Necrotic plaque on the calf and thigh of a woman with cryptogenic cirrhosis.

Case Report

This case we present here is a 36-year-old female with a liver transplant at age 25 for cryptogenic cirrhosis (maintained on tacrolimus, cyclosporine and prednisone) who presented with a 2 month history of a 10 cm tender reticulated necrotic plaque with surrounding erythema on her right medial posterior calf and right posterior thigh [Figures 1 and 2]. Concurrent with the rash, she had developed end stage renal disease, hand and foot dysesthesias, fevers of 104 F, arthralgias, myalgias, weight loss and fatigue. Following initial presentation, she developed painful skin-colored nodules and firm purplish papules on the bilateral lower legs, and ulcers on the abdomen, shins, calves and buccal mucosa.

Figure 1

Necrotic plaque on right medial posterior calf

Figure 2

Necrotic plaque on right posterior thigh

Serologies were negative for hepatitis B and C, antinuclear antibody, rheumatoid factor, perinuclear-anti-neutrophil cytoplasmic antibody, cytoplasmic-anti-neutrophil cytoplasmic antibody, alpha-1 antitrypsin, Q fever titers, anti-phospholipid antibodies and cryoglobulins. Lower extremity ultrasound was negative for deep vein thrombosis. Computed tomography angiogram of abdomen and pelvis was negative for vascular micro-aneurysms.

A biopsy, from the abdominal ulcer, revealed a medium vessel vasculitis with leukocytoclasis [Figure 3].

Figure 3

Punch biopsy from an ulcer on the abdomen. High power view of dermis showing medium-sized vessel vasculitis characterized by fibrin thrombus and leukocytoclasis surrounding the blood vessel and infiltrating the vessel wall (H and E, ×20)

Diagnosis

Polyarteritis nodosa (PAN).

Discussion

According to the American College of Rheumatology (ACR) criteria, three of the following 10 must be met in order have a diagnosis of PAN:[1] (1) Weight loss ≥4.5 kg, (2) livedo reticularis, (3) t esticular pain or tenderness, (4) muscle pain, weakness, or leg tenderness, (5) nerve disease, (6) diastolic blood pressure greater than 90 mmHg, (7) elevated kidney blood tests (blood urea nitrogen [BUN] >40 mg/dl or creatinine >1.5 mg/dl), (8) hepatitis B virus (HBV) or hepatitis C virus (HCV) tests positive, (9) arteriogram showing the aneurysms or constricted by the blood vessel inflammation and (10) biopsy of tissue showing the arteritis.

Our patient fulfilled five criteria for the diagnosis of systemic PAN: Myalgias, nerve disease, biopsy showing arteritis, livedo reticularis and weight loss. Although she had elevated BUN and creatinine, this was attributed to her diagnosis of end stage renal disease secondary to calcineurin inhibitor toxicity.

Cutaneous and systemic PAN (when presenting on the skin) usually appear first as livedo reticularis, tender subcutaneous nodules, or cutaneous ulcerations.[2] Other cutaneous findings include petechiae, purpura, cutaneous necrosis, and autoamputations.[23] The legs are the most commonly affected at 97% of the time, followed by arms in 33% and the trunk in 8% respectively.[2] Although cutaneous PAN predominately affects the skin, extracutaneous findings can be present as in systemic PAN including fever, malaise, myalgias, arthralgias and neuropathy. The distinction between cutaneous and systemic PAN is the multi-organ involvement found in systemic PAN, particularly of the heart, kidneys and liver.[3]

Our patient's history of cryptogenic cirrhosis may have been associated with PAN. Liver involvement in patients with PAN ranges from asymptomatic serum aminotransferase elevation, hepatic infarction and intrahepatic or extra-hepatic aneurysm rupture to cirrhosis.[45] Although the patients serologies for HBV and HCV were negative, which can be associated with PAN,[1] the patient's initial cirrhotic liver was most likely was the first manifestation of PAN. Only after her cutaneous manifestations of PAN did the diagnosis of PAN become part of the differential.

Learning Points

3 of the 10 criteria designated by ACR must be met in order have a diagnosis of systemic PAN.

These 10 criteria include weight loss ≥4.5 kg, livedo reticularis, testicular pain/tenderness, myalgias, nerve disease, elevated diastolic blood pressure, elevated kidney blood tests BUN or creatinine, positive HBV or HCV, arteriogram showing the aneurysms or blood vessel constriction and biopsy of tissue showing the arteritis.

Cutaneous PAN manifests as livedo reticularis, tender subcutaneous nodules, or cutaneous ulcerations, but extracutaneous findings can be present including fever, malaise, myalgias, arthralgias, and neuropathy.

Various types of liver disease may be associated with PAN.