a case of generalized atypical pyoderma gangrenosum mimicking sweet syndrome
Post on 27-Dec-2016
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P6920A case of disseminated cutaneous miliarial gout
Audrey Wang, MD, University of California, Davis, Sacramento, CA, United States;Rivkah Isseroff, MD, University of California, Davis, Sacramento, CA, UnitedStates
We describe a 34-year-old morbidly obese African American man with a history oftype 2 diabetes mellitus, congestive heart failure, pulmonary embolus (oncoumadin), cerebrovascular accident, and chronic renal insufficiency, who pre-sented with multiple draining, firm nodules within tender, hyperpigmented,indurated plaques on the lower abdomen and several smaller nondraining noduleson the medial thighs bilaterally. Several draining nodules notably extruded a thinwhite, milky fluid. The lesions started on his lower abdomen approximately 1 yearbefore evaluation, where he had injected enoxaparin subcutaneously for 4 days. Sixmonths afterward, he noted similar lesions on his medial midthighs bilaterally in theabsence of trauma. Skin biopsies obtained from the right thigh and left upper arm(processed in formalin) showed changes consistent with tophaceous gout in thedermis. An outside provider prescribed allopurinol 300mg bymouth twice daily anda prednisone taper several weeks before our evaluation, but the patient reported noimprovement in the lesions or symptoms. Laboratory evaluation was significant forcreatinine of 1.4 to 1.9mg/dL and normal serum uric acid level. The patient reportedno fevers/chills, joint pain or swelling, or known history of gout involving the joints.He endorsed social alcohol use. Cutaneous deposition diseases involve accumula-tion of endogenous materials within the dermis or subcutis. Cutaneous gouttypically presents with aggregates of negatively birefringent monosodium uratecrystals in the periarticular regions and ear helices several years after the onset ofgouty arthritis. Few case reports have described involvement of other areas, such asthe legs and breast. Rare manifestations of gout also include tender panniculitis withindurated subcutaneous plaques and miliarial gout involving multiple small papulescontaining a white material on the skin. Our case has clinical features of bothpanniculitic and miliarial gout in the absence of arthritis and hyperuricemia. Theetiology for this patient’s presentation is unclear, but given his multiple comorbid-ities, he may be at increased risk of developing gouty arthritis in the future. Thoughrare, this case highlights the importance of considering atypical cutaneous gout inthe differential of deposition disorders.
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cial support: None identified.
CommerP6208A case of generalized atypical pyoderma gangrenosum mimicking Sweetsyndrome
Venessa Pena-Robichaux, MD, Henry Ford Health System, Detroit, MI, UnitedStates; Christopher Burnett, MD, Henry Ford Health System, Detroit, MI, UnitedStates
Pyoderma gangrensoum (PG) is an uncommon neutrophilic dermatosis that usuallypresents as chronic recurrent ulcerative lesions with a distinct clinical appearance.Atypical (bullous) PG is a variant of this disease whose clinical appearance is similarand often confused with the superficial bullous variant of Sweet syndrome giventheir morphologic and histopathologic similarities. Atypical PG, unlike classic PG,most commonly presents with painful cutaneous lesions on the face and upperextremities (typically the dorsal surfaces of the hands) and is more closely associatedwith hematologic disorders, such as acute myelogenous leukemia and myelodys-plasia. We describe a case of a 28-year-old white man who was admitted to thehospital with an acute, painful vesiculobullous eruption that started on the dorsalsurfaces of his hands and feet and over a few days spread to involve his trunk, arms,legs, face, buttocks, genitals, and oral mucosa. He had no history of fever or infectionand laboratories were only significant for a minor elevation in his AST and ALT.Histopathology of a punch biopsy specimen demonstrated a dense dermal neutro-philic infiltrate with papillary dermal edema, most consistent with Sweet syndrome,although atypical PG could not be ruled out. Direct immunofluroescence of aperilesional skin specimen was negative. He was started on prednisone 60 mg daily.On follow-up, many cutaneous lesions had cleared, but several of the lowerextremity lesions had developed central ulceration with violaceous underminededges. A repeat skin biopsy demonstrated ulceration, dermal necrosis, and vascu-litis, consistent with PG. Dapsone 50 mg daily was added to the treatment regimen,but thereafter the patient became lost to follow-up. To our knowledge, this examplerepresents the first reported case of a generalized presentation of atypical PG.Hematologic malignancy was ruled out in our patient given a normal completeblood count and differential. Although he described a history of recurrent abdom-inal pain, subsequent colonoscopy and upper endoscopy studies were negative. It isimportant to consider atypical PG as a diagnosis in patients with a generalizedpainful vesiculobullous Sweet syndromeelike eruption.
cial support: None identified.
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P6988A case of migratory panniculitis
Davide Altamura, MBBS, MD, Princess Alexandra Hospital, Harlow, UnitedKingdom; Ravinder Atkar, MBBS, Royal London Hospital, London, UnitedKingdom; Roberto Verdolini, MBBS, MD, Princess Alexandra Hospital, Harlow,United Kingdom
We report a case of a 32-year-old Hungarian man presented with a 2-year history ofrecurrent episodes of migratory swellings on lower legs and arms, including hispalms. He spontaneously developed multiple crescentic subcutaneous nodulesmigrating along the extremities and tending to a self-resolution in a period of 2 to 3weeks. The nodules were minimally inflamed, slightly painful, firm in consistency,and varying in size from 1 to 3 cm. There was associated fever, monoarthritis, sorethroat, and sometimes myalgia. The patient also reported a progressive weight lossand weakness. He had no medical and medication history of relevance and deniedprevious dermatologic diseases. An original diagnosis of erythema nodosum wassuspected. The full screening for panniculitis revealed mild anaemia, raised CRP andESR, with a raised ASO titre of 800 IU/mL. Subsequent throat swab was negative anda chest radiograph showed clear lungs. A differential diagnosis of rheumatic feverwas considered but the cardiovascular examination was unremarkable with noevidence of murmurs and normal ECG. A skin biopsy was performed and showed amixed lobular and septal panniculitis with a component of subcutaneous lympho-cytic vasculitis and plasmacells infiltrates. The clinical and histhopathologic findingswere suggestive of a subacute migratory panniculitis resembling Vilanova diseasebut lacking granulomas at the histology. The patient was commenced on doxycyline100mg o.d. and a 6-week follow-upwas arranged. At review, he reported not furtherepisodes of swellings and a significant reduction of systemic symptoms. Vilanovadisease, or subacute nodular migratory panniculitis, was described in 1954. There issome belief that Vilanova disease is a version of erythema nodosum because ofsimilar histology. No specific etiologic agent has been found, although streptococcalinfections and thyroid disease have been suggested. Clinical presentation is withtransient nodules affecting the extremities with minimal inflammation; over weeksor months, the nodules self-resolve or migrate and can sometimes becomecrescentic in nature. Some patients describe concurrent sore throat, fever orarthralgias at the onset. The condition is rare and usually self-resolving.
cial support: None identified.
CommerP6981A case of pseudolympoma caused by therapy with medicinal leeches
Ravinder Atkar, MBBS, Royal London Hospital, London, United Kingdom; DavideAltanura, MBBS, MD, Princess Alexandra Hospital, Essex, United Kingdom;Roberto Verdolini, MBBS, MD, Princess Alexandra Hospital, Essex, UnitedKingdom
A 54-year-old Lithuanian woman presented with a 2-month history of multiple firm,itchy, brown to red, excoriated papules and nodules on the back. The skin eruptionappeared a few weeks after she received a natural therapy for chronic fibromyalgiabased on the application of medicinal leeches. She referred without systemicsymptoms and a general blood test was unremarkable. On examination there wereno relevant lymphoadenopathies nor hepatosplenomegaly. A series of punchbiopsies taken from the nodules on the back indicated a probable diagnosis ofprimary cutaneous marginal B-cell lymphoma and the histology was subsequentlyconfirmed from a second analysis carried out at the Pathology Department of atertiary referral center based at a University Hospital in London. A clinical reviewwas arranged but at the time of the consultation the skin eruption was self-resolvingand the patient only complained of a residual itchiness in the body areas previouslyinvolved by the rash. At that point, after a further clinicopathologic correlation, amore likely diagnosis of cutaneous pseudolymphoma induced by leeches applied tothe skin was hypothesized and the patient started on topical steroids and closefollow-up. The pathology specimens were also sent for a additional opinion to anexpert dermatopathologist who agreed with the latest diagnosis. The patient wasfollowed on a monthly basis and she did not develop any recurrence of the skineruption as well as evidence of systemic lymphoma during the following 9 months.The medicinal leeches (Hirudo medicinalis) have been historically used as naturalbloodletting and used as treatment for circulation disturbances. Therapy withmedicinal leeches is now frequently applied in plastic surgery and in the manage-ment of chronic venous insufficiency. More rarely, leech therapy has been tried forosteoarthritis, muscular pains, or injuries. Pseudolymphoma refers to a group ofnoncancerous lymphocytic disorders that simulate malignant lymphomas in bothclinical and histological findings. It represants an inflammatory response to knownor unknown stimuli that leads in a lymphomatous appearing but benign accumu-lation of inflammatory cells. Only a few cases of cutaneous pseudolymphomainduced by the Hirudo medicinalis have been reported so far.
cial support: None identified.
CommerJ AM ACAD DERMATOL AB39