Indian Journal of Dermatology
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E-IJD QUIZ
Year : 2015  |  Volume : 60  |  Issue : 2  |  Page : 218
Necrotic plaque on the calf and thigh of a woman with cryptogenic cirrhosis


1 David Geffen School of Medicine at UCLA, UCLA, Los Angeles, California, USA
2 Department of Pathology, Division of Dermatology, UCLA, Los Angeles, California, USA
3 Department of Medicine, Division of Dermatology, UCLA, Los Angeles, California, USA

Date of Web Publication3-Mar-2015

Correspondence Address:
Lily Adelzadeh
4255 La Salle Ave., Culver City, CA 90232
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.152601

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How to cite this article:
Adelzadeh L, Kerstetter J, Worswick S. Necrotic plaque on the calf and thigh of a woman with cryptogenic cirrhosis. Indian J Dermatol 2015;60:218

How to cite this URL:
Adelzadeh L, Kerstetter J, Worswick S. Necrotic plaque on the calf and thigh of a woman with cryptogenic cirrhosis. Indian J Dermatol [serial online] 2015 [cited 2020 Mar 30];60:218. Available from: http://www.e-ijd.org/text.asp?2015/60/2/218/152601



   Case Report Top


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 [Figure 1] and [Figure 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

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Figure 2: Necrotic plaque on right posterior thigh

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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)

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   Diagnosis Top


Polyarteritis nodosa (PAN).


   Discussion Top


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. [2],[3] 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. [4],[5] 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 Top


  1. 3 of the 10 criteria designated by ACR must be met in order have a diagnosis of systemic PAN.
  2. 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.
  3. 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.
  4. Various types of liver disease may be associated with PAN.


 
   References Top

1.
Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, McShane DJ, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum 1990;33:1088-93.  Back to cited text no. 1
    
2.
Daoud MS, Hutton KP, Gibson LE. Cutaneous periarteritis nodosa: A clinicopathological study of 79 cases. Br J Dermatol 1997;136:706-13.  Back to cited text no. 2
    
3.
Nakamura T, Kanazawa N, Ikeda T, Yamamoto Y, Nakabayashi K, Ozaki S, et al. Cutaneous polyarteritis nodosa: Revisiting its definition and diagnostic criteria. Arch Dermatol Res 2009;301:117-21.  Back to cited text no. 3
    
4.
Kart-Koseoglu H, Yucel AE, Aytekin C, Sahin B. Polyarteritis nodosa complicated by intrahepatic-perihepatic hemorrhage and acute appendicitis: Successful treatment with cyclophosphamide and corticosteroids. Clin Rheumatol 2003;22:251-3.  Back to cited text no. 4
    
5.
Mowrey FH, Lundberg EA. The clinical manifestations of essential polyanglitis (periarteritis nodosa), with emphasis on the hepatic manifestations. Ann Intern Med 1954;40:1145-64.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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