acute polyarticular synovitis as a rare presentation of kaposi sarcoma
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P6304Acute polyarticular synovitis as a rare presentation of Kaposi sarcoma
Larissa Larsen, MD, University of California Davis, Sacramento, CA, United States;Nasim Fazel, MD, DDS, University of California Davis, Sacramento, CA, UnitedStates
A 55-year-old man with a history of HIV on HAART therapy with CD4 of 309,undetectable viral load, and diffuse B-cell lymphoma presented to the hospital withan acute eruption of papules on his palms over 5 days. He also had associated fevers,polyarticular synovitis limiting his ambulation, conjunctivitis, and painful oralulcerations that started 2 days before the eruption. On cutaneous examination, hehad multiple pink-purple, firm, nonblanchable papules on the bilateral palms andpalmar fingers and discrete pink papules on the elbows and wrists. The mucosa ofthe lips, gingivae, and tongue had well defined, ;2- to 5-mm discrete ulcers witherythematous borders and a clean base. He did not have any of the above symptomsduring his course of diffuse B-cell lymphoma. Laboratory work-up revealed a CBCwith mild microcytic anemia and mild intermittent hypernatremia. The patient alsohad an elevated ESR and very mildly elevated rheumatoid factor, with an otherwisenormal autoimmune work-up, including a normal ANA and Anti-CCP antibody.Infectious work-up for cryptococcus, chlamydia, gonorrhea, histoplasma, barton-ella, rickettsia, Rocky Mountain spotted fever, and RPR was negative. EBV andsyphilis TPPAwere positive. Of note, IL-6 levels were elevated at 106. Arthrocentesiswas consistent with inflammatory fluid. Echocardiogram was negative for cardiacvegetations. Joint radiographic imaging was negative for a primary joint pathology.Biopsy of a palmar lesion was consistent with Kaposi sarcoma (KS) with positivestaining for HHV-8. The patient’s diagnosis is most consistent with interleukin-6erelated systemic inflammatory syndrome. This is a Kaposi sarcomaeassociatedsyndrome in the absence of multicentric Castleman disease. Patients with thiscondition present with inflammatory mixed connective tissue diseaseelike symp-toms without a diagnosable mixed connective tissue disease. The systemic inflam-matory responses in KS are felt to be secondary to elevated IL-6 inflammatorycytokine levels as was observed in our patient. Although B-cell lymphomas can havea variety of constitutional symptoms, synovitis is not typical. In addition, immunereconstitution syndrome is an unlikely explanation for the patient’s symptoms givenhis adherence to HAART therapy and stable CD4 count. The association of systemicinflammation and KS is very rare, and even rarer is the correlation with synovitis,making this a unique case of KS with synovitis as the presenting symptom.
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cial support: None identified.
CommerP6908An atypical case of disseminated visceral leishmaniasis/HIV coinfectionwidely involving the skin
Severino Persechino, Sant’Andrea Hospital, Rome, Italy; Alessandra Narcisi,Sant’Andrea Hospital, Rome, Italy; Antonella Tammaro, Sant’Andrea Hospital,Rome, Italy; Elisabetta Teti, Sant’Andrea Hospital, Rome, Italy; Flavia Persechino,Sant’Andrea Hospital, Rome, Italy; Lara Lambiase, Sant’Andrea Hospital, Rome,Italy; Massimo Marangi, Sant’Andrea Hospital, Rome, Italy
Visceral leishmaniasis (VL)-HIV coinfection is a well known clinical reality and it isemerging as an important predisposing factor for reactivation. We report a case of a51-year-old patient, HIV positive since 1999, currently under good viroimmunologiccontrol (HIV-RNA\20 copies/ml; 870 CD4 cells), coming fromMonterotondo, nearRome (an endemic area for leishmaniasis), detection of VL in 2000, recurrence in2005, 2011, and February 2012with a peculiar pulmonary localization, splenectomyin 2005 for risk of rupture. The patient presented to our observation in May 2012after a recent hospitalization for recurrent visceral leishmaniasis, referring quotidianfever, diarrhea (5 liquid discharges with evidence of mucus and blood), progressiveloss of weight, severe anemia, and a hyperpigmented lesion on his nose. Weperformed a gastroscopy and colonoscopy whose histology showed the presence ofmacrophages filled with Leishmania in the mucosa, a biopsy of the hyperpigmentedlesions of the nose that documented the presence of cutaneous leishmaniasis withbowenoid-like alterations overlying epidermis and a biopsy on intact skin where theskin appeared slightly atrophic and expanded ‘‘rete regis’’ with colonization ofamastigotes in groups of histiocytic-type cells in hair follicles, in the epidermissurrounding the meatus follicles and in the macrophages of the dermis, absence ofspecific immune response in form of granulomatous inflammation; the histologywas confirmed by the complete absence of follicle apparatus of the patient. Thediagnosis was disseminated visceral leishmaniasis. The patient was treated with anenhanced regimen of liposomal amphotericin B and miltefosine because of severityand spread of the disease. The peculiarity of this case report is certainly theextensive dissemination of visceral leishmaniasis (gastrointestinal, lung, healthyskin, and skin lesions), negative Leishmania serology, CD4[200 cells, splenectomy(primary reservoir of leishmaniasis, that we considered a negative prognostic factorand that is involved in such a serious dissemination), the particular and widelocalization of the cutaneous lesions, the involvement of healthy skin with a peculiarlocalization in the hair bulb and alopecia. Considering the marked skin involvement,biopsy of healthy skin will be used as a parameter for monitoring therapeuticefficacy.
cial support: None identified.
CommerJ AM ACAD DERMATOL
P6722An atypical distribution of erythema elevatum diutinum
Galia Ben-Zvi, MBBS, Addenbrooke’s Hospital, Cambridge University HospitalsNHS Trust, Cambridge, United Kingdom; Nigel Burrows, MBBS, MD,Addenbrooke’s Hospital, Cambridge University Hospitals NHS Trust,Cambridge, United Kingdom
A 47-year-old man presented with a 2-year history of 6 mildly tender, enlargingnodules on his back. There was no previous history of trauma or insect bites. Hewasotherwise well with no systemic symptoms. On examination, he had 6 reddish-brown, 1- to 2-cm nodules scattered asymmetrically on his upper back. They werewell demarcated with regular borders, firm but smooth and with no surface change.There was no lymphadenopathy or hepatosplenomegaly. Histologic analysisrevealed a dense superficial inflammatory cell infiltrate, separated from theepidermis by a Grenz zone, comprising mixed neutrophils, histiocytes, lympho-cytes, eosinophils, and plasma cells. The inflammation was vasculocentric andassociated with vascular necrosis. The deeper dermis was characterized by a denselaminar collagenous fibrosis, with a storiform pattern in areas, and a lesser degree ofmixed leukocytic infiltration and a central area of increased vascularity. Theappearances were consistent with erythema elevatum diutinum (EED). Full bloodcount, renal and liver function as well as immunoglobulins, serum angiotensinconverting enzyme and chest radiograph were all normal. Dapsone was offered butdeclined by the patient. He was therefore treated with intralesional triamcinoloneinjections which resulted in flattening of the lesions over 4 months. EED is a rare,chronic, cutaneous eruption characterized by fibrosing plaques with histologicevidence of leukocytoclastic vasculitis. Lesions most commonly occur symmetri-cally over the extensor aspects of extremities, such as the dorsal surfaces of thehands, elbows, buttocks, knees, and ankles. Rarely, atypical sites have been reportedincluding, palmar/plantar, retroauricular, and truncal lesions. These sites haveusually been reported to occur in conjunction with lesions at more typical sites.Lesions that involve the fingertips or pulps but spare the dorsal surfaces of the handshave also been described. Our case is unusual because of the site of presentationlocalized to the back only. Although dapsone is the recognized first line treatment forthis condition, patients with few lesions may respond to intralesional triamcinolone.
cial support: None identified.
CommerP6740An atypical plaque-like giant dermatofibroma
Serap Mellor, Royal Hallamshire Hospital, Sheffield, United Kingdom; Chris M.Stonard, Chesterfield Royal Hospital, Chesterfield, United Kingdom; Sue-MayAng, Royal Hallamshire Hospital, Sheffield, United Kingdom; Vinod I.Elangasinghe, Chesterfield Royal Hospital, Chesterfield, United Kingdom
We present a case of a 21-year-old woman with an ill defined, indurated, reddishbrown irregular plaque on her lateral left thigh measuring 10 cm 3 5.5 cm. Thislesionwas described as intermittently itchy but not painful. It had grown slowly over5 to 10 years without any periods of rapid growth. Our concerns were to excludedermatofibrosarcoma protuberance (DFSP) but considerations were given tosarcoidosis, interstistial granuloma annulare, and interstistial mastocytosis. A largeincisional biopsy revealed dermal infiltrating spindle cells with collagen entrapmentat the periphery. The epidermis and subcutaneous fat was normal.Immunohistochemical analysis was positive for factor XIIIa but negative for CD34.Smooth muscle actin, Melan A, and S100 were also negative. The histologic findingswere in keeping with a dermatofibroma, but the size and the clinical appearancepersuaded us to consider the diagnosis of a plaque like giant dermatofibromawhichis a rare clinical entitiy. Dermatofibroma (DF) is a common benign fibrohistiocyticlesion that usually appears as a slow-growing, firm dermal nodule with a predilectionfor the limbs of middle-aged women. They are usually \2 cm in diameter. Manyhistologic variants of DF have been described. Generally, the clinical and histologicfeatures are straightforward, but differentiating it from other cutaneous tumors canbe difficult in atypical cases and rare variants. A giant dermatofibroma is defined as alesion [5 cm in diameter, and the majority of them have been reported to bepedunculated. We believe plaque-like dermatofibroma is a rare variant belonging tothe group of giant dermatofibromas and\5 cases have been reported to date. Theselesions were symmetrical and well defined in clinical appearance; however, in ourcase, the lesions were asymmetrical and ill defined. Giant dermatofibromas areknown to have a benign course of progression but because of the atypicalappearance of our case we have decided to follow her up long-term.
cial support: None identified.
CommerAPRIL 2013