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A Common Purpuric Rash With Uncommon Association in a Woman With Pedal Edema

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 70-year-old Indian woman presented with a generalized rash over the lower extremity associated with pedal edema extending up to the shins. The onset was gradual and started on the lower extremities and gradually extended to involve the lower trunk. The rash was associated with mild discomfort. The patient denied itching, weeping, or bleeding from the rash. She denied any constitutional symptoms (weight loss, fever, night sweats), joint pains, history of blood in urine, periorbital edema, and recent intake of medications or herbal medications. Social history was significant for 25 pack-years of smoking. Physical examination revealed multiple well-defined, coalescent, erythematous, palpable, purpuralike lesions that were predominantly present over the trunk and lower extremities (Figure, A and B). Laboratory test results at presentation included a hemoglobin level of 11.2 g/dL (to convert to g/L, multiply by 10), mean corpuscular volume of 84 fL, erythrocyte sedimentation rate of 72 mm/h, and a lactate dehydrogenase level of 412 IU/L. Hepatic and renal function levels were within normal limits. Complement levels were normal. Hepatitis B and C and HIV profiles were negative. A chest radiograph showed a 2-cm lesion in the upper lobe of the left lung. A computed tomographic scan of the chest showed a spiculated mass in the same region along with mediastinal lymphadenopathy. An ultrasound of the abdomen showed bilateral adrenal masses. A lesional skin biopsy specimen was obtained (Figure, C).

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B. Cutaneous leukocytoclastic vasculitis

The histopathologic findings from a lesional skin biopsy specimen revealed neutrophilic infiltration of the small blood vessels in the skin along with necrosis and extravasation of the red blood cells (Figure, C). The findings from a biopsy specimen of a lung lesion revealed small cell carcinoma. The patient was diagnosed with extensive-stage small cell lung cancer and began systemic chemotherapy with cisplatin and etoposide. Unfortunately, the patient died soon after the first dose of chemotherapy due to neutropenic sepsis.

Cutaneous manifestation of an internal malignant neoplasm may present as direct infiltration into the skin or may present indirectly as a paraneoplastic syndrome (PNS).1 The Curth postulates are the cornerstone for defining the association between internal malignant neoplasms and cutaneous disorders.1 Leukocytoclastic vasculitis is a histopathologic term denoting vasculitis of the small vessels with predominantly neutrophilic infiltrate.2 After degranulation, the neutrophils undergo apoptosis, a process known as leukocytoclasis, releasing nuclear debris, which is also called nuclear dust.2 According to the consensus statement developed at the Chapel Hill Conference on Nomenclature of Systemic Vasculitis in 1994, cutaneous leukocytoclastic vasculitis (CLV) is defined as an“[i]solated cutaneous leukocytoclastic angiitis without systemic vasculitis or glomerulonephritis.”2,3 As a PNS, CLV is the most commonly diagnosed vasculitis (45%) followed by polyarteritis nodosa (36.7%).4 Cutaneous leukocytoclastic vasculitis occurs most frequently with hematologic cancers followed by lung, gastrointestinal, or urinary tract tumors.5,6 Approximately 20% to 26% of patients with paraneoplastic CLV have lung cancer.4,6 Among lung cancers, adenocarcinoma and squamous cell are most commonly associated with CLV.4,6,7

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Article Information

Corresponding Author: Ankit Mangla, MD, Division of Hematology Oncology, Department of Internal Medicine, University Hospitals Seidman Cancer Center, Case Western Reserve University, School of Medicine, 11100 Euclid Ave, Lakeside Ste 1200, Cleveland, OH 44106 (ankit.mangla@UHhospitals.org).

Published Online: April 4, 2019. doi:10.1001/jamaoncol.2019.0174

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s family for granting permission to publish this information.

References
1.
Thiers  BH, Sahn  RE, Callen  JP.  Cutaneous manifestations of internal malignancy.  CA Cancer J Clin. 2009;59(2):73-98. doi:10.3322/caac.20005PubMedGoogle ScholarCrossref
2.
Gota  CE, Calabrese  LH.  Diagnosis and treatment of cutaneous leukocytoclastic vasculitis.  Int J Clin Rheumatol. 2013;8(1):49-60. doi:10.2217/ijr.12.79Google ScholarCrossref
3.
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. doi:10.1002/art.1780370206PubMedGoogle ScholarCrossref
4.
Kanaji  N, Watanabe  N, Kita  N,  et al.  Paraneoplastic syndromes associated with lung cancer.  World J Clin Oncol. 2014;5(3):197-223. doi:10.5306/wjco.v5.i3.197PubMedGoogle ScholarCrossref
5.
Pelosof  LC, Gerber  DE.  Paraneoplastic syndromes: an approach to diagnosis and treatment.  Mayo Clin Proc. 2010;85(9):838-854. doi:10.4065/mcp.2010.0099PubMedGoogle ScholarCrossref
6.
Solans-Laqué  R, Bosch-Gil  JA, Pérez-Bocanegra  C,  et al.  Paraneoplastic vasculitis in patients with solid tumors: report of 15 cases.  J Rheumatol. 2008;35(2):294-304.PubMedGoogle Scholar
7.
Kathula  SK, Thomas  DE, Anstadt  MP, Khan  AU.  Paraneoplastic cutaneous leukocytoclastic vasculitis and iron deficiency anemia as the presenting features of squamous cell lung carcinoma.  J Clin Oncol. 2011;29(4):e83-e85. doi:10.1200/JCO.2010.31.4708PubMedGoogle ScholarCrossref
8.
Podjasek  JO, Wetter  DA, Pittelkow  MR, Wada  DA.  Henoch-Schönlein purpura associated with solid-organ malignancies: three case reports and a literature review.  Acta Derm Venereol. 2012;92(4):388-392. doi:10.2340/00015555-1288PubMedGoogle ScholarCrossref
9.
Zurada  JM, Ward  KM, Grossman  ME.  Henoch-Schönlein purpura associated with malignancy in adults.  J Am Acad Dermatol. 2006;55(5)(suppl):S65-S70. doi:10.1016/j.jaad.2005.10.011PubMedGoogle ScholarCrossref
10.
Larson  AR, Granter  SR.  Utility of immunofluorescence testing for vascular IgA in adult patients with leukocytoclastic vasculitis.  Am J Clin Pathol. 2014;142(3):370-374. doi:10.1309/AJCPMB1A9QSWUJDYPubMedGoogle ScholarCrossref
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