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Linear IgA dermatosis in Tunisian children: 35 cases
P1608
P1610
Linear IgA dermatosis in Tunisian children: 35 cases Monia Kharfi, PhD, Department of Dermatology. Charles Nicolle Hospital, Tunis, Tunisia, Hana Mhalla, Department of Dermatology. Charles Nicolle Hospital, Tunis, Tunisia, Amel Karaa, MD, Department of Dermatology. Charles Nicolle Hospital, Tunis, Tunisia, Mohamed Ridha Kamoun, PhD, Department of Dermatology. Charles Nicolle Hospital, Tunis, Tunisia
Paraneoplastic pemphigus without an underlying neoplasm Jee Bum Lee, MD, PhD, Department of Dermatology, Chonnam University Medical School, Gwang Ju, South Korea; Sook Jung Yun, MD, PhD, Department of Dermatology, Chonnam University Medical School, Gwang Ju, South Korea; Seung Chul Lee, MD, PhD, Department of Dermatology, Chonnam University Medical School, Gwang Ju, South Korea; Hyoung Ryun Park, PhD, Department of Chemistry, Chonnam National University, Gwang Ju, South Korea We describe a 52-year-old man with paraneoplastic pemphigus (PNP) without any evidence of an underlying neoplasm over an 8-year follow-up period. He had suffered from a chronic relapsing vesiculobullous eruption for approximately 7 years (from April 1998 to May 2005). Initially, scattered flaccid vesicles with crusts developed on the face and trunk, which waxed and waned several times. This case was diagnosed as PNP based on immunopathological PNP criteria (ie, histopathologic, immunoblotting, and immunoprecipitation analyses). However, physical and laboratory examinations, including serial blood tests with peripheral blood smear, whole body positron emission tomography/computed tomography, and ultrasonography were unable to detect any underlying neoplasm over an 8-year follow-up period. This case indicates that more studies are required to identify the factors that elicit an immune reaction and induce PNP in the absence of an underlying neoplasm.
Linear IgA dermatosis (LAD) is a rare, autoimmune, sub-epidermal bullous disease. It is relatively frequent in children in Africa. This is a retrospective study (January 1976 to June 2006). Children (0-16 yrs of age) with chronic acquired bullous diseases seen at the Charles Nicolle Hospital in Tunis and for who direct immunofluorescence(DIF) of the perilesional skin demonstrated immunoglobulin deposits were included in the study population. Fifty-one children with chronic blistering dermatosis were studied. Of these, 35 were found to have LAD, 7 had bullous pemphigoid, 5 had dermatitis herpetiformis, 3 had juvenile pemphigus and one had cicatricial pemphigoid. The mean age for the LAD case, was 5 years (range: 8 mos-16 yrs). The males outnumbered the females in a M:F ratio of 2:3. Fourteen children gave a history of a sudden onset of blisters. The skin lesions showed tense pruritic blisters of variable size, and in some cases small, clear-filled vesicles were associated (12/35); these arose on normal-appearing skin, or at the periphery of an annular erythema, and others had a cluster of jewels appearance, with lesions in a rosette pattern (15/30). Most of the children had generalized eruption (30/35) but more profuse on the face, pelvic region, buttocks, and limbs. The scalp, groin, and back were sometimes affected. Mucosal lesions happened in only 4 children. Histologic features showed a sub-epidermal bulla in all patients with eosinophiles and neutrophiles, 4 children had papillar abscesses. DIF tests demonstrated linear IgA deposits along the dermaleepidermal junction in all patients. IgG, IgM, and complement were also seen (21/35). Indirect imunoflurescent studies were negative in 67% of the LAD serum of patients. Malabsorption tests were positive in only 1 child with no clinical signs. HLA studies showed the B8 and DR3 antigens in 2 of the 3 patients studied. Most of patients responded to dapsone in low doses; however, prednisone had to be added in 7 children to control the disease and antimicrobials in 9 others. The mean duration of the disease was 14 months. This study confirms that LAD is the most common autoimmune bullous disease in children in Tunisia, which occur frequently in males in contrast to the reported cases elsewhere in the world. LAD is also characterised in our patient by relative rare mucosal lesions and a shorter duration.
Commercial support: None identified.
Commercial support: None identified.
P1609 Pemphigus erythematosus presenting as a mimic of seborrheic dermatitis Laci LaFleur, MD, Oklahoma University HSC Department of Dermatology, Oklahoma City, OK, United States; Kelli Lovelace, MD, Oklahoma University HSC Department of Dermatology, Oklahoma City, OK, United States; Richard Sontheimer, MD, Oklahoma University HSC Department of Dermatology, Oklahoma City, OK, United States; Michael John, MD, Oklahoma University HSC Department of Dermatology, Oklahoma City, OK, United States Pemphigus erythromatosus (PE) originally known as SeneareUsher syndrome, was described as a syndrome with features of both lupus erythematosus and pemphigus. Since this original report by Senear and Usher, there has been a debate on whether PE is a separate entity or is a localized form of superficial pemphigus such as pemphigus foliaceus (PF). We present a case of a 37-year-old white male treated for seborrheic dermatitis for several months with no improvement. The patient presented with erythematous scaly plaques on his scalp and face, and especially on the medial surface of his cheeks, which was recalcitrant to topical steroids. The eruption spread to his abdomen and chest. H&E exam of a biopsy done on his face revealed a spongiotic dermatitis. Because the area originally biopsied was impetiginized, a primary lesion on his back was biopsied and sent for H&E and direct immunofluorescence (DIF) examinations. The second biopsy showed superficial epidermal acantholytic changes with extensive acantholytic changes focally in a suprabasilar location. DIF exam showed a granular intercellular deposition of C3 involving the entire thickness of the epidermis with focal granular deposition of IgG in the superficial epidermis. Ig and complement deposition was not observed at the dermaleepidermal junction (DEJ). The criteria for diagnosing PE remain controversial. In most reports of PE, patients have had a positive ANA and immunopathology consistent with PE which is typified by superficial epidermal acantholysis associated with IgG and/or C3 deposition on keratinocyte surfaces and at the DEJ. Karlhofer et al reported for the first time in 2003 three otherwise typical PE patients with antibodies reactive to desmoglein 1, 230 kDa bullous pemphigoid antigen (BP230), 190 kDa protein comigrating with BP230, and periplakin. They suggested that this unique autoantibody profile may represent a new laboratory criterion for distinguishing between PE and PF. Our case is unique in that it is clinically and histologically consistent with PE but the immunopathology did not reveal typical Ig and/or complement deposition at the DEJ. Further workup of our patient’s serum including ANA and immunoblot/immunoprecipitation analysis are under way. Also, our case emphasizes the striking facial involvement that is typical of PE clinically and the need to re-evaluate when a banal dermatosis such as seborrheic dermatitis is not responding to treatment. Commercial support: None identified.
FEBRUARY 2007
P1611 Desmoglein 1 IgA pemphigus responsive to dapsone Marjan Yousefi, Geisinger Medical Center, Danville, PA, United States; Michele Maroon, MD, Geisinger Medical Center, Danville, PA, United States; William Tyler, MD, Geisinger Medical Center, Danville, PA, United States IgA pemphigus has 2 distinct types: subcorneal pustular dermatosis (SPD) and intraepidermal neutrophilic (IEP) type. Both forms present with vesicles or pustules arranged in an annular or circinate pattern and neutrophilic subcorneal pustules (in SPD type) or suprabasilar pustules (IEN type) with epidermal intercellular IgA on direct immunofluorescence (DIF). We present a case of IgA pemphigus with suprabasilar acantholysis, desmoglein 1 positivity on enzyme-linked immunosorbent assay (ELISA), and indirect immunofluorescence (IIF) findings of both IgG and IgA, responsive to dapsone. A 49-year-old white male presented with 2-year history of pruritic rash on the abdomen, trunk, legs, and scalp initiating as ‘‘water blisters.’’ Clinically, superficial round erosions were observed on the scalp with erosions, crusts, and intact vesicles scattered on the trunk, abdomen, chest, and upper thighs. He had no mucosal findings. Histopathology results were consistent with pemphigus vulgaris showing a suprabasilar acantholytic cleavage plane with intravesicular neutrophils. DIF showed smooth staining of intercellular spaces in lower portions of epidermis with both IgG and IgA, as well as occasional granules of IgG and IgA along the basement memebrane zone. Serum IIF to pemphigus antibody was positive for IgG and IgA. ELISA testing was positive for IgG to Dsg 1 and negative to Dsg 3. IgA ELISA and desmocollin testing were unavailable. The patient was treated successfully with dapsone and tapering prednisone. Our patient presented with a bullous disorder clinically similar to pemphigus foliaceous, lacking mucosal involvement and annular lesions. Histopathology of suprabasilar acantholysis with lower epidermal intercellular IgG on DIF is found in pemphigus vulgaris. Our patient, however, demonstrated Dsg 1 positivity and Dsg 3 negativity; pemphigus vulgaris should be positive for Dsg 3 and usually demonstrated mucosal disease. Thus, with negative Dsg 3 and absent mucosal involvement, presence of intercellular IgG and IgA, as well as IIF findings and response to Dapsone, he was categorized as Dsg 1 IgA pemphigus. The autoimmune targets in IEN type of IgA pemphigus have not been identified, however, a subset of IgA pemphigus patients have IgA autoantibodies against Desmoglein 1 or 3. We are presenting our patient to obtain recognition and shed light on this uncommon entity. Commercial support: None identified.
J AM ACAD DERMATOL
AB117
P1610
Linear IgA dermatosis in Tunisian children: 35 cases Monia Kharfi, PhD, Department of Dermatology. Charles Nicolle Hospital, Tunis, Tunisia, Hana Mhalla, Department of Dermatology. Charles Nicolle Hospital, Tunis, Tunisia, Amel Karaa, MD, Department of Dermatology. Charles Nicolle Hospital, Tunis, Tunisia, Mohamed Ridha Kamoun, PhD, Department of Dermatology. Charles Nicolle Hospital, Tunis, Tunisia
Paraneoplastic pemphigus without an underlying neoplasm Jee Bum Lee, MD, PhD, Department of Dermatology, Chonnam University Medical School, Gwang Ju, South Korea; Sook Jung Yun, MD, PhD, Department of Dermatology, Chonnam University Medical School, Gwang Ju, South Korea; Seung Chul Lee, MD, PhD, Department of Dermatology, Chonnam University Medical School, Gwang Ju, South Korea; Hyoung Ryun Park, PhD, Department of Chemistry, Chonnam National University, Gwang Ju, South Korea We describe a 52-year-old man with paraneoplastic pemphigus (PNP) without any evidence of an underlying neoplasm over an 8-year follow-up period. He had suffered from a chronic relapsing vesiculobullous eruption for approximately 7 years (from April 1998 to May 2005). Initially, scattered flaccid vesicles with crusts developed on the face and trunk, which waxed and waned several times. This case was diagnosed as PNP based on immunopathological PNP criteria (ie, histopathologic, immunoblotting, and immunoprecipitation analyses). However, physical and laboratory examinations, including serial blood tests with peripheral blood smear, whole body positron emission tomography/computed tomography, and ultrasonography were unable to detect any underlying neoplasm over an 8-year follow-up period. This case indicates that more studies are required to identify the factors that elicit an immune reaction and induce PNP in the absence of an underlying neoplasm.
Linear IgA dermatosis (LAD) is a rare, autoimmune, sub-epidermal bullous disease. It is relatively frequent in children in Africa. This is a retrospective study (January 1976 to June 2006). Children (0-16 yrs of age) with chronic acquired bullous diseases seen at the Charles Nicolle Hospital in Tunis and for who direct immunofluorescence(DIF) of the perilesional skin demonstrated immunoglobulin deposits were included in the study population. Fifty-one children with chronic blistering dermatosis were studied. Of these, 35 were found to have LAD, 7 had bullous pemphigoid, 5 had dermatitis herpetiformis, 3 had juvenile pemphigus and one had cicatricial pemphigoid. The mean age for the LAD case, was 5 years (range: 8 mos-16 yrs). The males outnumbered the females in a M:F ratio of 2:3. Fourteen children gave a history of a sudden onset of blisters. The skin lesions showed tense pruritic blisters of variable size, and in some cases small, clear-filled vesicles were associated (12/35); these arose on normal-appearing skin, or at the periphery of an annular erythema, and others had a cluster of jewels appearance, with lesions in a rosette pattern (15/30). Most of the children had generalized eruption (30/35) but more profuse on the face, pelvic region, buttocks, and limbs. The scalp, groin, and back were sometimes affected. Mucosal lesions happened in only 4 children. Histologic features showed a sub-epidermal bulla in all patients with eosinophiles and neutrophiles, 4 children had papillar abscesses. DIF tests demonstrated linear IgA deposits along the dermaleepidermal junction in all patients. IgG, IgM, and complement were also seen (21/35). Indirect imunoflurescent studies were negative in 67% of the LAD serum of patients. Malabsorption tests were positive in only 1 child with no clinical signs. HLA studies showed the B8 and DR3 antigens in 2 of the 3 patients studied. Most of patients responded to dapsone in low doses; however, prednisone had to be added in 7 children to control the disease and antimicrobials in 9 others. The mean duration of the disease was 14 months. This study confirms that LAD is the most common autoimmune bullous disease in children in Tunisia, which occur frequently in males in contrast to the reported cases elsewhere in the world. LAD is also characterised in our patient by relative rare mucosal lesions and a shorter duration.
Commercial support: None identified.
Commercial support: None identified.
P1609 Pemphigus erythematosus presenting as a mimic of seborrheic dermatitis Laci LaFleur, MD, Oklahoma University HSC Department of Dermatology, Oklahoma City, OK, United States; Kelli Lovelace, MD, Oklahoma University HSC Department of Dermatology, Oklahoma City, OK, United States; Richard Sontheimer, MD, Oklahoma University HSC Department of Dermatology, Oklahoma City, OK, United States; Michael John, MD, Oklahoma University HSC Department of Dermatology, Oklahoma City, OK, United States Pemphigus erythromatosus (PE) originally known as SeneareUsher syndrome, was described as a syndrome with features of both lupus erythematosus and pemphigus. Since this original report by Senear and Usher, there has been a debate on whether PE is a separate entity or is a localized form of superficial pemphigus such as pemphigus foliaceus (PF). We present a case of a 37-year-old white male treated for seborrheic dermatitis for several months with no improvement. The patient presented with erythematous scaly plaques on his scalp and face, and especially on the medial surface of his cheeks, which was recalcitrant to topical steroids. The eruption spread to his abdomen and chest. H&E exam of a biopsy done on his face revealed a spongiotic dermatitis. Because the area originally biopsied was impetiginized, a primary lesion on his back was biopsied and sent for H&E and direct immunofluorescence (DIF) examinations. The second biopsy showed superficial epidermal acantholytic changes with extensive acantholytic changes focally in a suprabasilar location. DIF exam showed a granular intercellular deposition of C3 involving the entire thickness of the epidermis with focal granular deposition of IgG in the superficial epidermis. Ig and complement deposition was not observed at the dermaleepidermal junction (DEJ). The criteria for diagnosing PE remain controversial. In most reports of PE, patients have had a positive ANA and immunopathology consistent with PE which is typified by superficial epidermal acantholysis associated with IgG and/or C3 deposition on keratinocyte surfaces and at the DEJ. Karlhofer et al reported for the first time in 2003 three otherwise typical PE patients with antibodies reactive to desmoglein 1, 230 kDa bullous pemphigoid antigen (BP230), 190 kDa protein comigrating with BP230, and periplakin. They suggested that this unique autoantibody profile may represent a new laboratory criterion for distinguishing between PE and PF. Our case is unique in that it is clinically and histologically consistent with PE but the immunopathology did not reveal typical Ig and/or complement deposition at the DEJ. Further workup of our patient’s serum including ANA and immunoblot/immunoprecipitation analysis are under way. Also, our case emphasizes the striking facial involvement that is typical of PE clinically and the need to re-evaluate when a banal dermatosis such as seborrheic dermatitis is not responding to treatment. Commercial support: None identified.
FEBRUARY 2007
P1611 Desmoglein 1 IgA pemphigus responsive to dapsone Marjan Yousefi, Geisinger Medical Center, Danville, PA, United States; Michele Maroon, MD, Geisinger Medical Center, Danville, PA, United States; William Tyler, MD, Geisinger Medical Center, Danville, PA, United States IgA pemphigus has 2 distinct types: subcorneal pustular dermatosis (SPD) and intraepidermal neutrophilic (IEP) type. Both forms present with vesicles or pustules arranged in an annular or circinate pattern and neutrophilic subcorneal pustules (in SPD type) or suprabasilar pustules (IEN type) with epidermal intercellular IgA on direct immunofluorescence (DIF). We present a case of IgA pemphigus with suprabasilar acantholysis, desmoglein 1 positivity on enzyme-linked immunosorbent assay (ELISA), and indirect immunofluorescence (IIF) findings of both IgG and IgA, responsive to dapsone. A 49-year-old white male presented with 2-year history of pruritic rash on the abdomen, trunk, legs, and scalp initiating as ‘‘water blisters.’’ Clinically, superficial round erosions were observed on the scalp with erosions, crusts, and intact vesicles scattered on the trunk, abdomen, chest, and upper thighs. He had no mucosal findings. Histopathology results were consistent with pemphigus vulgaris showing a suprabasilar acantholytic cleavage plane with intravesicular neutrophils. DIF showed smooth staining of intercellular spaces in lower portions of epidermis with both IgG and IgA, as well as occasional granules of IgG and IgA along the basement memebrane zone. Serum IIF to pemphigus antibody was positive for IgG and IgA. ELISA testing was positive for IgG to Dsg 1 and negative to Dsg 3. IgA ELISA and desmocollin testing were unavailable. The patient was treated successfully with dapsone and tapering prednisone. Our patient presented with a bullous disorder clinically similar to pemphigus foliaceous, lacking mucosal involvement and annular lesions. Histopathology of suprabasilar acantholysis with lower epidermal intercellular IgG on DIF is found in pemphigus vulgaris. Our patient, however, demonstrated Dsg 1 positivity and Dsg 3 negativity; pemphigus vulgaris should be positive for Dsg 3 and usually demonstrated mucosal disease. Thus, with negative Dsg 3 and absent mucosal involvement, presence of intercellular IgG and IgA, as well as IIF findings and response to Dapsone, he was categorized as Dsg 1 IgA pemphigus. The autoimmune targets in IEN type of IgA pemphigus have not been identified, however, a subset of IgA pemphigus patients have IgA autoantibodies against Desmoglein 1 or 3. We are presenting our patient to obtain recognition and shed light on this uncommon entity. Commercial support: None identified.
J AM ACAD DERMATOL
AB117