P8001T-helper and regulatory T cell cytokines and their correlation withdesmoglein antibody levels in pemphigus vulgaris

Vinod Sharma, MD, All India Institute of Medical Sciences, New Delhi, India;Manoj Tembhre, MS, All India Institute of Medical Sciences, New Delhi, India

Background: Pemphigus vulgaris (PV) is an autoimmune skin blistering diseasetargeting to cell adhesionmolecules, such as desmoglein (Dsg), and characterized byintraepithelial lesions involving the skin and mucous membrane. The role ofautoreactive T cells and cytokines has been proposed in the etiopathomechanism ofpemphigus that is yet not completely understood. Moreover, correlation betweenserum antibodies to desmogleins and cytokines has not been studied.

Objectives: To investigate the status of serum T-helper and regulatory T cellcytokines in PVand their correlation with antibodies to Dsg-1 and Dsg-3 to establishcytokines as biomarkers.

Method: Forty patients with active pemphigus and 40 age- and sex-matched healthycontrol subjects were included in the study. Serum IL-2, IFN-g, IL-10, IL-13, IL-17A,and TGF-b1 were measured by enzyme linked immunosorbent assay in both thegroups. In addition, serum Dsg-1 and Dsg-3 levels were estimated in all the PVpatients and were correlated with serum cytokines.

Results: The concentration of IFN-g, IL-17A and TGF-b1 were found to besignificantly higher (P\.05) in PV compared to controls. No significant differencewas observed for IL-2, IL-10, and IL-13 (P[.05) in both groups. Significant positivecorrelation was found between Dsg3 levels and cytokines IFN-g and TGF-b1.

Conclusion: Increased levels of IFN-g and IL-17A and TGF-b1 indicated an alteredTh1, Th17, and Treg function. Positive correlation of Dsg-3 antibody with cytokinesIFN-g and TGF-b1 suggest an important role of these cytokines in the pathogenesisof pemphigus vulgaris.

MAY 201

cial support: None identified.

Commer

P8455Treatment of recalcitrant bullous pemphigoid with rituximab

Anthony Chiaravalloti, SUNY Upstate Medical University, Syracuse, NY, UnitedStates

A 39-year-old woman with severe bullous pemphigoid of 1.5 years’ duration wasreferred to our dermatology practice. Her disease had been refractory to multipleimmunosuppressive medications, including high-dose prednisone, methotrexate,mycophenolate mofetil, and azathioprine with significant morbidity of candidaesophagitis and osteopenia. Based on limited but promising data using rituximab forrecalcitrant bullous pemphigoid, we initiated treatment using doses previouslystudied for pemphigus vulgaris. After a total of 10 infusions over a 6-month periodour patient had a lasting remission without additional adjuvant therapy. This reportadds to the growing body of literature that demonstrates rituximab is an efficaciousapproach to refractory bullous pemphigoid. If considered earlier in our patient’sdisease course, significant medication-related morbidity may have been avoided. Werecommend that clinicians consider rituximab early in the course of the disease as anefficacious, steroid-sparing treatment option.

cial support: None identified.

Commer

4

P7608Vancomycin-induced linear IgA bullous dermatosis with isomorphicphenomenon

Samreen Choudhry, MD, Henry Ford Hospital, Department of Dermatology,Detroit, MI, United States; Henry W. Lim, MD, Henry Ford Hospital, Departmentof Dermatology, Detroit, MI, United States

Background: Linear IgA bullous dermatosis is a rare, immune-mediated subepider-mal vesiculobullous eruption that occurs because of the linear deposition of IgA inthe lamina lucida of the cutaneous basement membrane. Clinical features rangefrom erythematous plaques, blanching macules and papules, and targetoid lesions;however, the most classic presentation is tense bullae in a herpetiform arrangementsimulating a ‘‘cluster of jewels’’ or ‘‘string of beads’’ appearance.

Case report: A 60-year-old morbidly obese white female presented as a transfer froman outside hospital for a 2-week history of encephalopathy of unknown origin. Shewas found to have Clostridium difficile colitis and started on vancomycin and flagylfor 2 weeks before presentation to our hospital. Because of her poor nutritionalstatus, a percutaneous endoscopic gastrostomy (PEG) tube was placed. Onadmission to our hospital, she was noted to have annular and grouped flaccidbullae on the bilateral upper lateral thorax in midaxillary line, around PEG tube site,in a straight line across the right abdomen where her PEG tube was taped across herabdomen, and oral involvement. The next day, she had involvement of the anterioraspect of her neck where her nasal cannula was rubbing against her neck. Per herhusband, she had no previous history of skin problems. Extensive laboratory work-up for autoimmune, infectious, metabolic, and neurologic causes was significantonly for an elevated erythrocyte sedimentation rate (ESR) at 121 mm/hr andincreased IgA level at 534 mg/dL. A biopsy of one of the blisters revealed asubepidermal bulla with neutrophilic infiltrate at the base of the bulla. Directimmunofluorescence showed a 3+ linear IgA/C3 stain at the dermoepidermaljunction. The patient was diagnosed with vancomycin-induced linear IgA bullousdermatosis and vancomycin was stopped. She was treated with intravenousimmunoglobulin (IVIG) with improvement of her skin lesions, but continued tomedically deteriorate until she passed away on day 3 of IVIG treatment.

Discussion: To our knowledge, there have been 2 case reports in the literature ofisomorphic response seen in patients with drug-induced linear IgA dermatosis. It hasbeen thought that trauma and friction triggers an increase in local blood flow,bringing more autoantibodies to the site. The significance of this finding implies thatavoidance of trauma and gentle handling of such patients can lead to early recoveryfrom a self-limiting disease.

cial support: None identified.

Commer

INFECTION—BACTERIAL AND PARASITIC

P8644A case of mucocutaneous leishmaniasis

Aparna Tamirisa, MD, Center for Clinical Studies, Webster, TX, United States;Christopher Downing, MD, Center for Clinical Studies, Webster, TX, UnitedStates; Farhan Khan, MD, MBA, Center for Clinical Studies, Houston, TX, UnitedStates; Stephen Tyring, MD, PhD, Dermatologic Association of Texas, Webster,TX, United States

Introduction: Leishmaniasis diagnosed in the United States is usually acquiredoutside of the country. We discuss a case of leishmaniasis acquired on a trip toVenezuela.

Case report: A 32-year-oldmale presentedwith lesions of the right neck and chin andleft nasal ala. On examination, there were multiple erythematous and induratedpapules of the right neck, erythema and swelling of the left nasal ala, and disruptionof the nasal architecture with crusting of the left nare. The lesions began to developafter he visited the Venezuelan rainforests. He was treated with several antibioticswithout benefit. Repeated biopsies of the left neck showed pseudoepitheliomatoushyperplasia with seroinflammatory crust and prominent lymphoplasmocyticinflammation with no growth of microorganisms on staining. Of note, the Giemsastain was negative for leishmania. Ultimately, a biopsy sample was sent to the CDCfor tissue culture and PCR analysis which was positive for leishmania. The plan wasto treat the patient with liposomal amphotericin B.

Discussion: Mucocutaneous leishmaniasis is endemic in northern South Americaand is associated most commonly with Leishmania braziliensis. Forest rodents arethe reservoirs for L braziliensis, and the transmission vector to humans is thesandfly. Leishmaniasis is most common between 20 to 40 years of age. Diagnosis inthe US is usually from disease acquired outside of the country, and the primaryendemic area in the US for leishmaniasis is south central Texas. The clinicalmanifestations of mucocutaneous leishmaniasis range from edema of the lips andnose to perforation of the cartilage of the nose and larynx. The characteristic tapirface is a result of extensive tissue loss in the mouth and nose, as with this patient.The histologic findings include areas of ulceration with pseudoepitheliomatoushyperplasia and inflammatory infiltrate. Tissue culture is positive in approximately40% of cases. The PCR assay method is considered to be the most sensitive andspecific diagnostic test. The differential for mucocutaneous leishmaniasis is para-coccidiomycosis and tertiary syphilis. Wegener granulomatosis and NK T-celllymphoma should be considered for lesions affecting the central face. Cutaneousdisease is treated to accelerate healing so as to avoid excess scarring and to preventdissemination or relapse of disease.

cial support: None identified.

Commer

J AM ACAD DERMATOL AB99