The Clinical Picture Salvador Arias-Santiago, MD Jo sé Aneiros-Fernández, MD María Sierra Girón-Prieto, MD Department of Dermatology, San Cecilio University Department of Pathology, San Cecilio Univer- Department of Dermatology, San Cecilio University Hospital, Granada, Spain sity Hospital, Granada, Spain Hospital, Granada, Spain

María Antonia Fernández-Pugnaire, PhD R amón Naranjo-Sintes, PhD Department of Dermatology, San Cecilio University Hospital, Department of Dermatology, San Cecilio University Granada, Spain Hospital, Granada, Spain

The Clinical Picture Palpable

healthy 47-year-old woman presents Awith a 3-day history of widespread asymp- tomatic lesions in the extremities, fever, ar- thralgias, and mild abdominal pain. A physical examination reveals a symmetric rash in the lower legs with erythematous purple papules. These papules do not blanch when pressure is applied and so are clinically compatible with palpable purpura (Figure 1). Skin biopsy shows leukocytoclastic with immunoglobu- lin A (IgA) deposits in the vascular wall. Q: Which is the most likely diagnosis? □□ Idiopathic thrombocytopenic purpura □□ Vitamin C deficiency (scurvy) A s Henoch- □□ Kaposi sarcoma not related to human immunodeficiency virus (HIV) infection Schönlein □□ Henoch-Schönlein purpura purpura is less □□ common in A: The correct answer is Henoch-Schönlein adults, biopsy purpura. Idiopathic thrombocytopenic purpura is an plays an caused by specific antibod- important ies against platelet-membrane glycoproteins. It is characterized by thrombocytopenia not ex- diagnostic role plainable by contact with toxic substances or by other causes. Along with nonpalpable pur- F Igure 1. Erythematous purple papules on pura, other common signs are epistaxis, gingival the lower legs. bleeding, menorrhagia, and retinal hemorrhage. Scurvy is an uncommon deficiency of forearms, small corkscrew hairs, and sicca syn- ascorbic acid (vitamin C). The elderly and drome, which is more common in adults. alcoholics are at higher risk, as they do not Non-HIV Kaposi sarcoma usually affects take in enough vitamin C in the diet. Patients elderly patients, with pink, red, or brown pap- usually show perifollicular hemorrhages of the ules or nodules on the legs and, less commonly, skin and mucous membranes, typically pete- on the head and neck. Histopathologic exami- chial hemorrhage or ecchymosis of the gums nation shows newly formed irregular blood ves- around the upper incisors. Other cutane- sels with an inflammatory infiltrate of plasma ous signs are follicular hyperkeratosis on the cells and lymphocytes; immunohistochemical doi:10.3949/ccjm.77a.09065 human herpes virus staining is usually positive.

CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 77 • NUMBER 3 MARCH 2010 205 Downloaded from www.ccjm.org on September 30, 2021. For personal use only. All other uses require permission. Pal pable Purpura

Polyarteritis nodosa is a that af- Management of Henoch-Schönlein purpura is mainly fects medium or small arteries with necrotizing inflam- directed at the symptoms, with oral hydration and non- mation; renal glomeruli and arterioles, capillaries, and steroidal anti-inflammatory drugs. For severe cases, a short venules are unaffected. Skin manifestations include pal- course of corticosteroids (0.5–1 mg/kg) may be used. pable purpura, livedo reticularis, ulcers, and distal gan- Although no controlled clinical trial has proven that grene. The condition also usually affects the kidneys, the Henoch-Schönlein purpura responds to corticosteroids, heart, and the musculoskeletal and nervous systems. colchicine, or other drugs, corticosteroids are used most often, especially in patients with renal disease. Patients ■■ A systemic vasculitis with severe renal insufficiency, abdominal pain, joint involvement, or bleeding should be hospitalized. Plas- Henoch-Schönlein purpura is a systemic vascu­litis af- mapheresis3 has been used in severe cases. fecting the skin, gastrointestinal tract, kidneys, and joints. Palpable purpura and joint pain are the most ■■ Henoch-Schönlein purpura common and consistent presenting symptoms. The AND MALIGNANCY kidneys are affected in about one-third of children and in 60% of adults, and this is the major factor determin- During a follow-up evaluation 1 month later, our ing the long-term outcome.1 patient was diagnosed with adenocarcinoma of the In our patient, laboratory testing that included a breast. This highlights the value of a workup for can- complete blood cell count, biochemical testing (includ- cer in adults with cutaneous vasculitis. ing IgA levels), urinary sediment, and coagulation stud- Cutaneous vasculitis can represent a paraneoplas- ies showed no abnormalities except elevations of the tic syndrome associated with a malignant tumor. The erythrocyte sedimentation rate and the concentration pathophysiology of this association is unclear, but of C-reactive protein (an acute-phase reactant). These one proposed mechanism is the exaggerated produc- can be normal in some patients. Renal involvement tion of antibodies that react against tumor neoanti- was also not present. gens, leading to the formation of immune complexes, or that occasionally recognize endothelial cells be- ■■ DIAGNOSIS cause of similarities with tumor antigens. Another theory is that abnormally high levels of inflamma- The diagnosis relies on clinical manifestations. Because tory cytokines are produced by neoplastic cells or in Henoch-Schönlein purpura is less common in adults, response to decreased clearance. biopsy plays a more important role in establishing the Yet another theory is that hyperviscosity of the diagnosis in this age group, and it does this by dem- blood, seen in some cancers, increases the contact time onstrating leukocytoclastic vasculitis with a predomi- for deposition of immune complexes and causes endo- nance of IgA deposition under immunofluorescence. thelial damage. Drugs used to treat cancer have also Recent studies in children showed that an elevated been reported to produce Henoch-Schönlein purpura.4 IgA concentration along with reduced IgM levels was Although hematologic malignancy is three to five associated with a higher rate of severe complications.2 times more common than solid tumors in patients However, depending on the age of the biopsied lesion, with small-vessel vasculitis, the disease has been as- IgA may not be detected. sociated with solid tumors of the liver, skin, colon, and breast in adults over age 40.5 An evaluation for ■■ TREATMENT directed at symptoms neoplasm is therefore reasonable in adults with He- noch-Schönlein purpura, as is an evaluation for tu- Our patient received oral corticosteroids 0.5 mg/kg per mor recurrence or metastasis if the patient has been day for 20 days, and the lesions resolved by 4 weeks. previously treated for a malignant tumor. ■ ■■ REFERENCes and limit organ damage. Dermatology 2009; 219:167–170. 4. Mitsui H, Shibagaki N, Kawamura T, Matsue H, Shimada S. A clinical .R 1 ieu P, Noël LH. Henoch-Schönlein nephritis in children and adults. study of Henoch-Schönlein purpura associated with malignancy. J Eur Morphological features and clinicopathological correlations. Ann Med Acad Dermatol Venereol 2009; 23:394–401. Interne (Paris) 1999; 150:151–159. 5. Maestri A, Malacarne P, Santini A. Henoch-Schönlein syndrome 2. Fretzayas A, Sionti I, Moustaki M, Nicolaidou P. Clinical impact of associated with breast cancer. A case report. Angiology 1995; altered immunoglobulin levels in Henoch-Schönlein purpura. Pediatr 46:625–627. Int 2009; 51:381–384. 3. Donghi D, Schanz U, Sahrbacher U, et al. Life-threatening or organ- ADDRESS: Salvador Arias-Santiago, MD, San Cecilio University Hospital, Av impairing Henoch-Schönlein purpura: plasmapheresis may save lives Dr Oloriz 16, Granada 18012, Spain; e-mail [email protected].

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