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Long-term survival of a patient with paraneoplastic pemphigus due to follicular dendritic cell sarcoma
587
1060
Long-term survival of a patient with paraneoplastic pemphigus due to follicular dendritic cell sarcoma Mariko Ogawa, MD, Nagoya University Graduate School of Medicine, Nagoya, Japan; Kazumitsu Sugiura, MD, PhD, Nagoya University Graduate School of Medicine, Nagoya, Japan; Yoshinao Muro, MD, PhD, Nagoya University Graduate School of Medicine, Nagoya, Japan; Takaaki Matsumoto, MD, PhD, Nagoya University Graduate School of Medicine, Nagoya, Japan; Hiroshi Koga, MD, PhD, Department of Dermatology, Kurume University School of Medicine, Kurume, Japan; Takashi Hashimoto, MD, PhD, Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan; Masashi Akiyama, MD, PhD, Nagoya University Graduate School of Medicine, Nagoya, Japan
Nonbullous pemphigoid Erica Tillman, MD, Baylor Scott & White, Temple, TX, United States; Tahmina Mahmood, Baylor Scott & White, Temple, TX, United States; Palak Parekh, MD, Baylor Scott & White, Temple, TX, United States In the literature, there have been reports of patients with bullous pemphigoid (BP) presenting with only pruritus and no evidence of blisters, for which the term pruritic nonbullous pemphigoid has been proposed. This presentation of BP is more commonly seen in patients over the age of 70. Diagnosis of BP is based on a combination of clinical features and immunopathologic findings. Enzyme-linked immunosorbent assays (ELISA) using recombinant portions of the BP180 and BP230 antigen have been found to be highly sensitive and specific in diagnosis. Direct immunofluorescence (DIF) showing a linear deposition of IgG and C3 along the dermoepidermal junction is also suggestive of BP. We report two cases of patients who presented with pruritus without any evidence of blisters who were diagnosed with BP based on DIF. However, both patients had normal levels of BP180 and BP230 antigen antibody levels on ELISA. These two cases demonstrate a subset of patients who present with pruritic prepemphigoid lesions and may be more likely to have a positive DIF and a negative ELISA. Although further studies are needed to validate this, we propose that DIF may be a more reliable diagnostic study for the early stages of BP.
We report a 28-year-old Japanese male with paraneoplastic pemphigus (PNP) due to follicular dendritic cell sarcoma (FDCS) in the retroperitoneal area. He had been suffering from erosions on the oral mucosa and the skin for several months. At another hospital, he was diagnosed with autoimmune blistering disease and intensive immunosuppressive treatments including steroid pulse therapy were performed, although they were not effective. The patient was referred to our hospital. Indirect immunofluorescence staining showed circulating IgG autoantibodies against keratinocyte cell surfaces. Immunoblot analysis using epidermal extracts revealed circulating antibodies to envoplakin and to periplakin. Unexpectedly, immunoblot analysis using purified human laminin-332 demonstrated circulating IgG antibodies against laminin-332. Computed tomography showed a mass lesion in the right retroperitoneal area. The tumor was partially resected and, histopathologically, it was diagnosed as FDCs developed from Castleman disease. For the residual FDCS lesions disseminated over the peritoneum and retroperitoneum, chemotherapy was selected. High-dose intravenous immunoglobulin, oral prednisone, 60 mg/day, and four courses of COP therapies were effective for the cutaneous lesions and erosions remained only on the oral mucosa. More than three years after the onset, the patient is surviving and the skin lesions almost healed except for the papillomatous hyperplasia and erosions on the oral mucosa. In general, the prognosis of PNP is poor, especially in cases with residual neoplasms. In this context, the present case is thought to be a rare long-term survivor. FDCS is an infrequent tumor occurred from follicular dendritic cell of lymph node. FDCS frequently complicated with PNP, and 16 cases of PNP due to FDCS have been reported in the literature as far as we know. In FCDS, desmosomelike structures are frequently seen at intercellular contact sites and they might be associated with induction of anti-desmosome autoimmunity by FDCS. About 60% of FDCS lesions occur in the lymph node around the neck and the axilla. However, interestingly, in 9 out of 10 FDCS patients with PNP with available information of primary tumor sites, the primary tumor was found in the peritoneal and retroperitoneal areas. The peritoneal and retroperitoneal location of FDCS might be a risk factor for the occurrence of PNP.
Commercial support: None identified.
Commercial support: None identified.
553 Peculiar presentation of pemphigus in association with thymoma Usman Mohammad Bello, MD, Faculty of Medicine-Sofia, Alexsandrovska University Hospital, Sofia, Bulgaria; Kossara Drenovska, MD, PhD, Karen Manuelyan, MD, PhD, Snejina Vassileva, MD, PhD, Medical University-Faculty of Medicine Sofia, Department of Dermatology and Venereology, Sofia, Bulgaria Introduction: Pemphigus is a rare autoimmune bullous disease mediated by autoantibodies against desmosomal antigens. Besides the classically recognized pemphigus variants, unusual presentations of the disease have been described, especially in cases associated with another autoimmune or neoplastic disorder. We report a peculiar vesiculobullous eruption diagnosed as pemphigus in a patient with thymoma.
Materials and methods: Twenty four patients of pemphigus vulgaris diagnosed by Tzanck smear and skin biopsy were included in the study. DCP pulse therapy was given to them according to standard protocol. Results: The mean age of patients was 44.7 6 14.8 years. Three patients were lost to follow up. In five patients DCP therapy was discontinued due to precipitation of diabetes (2), arthritis (1), and cardiac complications (2). Out of sixteen patients, 10 are in phase I, 4 in phase II, 1 in phase III and one patient had relapse. The commonest side effects observed was increased susceptibility to infections, both bacterial and candidal in 15 patients. However, the infections responded to conventional antibacterial and antifungal treatment. Conclusion: All our patients have shown remarkable response to DCP therapy irrespective of their disease activity and severity. The side effects profile was comparable with those from previous studies.
Methodology: A 23-year-old woman presented with a generalized pruritic blistering eruption of 3 months’ duration. The development of the skin lesions has coincided with complaints of fatigue and shortness of breath. Chest radiography and computer tomography revealed a retrosternal tumor consistent with thymoma and the patient has undergone resection for a capsulated medullar spindle cell tumor of the thymus. Two months later the skin lesions were still present. Results: On clinical evaluation, erythematous annular and gyrate plaques with large flaccid bullae in the center and multiple tense vesicles at the periphery, frequently in herpetiform pattern, were observed. The Nikolsky sign was positive. No mucosal lesions were present. Histologic examination revealed superficial moderate acantholysis. Direct immunofluorescence was characterized by intraepidermal intercellular deposition of IgG and C3, and homogenous IgM band along the dermoepidermal junction. Indirect immunofluorescence on human esophagus detected high titers pemphigus antibodies in the absence of antinuclear antibodies. The patient’s serum did not react with rat bladder substrate. ELISA antidesmoglein 1 was strongly positive while antidesmoglein 3 titers were very low. Systemic treatment with corticosteroids and azathioprine resulted in good clinical response and no relapse of the disease has been documented within a 3-year period. Conclusions: The observed peculiar pemphigus presentation was difficult to classify. Paraneoplastic pemphigus (PNP) was initially discussed but the patient did not meet the defined criteria. Based on the clinical and laboratory findings we accepted pemphigus herpetiformis as the most probable in our patient although some features of pemphigus erythematosus were also detected. Another possible interpretation could be the multiple autoimmune syndrome (MAS), especially MAS type I combining pemphigus, thymoma, myasthenia gravis, etc. Similar associations may be considered as completely random, or pathogenically predetermined.
Commercial support: None identified.
Commercial support: None identified.
1025 Management of pemphigus vulgaris by dexamethasone cyclophosphamide pulse therapy: A SAIMS experience Kailash Bhatia, MD, Sri Aurobindo Medical College and PG Institute, Indore, India; Rajesh Kataria, MD, Sri Aurobindo Medical College and PG Institute, Indore, India; Rini Sharma, MBBS, Sri Aurobindo Medical College and PG Institute, Indore, India Background: The standard treatment of pemphigus comprises of systemic steroids and other immunosuppressive agents. Dexamethasone-cyclophosphamide pulse (DCP) therapy has shown promising results in the management of these diseases. The objective of the study was to assess the outcome of DCP therapy in pemphigus.
MAY 2015
J AM ACAD DERMATOL
AB121
1060
Long-term survival of a patient with paraneoplastic pemphigus due to follicular dendritic cell sarcoma Mariko Ogawa, MD, Nagoya University Graduate School of Medicine, Nagoya, Japan; Kazumitsu Sugiura, MD, PhD, Nagoya University Graduate School of Medicine, Nagoya, Japan; Yoshinao Muro, MD, PhD, Nagoya University Graduate School of Medicine, Nagoya, Japan; Takaaki Matsumoto, MD, PhD, Nagoya University Graduate School of Medicine, Nagoya, Japan; Hiroshi Koga, MD, PhD, Department of Dermatology, Kurume University School of Medicine, Kurume, Japan; Takashi Hashimoto, MD, PhD, Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan; Masashi Akiyama, MD, PhD, Nagoya University Graduate School of Medicine, Nagoya, Japan
Nonbullous pemphigoid Erica Tillman, MD, Baylor Scott & White, Temple, TX, United States; Tahmina Mahmood, Baylor Scott & White, Temple, TX, United States; Palak Parekh, MD, Baylor Scott & White, Temple, TX, United States In the literature, there have been reports of patients with bullous pemphigoid (BP) presenting with only pruritus and no evidence of blisters, for which the term pruritic nonbullous pemphigoid has been proposed. This presentation of BP is more commonly seen in patients over the age of 70. Diagnosis of BP is based on a combination of clinical features and immunopathologic findings. Enzyme-linked immunosorbent assays (ELISA) using recombinant portions of the BP180 and BP230 antigen have been found to be highly sensitive and specific in diagnosis. Direct immunofluorescence (DIF) showing a linear deposition of IgG and C3 along the dermoepidermal junction is also suggestive of BP. We report two cases of patients who presented with pruritus without any evidence of blisters who were diagnosed with BP based on DIF. However, both patients had normal levels of BP180 and BP230 antigen antibody levels on ELISA. These two cases demonstrate a subset of patients who present with pruritic prepemphigoid lesions and may be more likely to have a positive DIF and a negative ELISA. Although further studies are needed to validate this, we propose that DIF may be a more reliable diagnostic study for the early stages of BP.
We report a 28-year-old Japanese male with paraneoplastic pemphigus (PNP) due to follicular dendritic cell sarcoma (FDCS) in the retroperitoneal area. He had been suffering from erosions on the oral mucosa and the skin for several months. At another hospital, he was diagnosed with autoimmune blistering disease and intensive immunosuppressive treatments including steroid pulse therapy were performed, although they were not effective. The patient was referred to our hospital. Indirect immunofluorescence staining showed circulating IgG autoantibodies against keratinocyte cell surfaces. Immunoblot analysis using epidermal extracts revealed circulating antibodies to envoplakin and to periplakin. Unexpectedly, immunoblot analysis using purified human laminin-332 demonstrated circulating IgG antibodies against laminin-332. Computed tomography showed a mass lesion in the right retroperitoneal area. The tumor was partially resected and, histopathologically, it was diagnosed as FDCs developed from Castleman disease. For the residual FDCS lesions disseminated over the peritoneum and retroperitoneum, chemotherapy was selected. High-dose intravenous immunoglobulin, oral prednisone, 60 mg/day, and four courses of COP therapies were effective for the cutaneous lesions and erosions remained only on the oral mucosa. More than three years after the onset, the patient is surviving and the skin lesions almost healed except for the papillomatous hyperplasia and erosions on the oral mucosa. In general, the prognosis of PNP is poor, especially in cases with residual neoplasms. In this context, the present case is thought to be a rare long-term survivor. FDCS is an infrequent tumor occurred from follicular dendritic cell of lymph node. FDCS frequently complicated with PNP, and 16 cases of PNP due to FDCS have been reported in the literature as far as we know. In FCDS, desmosomelike structures are frequently seen at intercellular contact sites and they might be associated with induction of anti-desmosome autoimmunity by FDCS. About 60% of FDCS lesions occur in the lymph node around the neck and the axilla. However, interestingly, in 9 out of 10 FDCS patients with PNP with available information of primary tumor sites, the primary tumor was found in the peritoneal and retroperitoneal areas. The peritoneal and retroperitoneal location of FDCS might be a risk factor for the occurrence of PNP.
Commercial support: None identified.
Commercial support: None identified.
553 Peculiar presentation of pemphigus in association with thymoma Usman Mohammad Bello, MD, Faculty of Medicine-Sofia, Alexsandrovska University Hospital, Sofia, Bulgaria; Kossara Drenovska, MD, PhD, Karen Manuelyan, MD, PhD, Snejina Vassileva, MD, PhD, Medical University-Faculty of Medicine Sofia, Department of Dermatology and Venereology, Sofia, Bulgaria Introduction: Pemphigus is a rare autoimmune bullous disease mediated by autoantibodies against desmosomal antigens. Besides the classically recognized pemphigus variants, unusual presentations of the disease have been described, especially in cases associated with another autoimmune or neoplastic disorder. We report a peculiar vesiculobullous eruption diagnosed as pemphigus in a patient with thymoma.
Materials and methods: Twenty four patients of pemphigus vulgaris diagnosed by Tzanck smear and skin biopsy were included in the study. DCP pulse therapy was given to them according to standard protocol. Results: The mean age of patients was 44.7 6 14.8 years. Three patients were lost to follow up. In five patients DCP therapy was discontinued due to precipitation of diabetes (2), arthritis (1), and cardiac complications (2). Out of sixteen patients, 10 are in phase I, 4 in phase II, 1 in phase III and one patient had relapse. The commonest side effects observed was increased susceptibility to infections, both bacterial and candidal in 15 patients. However, the infections responded to conventional antibacterial and antifungal treatment. Conclusion: All our patients have shown remarkable response to DCP therapy irrespective of their disease activity and severity. The side effects profile was comparable with those from previous studies.
Methodology: A 23-year-old woman presented with a generalized pruritic blistering eruption of 3 months’ duration. The development of the skin lesions has coincided with complaints of fatigue and shortness of breath. Chest radiography and computer tomography revealed a retrosternal tumor consistent with thymoma and the patient has undergone resection for a capsulated medullar spindle cell tumor of the thymus. Two months later the skin lesions were still present. Results: On clinical evaluation, erythematous annular and gyrate plaques with large flaccid bullae in the center and multiple tense vesicles at the periphery, frequently in herpetiform pattern, were observed. The Nikolsky sign was positive. No mucosal lesions were present. Histologic examination revealed superficial moderate acantholysis. Direct immunofluorescence was characterized by intraepidermal intercellular deposition of IgG and C3, and homogenous IgM band along the dermoepidermal junction. Indirect immunofluorescence on human esophagus detected high titers pemphigus antibodies in the absence of antinuclear antibodies. The patient’s serum did not react with rat bladder substrate. ELISA antidesmoglein 1 was strongly positive while antidesmoglein 3 titers were very low. Systemic treatment with corticosteroids and azathioprine resulted in good clinical response and no relapse of the disease has been documented within a 3-year period. Conclusions: The observed peculiar pemphigus presentation was difficult to classify. Paraneoplastic pemphigus (PNP) was initially discussed but the patient did not meet the defined criteria. Based on the clinical and laboratory findings we accepted pemphigus herpetiformis as the most probable in our patient although some features of pemphigus erythematosus were also detected. Another possible interpretation could be the multiple autoimmune syndrome (MAS), especially MAS type I combining pemphigus, thymoma, myasthenia gravis, etc. Similar associations may be considered as completely random, or pathogenically predetermined.
Commercial support: None identified.
Commercial support: None identified.
1025 Management of pemphigus vulgaris by dexamethasone cyclophosphamide pulse therapy: A SAIMS experience Kailash Bhatia, MD, Sri Aurobindo Medical College and PG Institute, Indore, India; Rajesh Kataria, MD, Sri Aurobindo Medical College and PG Institute, Indore, India; Rini Sharma, MBBS, Sri Aurobindo Medical College and PG Institute, Indore, India Background: The standard treatment of pemphigus comprises of systemic steroids and other immunosuppressive agents. Dexamethasone-cyclophosphamide pulse (DCP) therapy has shown promising results in the management of these diseases. The objective of the study was to assess the outcome of DCP therapy in pemphigus.
MAY 2015
J AM ACAD DERMATOL
AB121