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Seronegative arthritis secondary to Mucha-Habermann disease
Artritis seronegativa secundaria a enfermedad de Mucha-Habermann
Maria del Mar Muñoz Gómez
Corresponding author
mari936@hotmail.com

Corresponding author.
, Marta Novella Navarro, Juan Salvatierra Ossorio
Servicio de Reumatología, Hospital Universitario San Cecilio, Granada, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Pityriasis lichenoides et varioliformis acuta &#40;PLEVA&#41; is a rare inflammatory skin disease&#46; Its most severe form of presentation is known as Mucha-Habermann disease&#44; which&#44; along with skin involvement also develops systemic symptoms&#44; including arthritis&#46;</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&#44; fever and malaise&#46; The patient reported no associated infective signs or symptoms&#44; no constitutional syndrome or epidemiological history of interest&#46; There was no introduction of new drugs&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Physical examination revealed the presence of polymorphous lesions &#40;macules&#44; papules&#44; vesicles&#44; blisters&#44; pustules and scabs&#41;&#44; non-confluent&#44; bilateral asymmetric distribution&#44; including palms&#44; soles&#44; scalp&#44; oral and nasal mucosa&#59; the rest of the physical examination by systems was normal&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was admitted to the Infectious Diseases Unit on suspicion of adult chickenpox&#46; The study was completed with serology tests &#8211; HBV&#44; HCV&#44; VZV&#44; CMV&#44; HSV 1 and 2&#44; syphilis&#44; <span class="elsevierStyleItalic">Rickettsia</span> &#8211; which were negative&#46; A gallbladder exudate culture and a VZV DNA study in vesicular fluid were both negative&#46; Blood cultures for aerobes and anaerobes were also negative&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">An empirical treatment was initiated with broad-spectrum antibiotics and symptomatic measures&#44; which resulted in fever remission and hospital discharge&#46; 48<span class="elsevierStyleHsp" style=""></span>h later the patient came back to the emergency room for progression of skin lesions&#44; fever recurrence and development of arthralgia with swelling in wrists&#44; knees and ankles&#44; so he was admitted to the Department of Rheumatology&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">On physical examination&#44; it was observed that the maculopapular lesions had progressed to well-defined&#44; confluent&#44; target morphology lesions with erythematous halo&#59; widespread pustular and scabby necrotic lesions were also noticed&#46; Oedematous arthritis was also observed in hands&#44; knees and ankles&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Additional tests showed an increase in acute phase reactants&#44; leukocytosis at the expense of neutrophils and eosinophils&#44; with negative autoimmunity study &#40;ANA&#44; ENA&#44; ANCA&#41;&#44; rheumatoid factor&#44; ACPA and serum complement &#40;C3&#44; C4 and C1q&#41; within normal values and proteinogram with a discrete polyclonal elevation of immunoglobulins&#46; 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&#46; The histological study of the vesicle biopsy was consistent with PLEVA&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">As a ulceronecrotic variant of PLEVA was suspected&#44; treatment with erythromycin and methotrexate was initiated&#44; escalating to 20<span class="elsevierStyleHsp" style=""></span>mg&#47;week together with folic acid&#46; The systemic symptoms remitted within 4 weeks&#44; with progressive resolution of skin lesions and polyarthritis after 8 weeks&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">PLEVA is a rare inflammatory skin disease of unknown etiology&#44; although some authors have postulated that it could be a T-cell lymphoproliferative disease&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> It can occur at any age&#44; being more frequent in young children and adults&#46;<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&#46; Febrile ulceronecrotic Mucha-Habermann disease is its most severe form&#44; and may be accompanied by high fever&#44; asthenia&#44; hematologic abnormalities&#44; cardiomyopathy&#44; CNS vasculitis&#44; arthritis and interstitial pneumonitis&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3&#44;4</span></a> It occurs in outbreaks of varying duration&#44; usually lasting from a few weeks to months&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">The diagnosis is based on skin lesion analysis and histological study&#44; which is usually characterized by the presence of leukocytoclastic vasculitis&#44; although the findings are not pathognomonic&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">Treatment with tetracyclines and phototherapy are considered first-line&#46; Methotrexate should be reserved as second-line treatment or for the ulceronecrotic variant of Mucha-Habermann&#46; Although elevated levels of TNF&#945; have been documented&#44; the role of anti-TNF&#945; drugs has not been well studied yet&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">In conclusion&#44; Mucha-Habermann disease should be considered in the differential diagnosis in patients with a polymorphous rash who develop a seronegative polyarthritis&#46;</p></span>"
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ISSN: 23870206
Original language: English
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