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Its most severe form of presentation is known as Mucha-Habermann disease, which, along with skin involvement also develops systemic symptoms, including arthritis.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a 56-year-old male with a history of hypertension who came to the emergency department due to 1-week history of pruritic midthoracic rash, fever and malaise. The patient reported no associated infective signs or symptoms, no constitutional syndrome or epidemiological history of interest. There was no introduction of new drugs.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Physical examination revealed the presence of polymorphous lesions (macules, papules, vesicles, blisters, pustules and scabs), non-confluent, bilateral asymmetric distribution, including palms, soles, scalp, oral and nasal mucosa; the rest of the physical examination by systems was normal.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was admitted to the Infectious Diseases Unit on suspicion of adult chickenpox. The study was completed with serology tests – HBV, HCV, VZV, CMV, HSV 1 and 2, syphilis, <span class="elsevierStyleItalic">Rickettsia</span> – which were negative. A gallbladder exudate culture and a VZV DNA study in vesicular fluid were both negative. Blood cultures for aerobes and anaerobes were also negative.</p><p id="par0025" class="elsevierStylePara elsevierViewall">An empirical treatment was initiated with broad-spectrum antibiotics and symptomatic measures, which resulted in fever remission and hospital discharge. 48<span class="elsevierStyleHsp" style=""></span>h later the patient came back to the emergency room for progression of skin lesions, fever recurrence and development of arthralgia with swelling in wrists, knees and ankles, so he was admitted to the Department of Rheumatology.</p><p id="par0030" class="elsevierStylePara elsevierViewall">On physical examination, it was observed that the maculopapular lesions had progressed to well-defined, confluent, target morphology lesions with erythematous halo; widespread pustular and scabby necrotic lesions were also noticed. Oedematous arthritis was also observed in hands, knees and ankles.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Additional tests showed an increase in acute phase reactants, leukocytosis at the expense of neutrophils and eosinophils, with negative autoimmunity study (ANA, ENA, ANCA), rheumatoid factor, ACPA and serum complement (C3, C4 and C1q) within normal values and proteinogram with a discrete polyclonal elevation of immunoglobulins. Musculoskeletal ultrasonography showed extensor tenosynovitis of both wrists and grade 2 effusion in radiocarpal and midcarpal joints as well as in the ankles without synovitis. The histological study of the vesicle biopsy was consistent with PLEVA.</p><p id="par0040" class="elsevierStylePara elsevierViewall">As a ulceronecrotic variant of PLEVA was suspected, treatment with erythromycin and methotrexate was initiated, escalating to 20<span class="elsevierStyleHsp" style=""></span>mg/week together with folic acid. The systemic symptoms remitted within 4 weeks, with progressive resolution of skin lesions and polyarthritis after 8 weeks.</p><p id="par0045" class="elsevierStylePara elsevierViewall">PLEVA is a rare inflammatory skin disease of unknown etiology, although some authors have postulated that it could be a T-cell lymphoproliferative disease.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> It can occur at any age, being more frequent in young children and adults.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">It appears as a papular and papulovesicular rash which rapidly develops into hemorrhagic or necrotic scabs. Febrile ulceronecrotic Mucha-Habermann disease is its most severe form, and may be accompanied by high fever, asthenia, hematologic abnormalities, cardiomyopathy, CNS vasculitis, arthritis and interstitial pneumonitis.<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3,4</span></a> It occurs in outbreaks of varying duration, usually lasting from a few weeks to months.</p><p id="par0055" class="elsevierStylePara elsevierViewall">The diagnosis is based on skin lesion analysis and histological study, which is usually characterized by the presence of leukocytoclastic vasculitis, although the findings are not pathognomonic.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Treatment with tetracyclines and phototherapy are considered first-line. Methotrexate should be reserved as second-line treatment or for the ulceronecrotic variant of Mucha-Habermann. Although elevated levels of TNFα have been documented, the role of anti-TNFα drugs has not been well studied yet.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">In conclusion, Mucha-Habermann disease should be considered in the differential diagnosis in patients with a polymorphous rash who develop a seronegative polyarthritis.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Muñoz Gómez MM, Novella Navarro M, Salvatierra Ossorio J. Artritis seronegativa secundaria a enfermedad de Mucha-Habermann. Med Clin (Barc). 2016;147:564.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Febrile ulceronecrotic Mucha-Habermann disease with clonality: a cutaneous T-cell lymphoma entity?" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Cozzio" 1 => "J. Hafner" 2 => "W. Kempf" 3 => "A. Häffner" 4 => "G. Palmedo" 5 => "S. 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Scientific letter
Seronegative arthritis secondary to Mucha-Habermann disease
Artritis seronegativa secundaria a enfermedad de Mucha-Habermann
Maria del Mar Muñoz Gómez
, Marta Novella Navarro, Juan Salvatierra Ossorio
Corresponding author
Servicio de Reumatología, Hospital Universitario San Cecilio, Granada, Spain