P6784Granulomatous variant of scleromyxedema mimicking a drug reaction

Julia Shlyankevich, University of Washington School of Medicine, Seattle, WA,United States; Evan George, MD, University of Washington Medicine Departmentof Pathology, Seattle, WA, United States; Galina Stetsenko, MD, University ofWashington Medicine Department of Medicine, Division of Dermatology, Seattle,WA, United States; Jay Vary, Jr., MD, PhD, University of Washington MedicineDepartment of Pathology, Seattle, WA, United States

Scleromyxedema is a rare and progressive disease characterized by generalizedwaxy papules and skin induration. Cardinal histologic features include increasedstellate fibroblasts, thickened collagen and mucin deposition. We present a caseof a 54-year-old man with an erythematous, pruritic rash of 3 months duration,involving the face, upper extremities, and torso originally thought to be due to adrug eruption after the initiation of allopurinol. After discontinuation of thismedication, his eruption persisted and worsened. On presentation to our clinic,the patient reported a 4-month history of skin redness and tightening as well aspapules on the forehead, neck, back, torso, and arms. He endorsed a decreasedability to open his mouth because of stiffness of the skin, intermittent swelling ofthe abdomen, difficulty breathing, and hand and finger swelling. His medicalhistory was significant for Crohn’s disease in remission, hypertension, and Gravesdisease status postablation on thyroid replacement. Physical examinationrevealed exaggerated wrinkling of the glabella, indurated skin on the sides ofthe nose, upper back and shoulders, and numerous 2- to 3-mm waxy papulesdiffusely scattered over the upper shoulders. Histologic examination of thelesional skin revealed increased deposition of collagen fibers, increased numbersof histiocytes with epithelioid and spindled cytology and dermal mucin depositsalong with sparse perivascular lymphocytic inflammation. Laboratory evaluationwas significant for monoclonal IgG and IgA lambda gammopathy on serumelectrophoresis. Clinical findings of scleromyxedema with histologic findings ofinterstitial granulomatous changes with dermal mucinosis and monoclonalgammopathy fit best with a diagnosis of the granulomatous variant of sclero-myxedema. The patient was initiated on lenalidomide and dexamethasone, assimilar therapies directed at an underlying gammopathy have shown efficacy inpatients with scleromyxedema. Although exceedingly rare, the findings in ourpatient lend further support to the existence of a granulomatous scleromyxe-dema variant and this diagnosis should be considered when faced with similarclinical and histologic findings.

AB86

cial support: None identified.

Commer

P6989Histiocytoid Sweet syndrome in a patient with myelofibrosis dominantmyelodysplastic syndrome

Sue May Ang, MBBS, Sheffield Teaching Hospital, Sheffield, United Kingdom; C.B. Chong, Chesterfield Royal Hospital, Calow, United Kingdom; DaneshTaraporewalla, Chesterfield Royal Hospital, Calow, United Kingdom; RobertCutting, Chesterfield Royal Hospital, Derbyshire, United Kingdom; VinodElangasinghe, Chesterfield Royal Hospital, Derbyshire, United Kingdom

We report a case of MDS-associated HSS in a 72-year-old man. He presented with an8-year history of a progressive generalised rash that has only responded tointermittent oral steroids. This asymptomatic rash can be described as a mixtureof multiple, urticated, erythematous purplish pink and oval shaped nodules (newlesions) and pinkish brown pigmented oval macules (old lesions). They werediffusely distributed all over his body and the macular lesions failed to demonstratethe Darier sign. He had no lymphadenopathy or hepatosplenomegaly. The diagnosisof myelofibrosis dominant MDSwasmade 10 years ago as an incidental finding whenhe was investigated for angina. His MDS had been managed with blood transfusionsbecause of lethargy and weakness. Few years agowhen he had presented with a lessprominent rash it had been treated as Jessner lymphocytic infiltrate because of theinconclusive biopsy findings. The Sweetoid clinical appearance at presentation to usprompted a repeat biopsy. Histology showed a dermal infiltrate of ‘‘histiocytelooking’’ cells that stained CD68 andmyeloperoxidase (MPO) but not S100.With thecharacteristic rash, histology and underlying MDS our patient met the criteria for thediagnosis of HSS. HSS is believed to be a variant of SS. Purplish pink juicy nodules andplaques are the characteristic feature of SS whereas urtication is the key differen-tiating feature in lesions of HSS. This is further supported by histology as the dermalinfiltrate in SS is made up of mature neutrophils whereas CD68, MPO staininghistiocytes form the bulk of the infiltrate in HSS. HSS has been reported inassociation with MDS and myeloproliferative disorders. Treatment of the underlyinghematologic malignancy resolved the rash in most cases. Rare cases of HSS withMDS/MPD linking to Bortezomib, cryofibrinogenemia and pseudotumor/myxomahave been reported. Our patient has had few episodes of cutaneous vasculitis, hadbeen on warfarin followed by scenocoumarol for AF for 6 to 7 years and hadpresented to the urologist for suspected Peyronie disease of his penis last year. Thesignificance of these clinical elements in our case can be linked to the previous casereports but needs further understanding of this haematological phenomenon whichmay be linked to a possible B cell dyscrasia. This case highlights key clinical featuresof rare HSS and provides new insights into its aetiopathogenesis.

cial support: None identified.

Commer

J AM ACAD DERMATOL

P6797Hyperpigmentation associated with intradermal tranexamic acid injec-tions for treatment of melasma

Richard Jamison, MD, Scott and White Healthcare; The Texas A&M HealthScience Center College of Medicine, Temple, TX, United States; John Greene, Jr,MD, Scott and White Healthcare; The Texas A&M Health Science Center Collegeof Medicine, Temple, TX, United States; Palak Parekh, MD, Scott and WhiteHealthcare; The Texas A&M Health Science Center College of Medicine, Temple,TX, United States

Melasma is a relatively common pigmentary disorder that remains a difficultcondition to treat. A newer therapy that was initially reported in 2006 describedintradermal injection of tranexamic acid as a promising approach for treatment ofmelasma. We report the first known case of melasma treated with intradermaltranexamic acid that resulted in disfiguring hyperpigmentation. Histologic evalua-tion of this case revealed a dual staining pattern of melanin and iron. We concludethis is a result of drug metabolite-protein-iron complexes similar to that seen in typeII minocycline hyperpigmentation. Clinicians should be aware of the possibleparadoxical hyperpigmentation that may occur with intradermal tranexamic acidinjection for the treatment of melasma.

cial support: None identified.

Commer

P6355Immunohistochemical staining in cutaneous marginal zone B-celllymphoma

Stephanie Frisch, MD, Saint Louis University, Saint Louis, MO, United States;Claudia Vidal, MD, PhD, Saint Louis University Department of Dermatology, SaintLouis, MO, United States; Eric Armbrecht, PhD, Saint Louis University, SaintLouis, MO, United States; Yadira Hurley, MD, CAE, Saint Louis UniversityDepartment of Dermatology, Saint Louis, MO, United States

Background: Cutaneous marginal zone B-cell lymphomas share histopathologic andimmunophenotypic characteristics with splenic and nodal marginal zone B celllymphomas. CD43 in particular has been studied in cutaneous, splenic, and nodalmarginal zone B cell lymphomas and its positivity in skin has been suggestive ofmalignancy. Staining of CD43 in all types is variable, some reporting about 50%.CD23 and CD30 have rarely been studied in cutaneous marginal zone B-celllymphomas.

Objective: We sought to compare immunohistochemical markers seen in splenicand nodal types of marginal zone B-cell lymphoma with cutaneous marginal zone B-cell lymphoma as well as evaluate CD30 positivity.

Methods: We performed a retrospective chart review of our computerized dermat-opathology lab data base searching for all reports with ‘‘marginal zone lymphoma.’’Cutaneous marginal zone B cell lymphoma cases that were available for additionalstaining were collected and if not already done CD23, CD30, and CD43 wereperformed.

Results: Of the cases queried, 8 cases were identified as marginal zone B-celllymphoma available for additional staining. Two dermatopathologists reviewed thecases for consistency. Of the 8 cases, 7 were partial (10-50%) or completely positive([50%) for CD43. All 8 cases were negative for CD30. CD23 was negative in 3 cases,partially positive in 2 cases, and completely positive in 3 cases.

Limitations: We evaluated only 8 cases of this entity. The result of immunohisto-chemical stains may be affected by deeper tissue sectioning. It is difficult to evaluatethe significance of partial positive staining.

Conclusion: Variable CD43 staining in the skin is consistent with spleen and lymphnode types as well as other reports of cutaneous marginal zone B-cell lymphomas.CD30 is negative in all cases. There is no clear trend or pattern with CD23 staining.

cial support: None identified.

Commer

APRIL 2013