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Severe erythema multiforme responding to high dose intravenous immunoglobulin therapy1
P362
P364
NORMAL ANAGEN EFFLUVIUM AS THE PRESENTING SIGN OF PEMPHIGUS VULGARIS: A CASE REPORT Mi-Yeon Kim, MD, Department of Dermatology, Kangnam St. Mary’s Hospital, College of Medicine, the Catholic University of Korea, Seoul, Korea, You Jeong Kim, MD, Young Min Park, MD, PhD, Hyung Ok Kim, MD, PhD Pemphigus vulgaris is an autoimmune blistering diseases of skin and mucous membranes characterized histologically by intraepidermal blisters and immunopathologically by the bound and circulating IgG directed to Dsg3 and, in part, Dsg1 of the epithelial desmosomes. In majority of patients, painful mucous membrane erosions, especially in oral cavity, are the presenting sign, and scalp involvement as a initial presentation is reported to occupy 3-6% in pemphigus vulgaris. Hair follicle is one of the preferential targets for pemphigus autoantibodies as the desmosomal proteins are overexpressed in follicular epithelium. Those proteins are especially distributed throughout the outer root sheath of the whole hair follicle and in the dermal bulb matrix cell and take charge of attachment of hair. A 47-year-old Korean man presented with crusted non-scarring alopecic patches on the scalp for 1 month and small blisters on the trunk and upper extremities for 1 week. Hairs were easily plucked from perilesional area and plucked hairs looked like normal anagen hairs with intact root sheaths. Histopathologic examination revealed acantholysis of basal layer in epidermis by the manner of “row of tombstones” and clefts in outer root sheathes of hair follicles. Direct immunifluorescence test, indirect immunofluorescence test and immunoblotting test were consistent with pemphigus vulgaris. This case shows normal anagen effluvium as well as painful oral mucous membrane erosions can be a presenting signs of pemphigus vulgaris.
SEVERE ERYTHEMA MULTIFORME RESPONDING TO HIGH DOSE INTRAVENOUS IMMUNOGLOBULIN THERAPY Minal N Singh, MBChB, The Dermatology Centre, University of Manchester, Hope Hospital, Manchester, England, Jason Williams, MBChB, The Dermatology Centre, University of Manchester, Hope Hopsital, Manchester, England, Tamara Griffiths, MD, Department of Dermatology, Macclesfield General Hospital, Macclesfield, England, Chris Griffiths, MD, The Dermatology Centre, University of Manchester, Hope Hospital, Manchester, England A 37 year old woman was admitted with a four year history of severe, persistent bullous erythema multiforme (EM), predominantly affecting the distal limbs, but also with oral changes. No precipitating factor had been elucidated, though the initial onset was following Tylex ingestion (paracetamol and codeine) and there was a vague history of preceding herpes simplex infection. Histology (2000) confirmed the diagnosis as EM and immunofluorescence was negative. The lesions persisted despite treatment with a prolonged course of oral aciclovir and dapsone, whilst azathioprine resulted in hepatitis. Ciclosporin (3.7mg/kg/day) was ineffective and the patient was still requiring oral corticosteroids for flares. The patient was unable to tolerate mycophenolate mofetil due to severe nausea and vomiting. On admission the patient had extensive well-demarcated areas of symmetrical, superficial erosion on the limbs, along with target lesions and blisters affecting 10% body surface area. There was oral mucosa involvement. She continued to develop new lesions during admission prior to treatment. In view of the recalcitrant nature and severity of the patient’s condition, she was treated with high dose IVIG. Immunoglobulin levels were checked and the patient was treated with IVIG (0.4g/kg/day) for 5 days. The patient tolerated the treatment well, having only mild pyrexia during infusions. There were no significant adverse effects noted. At the time of discharge, there had been an 80% improvement in erosions and blistering and the patient was requiring significantly less analgesia. The condition flared at 6 months requiring further IVIG therapy. This resulted in a similar clinical improvement. Persistent EM is characterised by an uninterrupted occurrence of both typical and atypical lesions, which are widespread and commonly papulonecrotic or bullous. Intravenous immunoglobulin (IVIG) therapy has been used in the successful treatment of StevensJohnson syndrome. IVIG has been suggested as a treatment for EM, but the only published report of its use is in a cat. As far as we are aware, this is the first report of the successful use of human IVIG in the treatment of EM. IVIG appears to be an excellent therapeutic option in cases of severe persistent EM.
Disclosure not available at press time.
Disclosure not available at press time.
P363 TRIETHANOLAMINE INDUCED LINEAR IGA DERMATOSIS Molly Smith, MD, Eastern Virginia Medical School, Norfolk, VA, United States, Judith Williams, MD, Eastern Virginia Medical School, Norfolk, VA, United States A previously healthy 10-year-old boy was assisting his mother in removing wallpaper using aerosolized “EZ-Strip” in a non-ventilated bedroom. The following day he developed mild upper respiratory symptoms and blistering on his right arm. One week later, he developed conjunctival injection and blistering near his medial canthus, then oral mucosal lesions, tense bullae on his palms and soles and fragile bullae in the axilla and groin. His mother, who participated in the wallpaper removal, remained asymptomatic. He was hospitalized and treated for presumptive coxsackievirus infection and staphylococcal superinfection. While in the hospital, his liver enzymes were noted to be elevated, and he developed more extensive mucosal and skin involvement, with blisters spreading onto his trunk. A pediatric dermatology consult was requested and skin biopsies were obtained. Histologic examination of biopsies taken from his axilla showed subepidermal bulla with full thickness necrosis of the epidermis and a perivascular eosinophilic and lymphocytic infiltrate extending into the mid-reticular dermis. Immunofluorescence demonstrated 3⫹ linear IgA and C3 deposition along the basement membrane zone. There were no circulating antibodies detected in his serum. Linear IgA bullous dermatoses (LABD) is an autoimmune blistering disease that occurs spontaneously, following drug exposure or in association with various malignancies. Triethanolamine has been shown to cause IgE mediated responses and contact allergic reactions, but there has been no report of a blistering disorder or drug hypersensitivity syndrome. Reports of a blistering dermatitis have been seen in Asians exposed to other solvents, such as trichloroethylene, with a possible link to HLA type. We present a case of LABD in a child with an Asian father and Caucasian mother following physical exposure to triethanolamine, the main component of a particular wallpaper stripping solution. Our patient improved with prednisone and dapsone, without signs of recurrence to date.
PARANEOPLASTIC PEMPHIGUS ASSOCIATED WITH SQUAMOUS CELL CARCINOMA OF THE LUNG Liborka Kos, MD, Henry Ford Hospital, Detroit, MI, United States, Henry Wong, MD, Henry Ford Hospital, Detroit, MI, United States, Adrian Ormsby, MD, Henry Ford Hospital, Detroit, MI, United States Paraneoplastic pemphigus (PNP) is an autoimmune bullous disease characterized by polymorphic cutaneous lesions, severe mucous membrane involvement and an underlying neoplasm. The most commonly associated neoplasms are lymphoproliferative malignancies. Associations with solid cancers are rare. We describe a case of a 77-year-old man presenting with confusion and syncope who was found to have severe stomatitis, flaccid bullae and widespread erosions on hospital admission. A routine chest x-ray revealed a lung mass which was proven to be a squamous cell carcinoma. Histologic examination and direct and indirect immunofluorescence analysis of skin biopsy specimens confirmed the diagnosis of PNP. Our case demonstrates an unusual association of PNP with a squamous cell carcinoma of the lung.
Disclosure not available at press time.
Disclosure not available at press time.
MARCH 2004
P365
J AM ACAD DERMATOL
P95
P364
NORMAL ANAGEN EFFLUVIUM AS THE PRESENTING SIGN OF PEMPHIGUS VULGARIS: A CASE REPORT Mi-Yeon Kim, MD, Department of Dermatology, Kangnam St. Mary’s Hospital, College of Medicine, the Catholic University of Korea, Seoul, Korea, You Jeong Kim, MD, Young Min Park, MD, PhD, Hyung Ok Kim, MD, PhD Pemphigus vulgaris is an autoimmune blistering diseases of skin and mucous membranes characterized histologically by intraepidermal blisters and immunopathologically by the bound and circulating IgG directed to Dsg3 and, in part, Dsg1 of the epithelial desmosomes. In majority of patients, painful mucous membrane erosions, especially in oral cavity, are the presenting sign, and scalp involvement as a initial presentation is reported to occupy 3-6% in pemphigus vulgaris. Hair follicle is one of the preferential targets for pemphigus autoantibodies as the desmosomal proteins are overexpressed in follicular epithelium. Those proteins are especially distributed throughout the outer root sheath of the whole hair follicle and in the dermal bulb matrix cell and take charge of attachment of hair. A 47-year-old Korean man presented with crusted non-scarring alopecic patches on the scalp for 1 month and small blisters on the trunk and upper extremities for 1 week. Hairs were easily plucked from perilesional area and plucked hairs looked like normal anagen hairs with intact root sheaths. Histopathologic examination revealed acantholysis of basal layer in epidermis by the manner of “row of tombstones” and clefts in outer root sheathes of hair follicles. Direct immunifluorescence test, indirect immunofluorescence test and immunoblotting test were consistent with pemphigus vulgaris. This case shows normal anagen effluvium as well as painful oral mucous membrane erosions can be a presenting signs of pemphigus vulgaris.
SEVERE ERYTHEMA MULTIFORME RESPONDING TO HIGH DOSE INTRAVENOUS IMMUNOGLOBULIN THERAPY Minal N Singh, MBChB, The Dermatology Centre, University of Manchester, Hope Hospital, Manchester, England, Jason Williams, MBChB, The Dermatology Centre, University of Manchester, Hope Hopsital, Manchester, England, Tamara Griffiths, MD, Department of Dermatology, Macclesfield General Hospital, Macclesfield, England, Chris Griffiths, MD, The Dermatology Centre, University of Manchester, Hope Hospital, Manchester, England A 37 year old woman was admitted with a four year history of severe, persistent bullous erythema multiforme (EM), predominantly affecting the distal limbs, but also with oral changes. No precipitating factor had been elucidated, though the initial onset was following Tylex ingestion (paracetamol and codeine) and there was a vague history of preceding herpes simplex infection. Histology (2000) confirmed the diagnosis as EM and immunofluorescence was negative. The lesions persisted despite treatment with a prolonged course of oral aciclovir and dapsone, whilst azathioprine resulted in hepatitis. Ciclosporin (3.7mg/kg/day) was ineffective and the patient was still requiring oral corticosteroids for flares. The patient was unable to tolerate mycophenolate mofetil due to severe nausea and vomiting. On admission the patient had extensive well-demarcated areas of symmetrical, superficial erosion on the limbs, along with target lesions and blisters affecting 10% body surface area. There was oral mucosa involvement. She continued to develop new lesions during admission prior to treatment. In view of the recalcitrant nature and severity of the patient’s condition, she was treated with high dose IVIG. Immunoglobulin levels were checked and the patient was treated with IVIG (0.4g/kg/day) for 5 days. The patient tolerated the treatment well, having only mild pyrexia during infusions. There were no significant adverse effects noted. At the time of discharge, there had been an 80% improvement in erosions and blistering and the patient was requiring significantly less analgesia. The condition flared at 6 months requiring further IVIG therapy. This resulted in a similar clinical improvement. Persistent EM is characterised by an uninterrupted occurrence of both typical and atypical lesions, which are widespread and commonly papulonecrotic or bullous. Intravenous immunoglobulin (IVIG) therapy has been used in the successful treatment of StevensJohnson syndrome. IVIG has been suggested as a treatment for EM, but the only published report of its use is in a cat. As far as we are aware, this is the first report of the successful use of human IVIG in the treatment of EM. IVIG appears to be an excellent therapeutic option in cases of severe persistent EM.
Disclosure not available at press time.
Disclosure not available at press time.
P363 TRIETHANOLAMINE INDUCED LINEAR IGA DERMATOSIS Molly Smith, MD, Eastern Virginia Medical School, Norfolk, VA, United States, Judith Williams, MD, Eastern Virginia Medical School, Norfolk, VA, United States A previously healthy 10-year-old boy was assisting his mother in removing wallpaper using aerosolized “EZ-Strip” in a non-ventilated bedroom. The following day he developed mild upper respiratory symptoms and blistering on his right arm. One week later, he developed conjunctival injection and blistering near his medial canthus, then oral mucosal lesions, tense bullae on his palms and soles and fragile bullae in the axilla and groin. His mother, who participated in the wallpaper removal, remained asymptomatic. He was hospitalized and treated for presumptive coxsackievirus infection and staphylococcal superinfection. While in the hospital, his liver enzymes were noted to be elevated, and he developed more extensive mucosal and skin involvement, with blisters spreading onto his trunk. A pediatric dermatology consult was requested and skin biopsies were obtained. Histologic examination of biopsies taken from his axilla showed subepidermal bulla with full thickness necrosis of the epidermis and a perivascular eosinophilic and lymphocytic infiltrate extending into the mid-reticular dermis. Immunofluorescence demonstrated 3⫹ linear IgA and C3 deposition along the basement membrane zone. There were no circulating antibodies detected in his serum. Linear IgA bullous dermatoses (LABD) is an autoimmune blistering disease that occurs spontaneously, following drug exposure or in association with various malignancies. Triethanolamine has been shown to cause IgE mediated responses and contact allergic reactions, but there has been no report of a blistering disorder or drug hypersensitivity syndrome. Reports of a blistering dermatitis have been seen in Asians exposed to other solvents, such as trichloroethylene, with a possible link to HLA type. We present a case of LABD in a child with an Asian father and Caucasian mother following physical exposure to triethanolamine, the main component of a particular wallpaper stripping solution. Our patient improved with prednisone and dapsone, without signs of recurrence to date.
PARANEOPLASTIC PEMPHIGUS ASSOCIATED WITH SQUAMOUS CELL CARCINOMA OF THE LUNG Liborka Kos, MD, Henry Ford Hospital, Detroit, MI, United States, Henry Wong, MD, Henry Ford Hospital, Detroit, MI, United States, Adrian Ormsby, MD, Henry Ford Hospital, Detroit, MI, United States Paraneoplastic pemphigus (PNP) is an autoimmune bullous disease characterized by polymorphic cutaneous lesions, severe mucous membrane involvement and an underlying neoplasm. The most commonly associated neoplasms are lymphoproliferative malignancies. Associations with solid cancers are rare. We describe a case of a 77-year-old man presenting with confusion and syncope who was found to have severe stomatitis, flaccid bullae and widespread erosions on hospital admission. A routine chest x-ray revealed a lung mass which was proven to be a squamous cell carcinoma. Histologic examination and direct and indirect immunofluorescence analysis of skin biopsy specimens confirmed the diagnosis of PNP. Our case demonstrates an unusual association of PNP with a squamous cell carcinoma of the lung.
Disclosure not available at press time.
Disclosure not available at press time.
MARCH 2004
P365
J AM ACAD DERMATOL
P95