P6290A distinct oral phenotype in immunoglobulin A predominant mucousmembrane pemphigoid

Blaithin Moriarty, MBBCh, St. John’s Institue of Dermatology, London, UnitedKingdom; Jane F. Setterfield, MBBS, MD, Guys Dental Institute and St. John’sInstitute of Dermatology, Kings College, London, United Kingdom; Jonathan N.Leonard, MBBS, Imperial College Health care Trust, London, United Kingdom;Karen E. Harman, MD, Leicester Royal Infirmary, Leicestershire, United Kingdom;Stephen J. Challacombe, PhD, Guys Dental Institute, Kings College, London,United Kingdom

Mucous membrane pemphigoid (MMP) comprises a heterogeneous group ofpatients presenting with a predominantly mucosal disease and characterized bysubepithelial blistering and linear deposition of IgG, IgA, or C3 along the epithelialbasement membrane zone (BMZ). The First International Consensus statement onMMP published in 2002 unified the earlier terminology and also included patientswith linear deposition of IgA on direct immunofluorescence (DIF) presenting with amucosal predominant disease. We present a series of 8 patients with IgA predom-inant MMPall of whom demonstrated ulceration of the dorsal aspect of the tongue, afeature not usually seen in MMP. Patients (6 females, 2 males) with an age range of 40to 60 years presented to a tertiary referral oral Medicine and Dermatology centerover an 18-year period. In addition to midline dorsal tongue ulceration, 2 had buccalulceration, 2 had soft palate ulceration and 7 patients had extraoral involvement(other mucous membrane sites in 4 and skin lesions in 6 patients). All patients hadlinear deposition of IgA at the BMZ on either DIF (n ¼ 6) or indirect IF (n ¼ 5) withC3 detected on DIF in 2 patients. No patient had detectable IgG antibodies. Sevenpatients required systemic therapy (eg, prednisolone with or without dapsone orcyclophosphamide [n ¼ 2]) while 1 patient gained adequate disease control with asteroid mouthwash alone. While approximately 15% patients with MMP maydemonstrate lingual lesions, these are usually on the ventrolateral aspects of thetongue. Disorders of the dorsal tongue are unusual, and include atrophic glossitissecondary to nutritional deficiencies, median rhomboidal glossitis, pemphigus,infections and squamous cell carcinoma. Dorsal tongue ulceration has only beendescribed in 1 previous patient with MMP and this patient also demonstrated linearBMZ IgA. In summary, we present 8 patients with a distinct oral phenotype in IgApredominant mucous membrane pemphigoid. The localization to this site suggests asite specific target antigen or epitope reactive only with the IgA subclass ofautoantibodies.

APRIL 20

cial support: None identified.

Commer

P6386A successful single-cycle rituximab treatment in radiation-induced pem-phigus vulgaris with squamous cell carcinoma of the tongue and immu-noglobulin G antibodies to desmocollins

Jaehwan Kim, Department of Dermatology, St. George Hospital, Sydney,Australia; Dedee Murrell, Department of Dermatology, St. George Hospital,Sydney, Australia; Hiroshi Koga, Department of Dermatology, Kurume UniversitySchool of Medicine, Fukuoka, Japan; Takashi Hashimoto, Department ofDermatology, Kurume University School of Medicine, Fukuoka, Japan

Pemphigus can be triggered or induced by many insults, including drugs, bacterialand viral infections, and neoplasms. Radiation-induced pemphigus vulgaris accom-panied by an internal malignancy has been rarely reported, but the link betweenpemphigus vulgaris and malignancy still needs to be elucidated. Moreover,treatment of pemphigus in this condition is often challenging because the patient’simmune system has been already suppressed by radiation and chemotherapy. A 54-year-old man presented with suffering from blisters and ulcers on the oral mucosa,starting after radiotherapy for squamous cell carcinoma (SCC) of the tongue. Blistersand ulcers were developed on the tongue and back of throat, affecting eating andswallowing. Subsequently, he also developed blisters on the nose and both side ofthe face. He was diagnosed with SCC on the back of tongue 5 years ago. For thetreatment of SCC, he received chemotherapy with cisplatin and 35 sessions ofradiation therapy. Blisters on the skin and mucosa first developed followingradiotherapy 2 years ago. Pathologic and immunofluorescence studies of skinbiopsy indicated pemphigus vulgaris. IgG ELISA indices of anti-desmoglein 3 (Dsg3)antibodies of 146 and anti-Dsg1 of 3 were also consistent with mucosal dominantpemphigus vulgaris. Interestingly, IgG antibodies against desmocollins 1, 2, and 3were also detected in the sera of the patient. This result suggests that desmocollin,which plays an important role in tumor development or metastasis of SCC, alsoperforms a pathogenic role in the development of pemphigus vulgaris and explainsthe link between SCC and pemphigus vulgaris. Clinically, the skin lesions did notrespond to previous treatment with high-dose prednisone, azathioprine, cyclophos-phamide, and IVIG. Considering his comorbidities and recalcitrant symptoms, thepatient was treated with 4weekly infusions of 375mg of rituximab per square meterof body surface area together with prednisone 40 mg per day. After rituximabtreatment, the patient achieved 90% remission in 2 months. To prevent recurrence,40 mg of prednisone was sustained for 3 months and then gradually tapered. Thiscase suggests that a single cycle of rituximab is an effective treatment for radiation-induced recalcitrant pemphigus accompanied by an internal malignancy.

cial support: None identified.

Commer

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P6376An erythema multiformeelike presentation of Sweet syndrome

Julian Ngo, DO, Largo Medical Center, Largo, FL, United States

Acute febrile neutrophilic dermatosis, also known as Sweet syndrome, is anuncommon disease characterized by dense neutrophilic infiltrates within the skin.The pathogenesis of Sweet syndrome is unknown, and the clinical presentation canbe extensivewith lesions ranging from tender, nonpruritic, erythematous papules totargetoid, vesicular, and necrotic plaques. We present a case of 49-year-old womanwho developed erythematous, targetoid, vesicular eruption over a 3-day periodinvolving face, back, upper, and lower extremities. No mucosal involvement wasnoted on examination. Patient’s PMH was significant for HSV type 1, hepatitis C,COPD, and pulmonary HTN and she complained of mild pruritus. The patient wasgiven aworking diagnosis of erythema multiforme and was started on IV solumedroland valacyclovir. A punch biopsy revealed dermal edema with a dense infiltrate ofneutrophils consistent with Sweet syndrome. We present this case to emphasize thebroad range of clinical features seen in Sweet syndrome and highlight the uniquetargetoid vesicular eruptions seen in our patient.

cial support: None identified.

Commer

P6845Bullous pemphigoid and dermatitis herpetiformis overlap

Richard Limbert, DO, Largo Medical Center, Largo, FL, United States; RobynMcCullem, MD, Jefferson City Medical Group, Jefferson City, MO, United States;Ryan Hick, MD, ProPath Dermatopathology, Dallas, TX, United States

Bullous pemphigoid (BP) is an autoimmune blistering disorder characterized bytense, subepidermal bullae. Characteristic histopathologic findings of BP includeeosinophils in the blister cavity and direct immunofluorescence microscopy (DIF)demonstrating linear IgG deposition along the basement membrane zone (BMZ).Dermatitis herpetiformis (DH) is another autoimmune blistering disorder charac-terized by grouped papulovesicles. Characteristic histopathologic findings of DHinclude neutrophils in the blister cavity and DIF demonstrating granular IgAdeposition in the dermal papillae. Rarely reported, these 2 distinct diseases mayoverlap. Epitope spreading is a hypothesized explanation of the concurrent findingsof both diseases. We report a case of a 75-year-old man with excoriated and intactbullae on elbows, knees, legs, and hands. His medical history is positive for celiacdisease diagnosed 1 year previously. Histologic examination of the lesions revealedcharacteristics of both BP and DH.

cial support: None identified.

Commer

J AM ACAD DERMATOL AB111