a case of generalized atypical pyoderma gangrenosum mimicking sweet syndrome

1
P6920 A 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, United States We describe a 34-year-old morbidly obese African American man with a history of type 2 diabetes mellitus, congestive heart failure, pulmonary embolus (on coumadin), 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 nodules on the medial thighs bilaterally. Several draining nodules notably extruded a thin white, milky fluid. The lesions started on his lower abdomen approximately 1 year before evaluation, where he had injected enoxaparin subcutaneously for 4 days. Six months afterward, he noted similar lesions on his medial midthighs bilaterally in the absence of trauma. Skin biopsies obtained from the right thigh and left upper arm (processed in formalin) showed changes consistent with tophaceous gout in the dermis. An outside provider prescribed allopurinol 300 mg by mouth twice daily and a prednisone taper several weeks before our evaluation, but the patient reported no improvement in the lesions or symptoms. Laboratory evaluation was significant for creatinine of 1.4 to 1.9 mg/dL and normal serum uric acid level. The patient reported no 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 gout typically presents with aggregates of negatively birefringent monosodium urate crystals in the periarticular regions and ear helices several years after the onset of gouty arthritis. Few case reports have described involvement of other areas, such as the legs and breast. Rare manifestations of gout also include tender panniculitis with indurated subcutaneous plaques and miliarial gout involving multiple small papules containing a white material on the skin. Our case has clinical features of both panniculitic and miliarial gout in the absence of arthritis and hyperuricemia. The etiology 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. Though rare, this case highlights the importance of considering atypical cutaneous gout in the differential of deposition disorders. Commercial support: None identified. P6208 A case of generalized atypical pyoderma gangrenosum mimicking Sweet syndrome Venessa Pena-Robichaux, MD, Henry Ford Health System, Detroit, MI, United States; Christopher Burnett, MD, Henry Ford Health System, Detroit, MI, United States Pyoderma gangrensoum (PG) is an uncommon neutrophilic dermatosis that usually presents as chronic recurrent ulcerative lesions with a distinct clinical appearance. Atypical (bullous) PG is a variant of this disease whose clinical appearance is similar and often confused with the superficial bullous variant of Sweet syndrome given their morphologic and histopathologic similarities. Atypical PG, unlike classic PG, most commonly presents with painful cutaneous lesions on the face and upper extremities (typically the dorsal surfaces of the hands) and is more closely associated with 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 the hospital with an acute, painful vesiculobullous eruption that started on the dorsal surfaces 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 infection and 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 a perilesional skin specimen was negative. He was started on prednisone 60 mg daily. On follow-up, many cutaneous lesions had cleared, but several of the lower extremity lesions had developed central ulceration with violaceous undermined edges. 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 example represents the first reported case of a generalized presentation of atypical PG. Hematologic malignancy was ruled out in our patient given a normal complete blood count and differential. Although he described a history of recurrent abdom- inal pain, subsequent colonoscopy and upper endoscopy studies were negative. It is important to consider atypical PG as a diagnosis in patients with a generalized painful vesiculobullous Sweet syndromeelike eruption. Commercial support: None identified. P6988 A case of migratory panniculitis Davide Altamura, MBBS, MD, Princess Alexandra Hospital, Harlow, United Kingdom; Ravinder Atkar, MBBS, Royal London Hospital, London, United Kingdom; 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 of recurrent episodes of migratory swellings on lower legs and arms, including his palms. He spontaneously developed multiple crescentic subcutaneous nodules migrating along the extremities and tending to a self-resolution in a period of 2 to 3 weeks. The nodules were minimally inflamed, slightly painful, firm in consistency, and varying in size from 1 to 3 cm. There was associated fever, monoarthritis, sore throat, and sometimes myalgia. The patient also reported a progressive weight loss and weakness. He had no medical and medication history of relevance and denied previous dermatologic diseases. An original diagnosis of erythema nodosum was suspected. The full screening for panniculitis revealed mild anaemia, raised CRP and ESR, with a raised ASO titre of 800 IU/mL. Subsequent throat swab was negative and a chest radiograph showed clear lungs. A differential diagnosis of rheumatic fever was considered but the cardiovascular examination was unremarkable with no evidence of murmurs and normal ECG. A skin biopsy was performed and showed a mixed lobular and septal panniculitis with a component of subcutaneous lympho- cytic vasculitis and plasmacells infiltrates. The clinical and histhopathologic findings were suggestive of a subacute migratory panniculitis resembling Vilanova disease but lacking granulomas at the histology. The patient was commenced on doxycyline 100 mg o.d. and a 6-week follow-up was arranged. At review, he reported not further episodes of swellings and a significant reduction of systemic symptoms. Vilanova disease, or subacute nodular migratory panniculitis, was described in 1954. There is some belief that Vilanova disease is a version of erythema nodosum because of similar histology. No specific etiologic agent has been found, although streptococcal infections and thyroid disease have been suggested. Clinical presentation is with transient nodules affecting the extremities with minimal inflammation; over weeks or months, the nodules self-resolve or migrate and can sometimes become crescentic in nature. Some patients describe concurrent sore throat, fever or arthralgias at the onset. The condition is rare and usually self-resolving. Commercial support: None identified. P6981 A case of pseudolympoma caused by therapy with medicinal leeches Ravinder Atkar, MBBS, Royal London Hospital, London, United Kingdom; Davide Altanura, MBBS, MD, Princess Alexandra Hospital, Essex, United Kingdom; Roberto Verdolini, MBBS, MD, Princess Alexandra Hospital, Essex, United Kingdom 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 eruption appeared a few weeks after she received a natural therapy for chronic fibromyalgia based on the application of medicinal leeches. She referred without systemic symptoms and a general blood test was unremarkable. On examination there were no relevant lymphoadenopathies nor hepatosplenomegaly. A series of punch biopsies taken from the nodules on the back indicated a probable diagnosis of primary cutaneous marginal B-cell lymphoma and the histology was subsequently confirmed from a second analysis carried out at the Pathology Department of a tertiary referral center based at a University Hospital in London. A clinical review was arranged but at the time of the consultation the skin eruption was self-resolving and the patient only complained of a residual itchiness in the body areas previously involved by the rash. At that point, after a further clinicopathologic correlation, a more likely diagnosis of cutaneous pseudolymphoma induced by leeches applied to the skin was hypothesized and the patient started on topical steroids and close follow-up. The pathology specimens were also sent for a additional opinion to an expert dermatopathologist who agreed with the latest diagnosis. The patient was followed on a monthly basis and she did not develop any recurrence of the skin eruption as well as evidence of systemic lymphoma during the following 9 months. The medicinal leeches (Hirudo medicinalis) have been historically used as natural bloodletting and used as treatment for circulation disturbances. Therapy with medicinal leeches is now frequently applied in plastic surgery and in the manage- ment of chronic venous insufficiency. More rarely, leech therapy has been tried for osteoarthritis, muscular pains, or injuries. Pseudolymphoma refers to a group of noncancerous lymphocytic disorders that simulate malignant lymphomas in both clinical and histological findings. It represants an inflammatory response to known or unknown stimuli that leads in a lymphomatous appearing but benign accumu- lation of inflammatory cells. Only a few cases of cutaneous pseudolymphoma induced by the Hirudo medicinalis have been reported so far. Commercial support: None identified. APRIL 2013 JAM ACAD DERMATOL AB39

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Page 1: A case of generalized atypical pyoderma gangrenosum mimicking Sweet syndrome

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.

APRIL 20

cial support: None identified.

Commer

P6208A 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.

Commer

13

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.

Commer

P6981A 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.

Commer

J AM ACAD DERMATOL AB39