentation was likely due to perivascular lymphocytic infiltrate on microscopy, leading to red cell extravasation. The second clinical stage of Wells’ Syndrome is resolution, which can take 2-8 weeks and leave behind skin atrophy and hyperpigmentation.3The etiology of Wells’ Syndrome is unknown, however proposed theories suggest this is a reactive condition to a trigger. Suggested triggers include insect bites, infections, hematologic disorders, and malignancies.3 Reported malignancies include chronic lymphocytic and myeloid leukemia, non-Hodgkin’s lymphoma, renal cell carcinoma, colonic adenocarcinoma, nasopharyngeal carcinoma, and squamous cell carcinoma.5,6,7 In addition, multiple drugs have been implicated including acetyl salicylic acid, penicillin G, bleomycin, and local anesthetics.4 Due to frequently associated urticaria, eosinophilia, and suggested drug and insect triggers, an allergic hypersensitivity may also play a role.4 This is consistent

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