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Archives of dermatology 08/2011; 147(8):989-91. · 4.76 Impact Factor
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International journal of dermatology 05/2011; 50(5):604-6. · 1.18 Impact Factor
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Archives of dermatology 11/2010; 146(11):1301-6. · 4.76 Impact Factor
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Journal of the American Academy of Dermatology 07/2010; 63(1):161-3. · 3.99 Impact Factor
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Archives of dermatology 05/2009; 145(4):500-1. · 4.76 Impact Factor
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ABSTRACT: The mainstay of therapy of autoimmune mucocutaneous blistering diseases has been prolonged high-dose systemic corticosteroids and immunosuppressive agents. Recently, high-dose intravenous immunoglobulin (IVIg) has been employed in selected cases, with excellent results in most of them.
We sought to evaluate the outcome of the use of IVIg in patients with autoimmune mucocutaneous blistering diseases refractory to conventional therapy or with contraindications for it.
We performed a retrospective analysis of clinical response to monthly cycles of IVIg in 19 patients affected with autoimmune mucocutaneous blistering diseases: 10 patients with pemphigus vulgaris (PV), 2 with pemphigus foliaceus (PF), 4 with mucous membrane pemphigoid (MMP), 2 with epidermolysis bullosa acquisita, and one with linear IgA bullous dermatosis.
Four (21%) of 19 cases presented a complete response (2 PV, 1 MMP and 1 epidermolysis bullosa acquisita). Five (26%) patients did not respond to the treatment (3 PV, 1 PF, 1 MMP). Ten patients (53%) had a partial response.
This was a retrospective noncontrolled study with a heterogeneous group of patients.
The effectiveness of IVIg was inferior to that previously reported. This difference could be attributed to the preparations employed, the different severity of the disease, or individual responses in each patient dependent on Fc receptor gamma polymorphisms.
Journal of the American Academy of Dermatology 07/2007; 56(6):960-7. · 3.99 Impact Factor
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ABSTRACT: Bullous pemphigoid (BP) is an autoimmune blistering disease that is rare in childhood. As in adult BP, antibodies against the 180-kDa antigen (BP180) seem to be involved in the pathogenesis of the disease, but, to date, only a small number of children with the disease have been examined immunologically.
We report the cases of 4 infants with BP aged 5 to 12 months. All of them had involvement of the hands and feet, and they all achieved a complete remission in less than 6 months when treated with oral prednisolone stearoyl glycolate. Three patients could be examined using antigen characterization techniques. Autoantibodies against the NC16A domain of BP180 were found by immunoblot assay in all 3 and by enzyme-linked immunosorbent assay in 2 of them. Interestingly, although IgA autoantibodies were detected in only 1 of them by indirect immunofluorescence, all of them had IgA autoantibodies, and 2 of the 3 had IgG autoantibodies against NC16A as detected by immunoblot assay. One patient also had IgG autoantibodies against the carboxyterminal domain of BP180.
IgA-specific antibodies against BP180 were detected in all our patients. These findings further raise the question about the relationship between BP and linear IgA bullous dermatosis, the most common autoimmune blistering disorder in children.
Archives of Dermatology 03/2007; 143(2):215-20. · 3.89 Impact Factor
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ABSTRACT: Epidermodysplasia verruciformis (EV) is a rare autosomal recessive genodermatosis characterized by an impairment of cellular immunity. It clinically manifests as widespread, long-lasting, pityriasis versicolor-like macules and flat, wart-like papules, usually occurring in early childhood. There is a risk of development of multiple skin cancers in the third decade, primarily in sun-exposed skin. EV-associated human papillomaviruses have been implicated in a number of cutaneous lesions in non-EV populations, such as seborrheic keratoses or psoriasis. They have also been implicated in the development of nonmelanoma skin cancer, especially in immunosuppressed patients. Patients affected with EV are not able to eliminate oncogenic viruses within lesions, leading to a malignant transformation.
To describe the dermoscopic characteristics of EV cutaneous tumors by performing histopathologic correlation.
Cutaneous lesions and tumors from two patients affected by EV were included. Clinical and dermoscopic images were obtained and excision with ulterior histopathology was performed in all suspicious tumors and characteristic lesions.
Dermoscopy and histology of pityiriasis versicolor-like macules, wart-like papules, seborrheic keratosis-like tumors, psoriasis-like plaques, collision tumors, and Bowen in situ carcinoma are described.
Dermoscopy in EV tumors correlated with histopathologic findings and improved the differential diagnosis of tumors in this disease.
Dermatologic Surgery 02/2006; 32(1):103-6. · 1.80 Impact Factor
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ABSTRACT: Paraneoplastic pemphigus is a life-threatening autoimmune bullous disease associated with neoplasia, generally of lymphoid origin. Immunosuppressive therapy is often disappointing and there are only a few reports of patients surviving more than 2 years. These cases were generally associated with benign neoplasms. We report here the case of a patient with paraneoplastic pemphigus associated with non-Hodgkin B-cell lymphoma who had a surprisingly good response to systemic corticosteroids and remains free of lesions more than 3 years later despite progression of her neoplasm.
Acta Dermato Venereologica 02/2005; 85(3):233-5. · 3.18 Impact Factor
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ABSTRACT: Bullous pemphigoid has developed in association with different types of malignant diseases, including a few cases of B-cell lymphoproliferative disorders. However, the paraneoplastic significance of this association is still controversial.
We describe a 39-year-old patient who presented with a bullous eruption and generalized lymphadenopathy. The results of histologic, immunofluorescence, and antigenic studies confirmed the diagnosis of bullous pemphigoid. The histopathologic and immunophenotypic features of a lymph node biopsy specimen were consistent with mantle cell lymphoma. There was total resolution of the mucocutaneous lesions when mantle cell lymphoma went into remission.
The age of the patient and the concomitant appearance and simultaneous remission of both diseases strongly suggest that bullous pemphigoid was a paraneoplastic phenomenon in the present case.
Archives of Dermatology 01/2005; 140(12):1496-9. · 3.89 Impact Factor
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ABSTRACT: Background
Bullous pemphigoid (BP) is an autoimmune blistering disease that is rare in childhood. As in adult BP, antibodies against the 180-kDa antigen (BP180) seem to be involved in the pathogenesis of the disease, but, to date, only a small number of children with the disease have been examined immunologically.Observations
We report the cases of 4 infants with BP aged 5 to 12 months. All of them had involvement of the hands and feet, and they all achieved a complete remission in less than 6 months when treated with oral prednisolone stearoyl glycolate. Three patients could be examined using antigen characterization techniques. Autoantibodies against the NC16A domain of BP180 were found by immunoblot assay in all 3 and by enzyme-linked immunosorbent assay in 2 of them. Interestingly, although IgA autoantibodies were detected in only 1 of them by indirect immunofluorescence, all of them had IgA autoantibodies, and 2 of the 3 had IgG autoantibodies against NC16A as detected by immunoblot assay. One patient also had IgG autoantibodies against the carboxyterminal domain of BP180.Conclusions
IgA-specific antibodies against BP180 were detected in all our patients. These findings further raise the question about the relationship between BP and linear IgA bullous dermatosis, the most common autoimmune blistering disorder in children.
Figures in this Article
Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease that commonly affects elderly individuals. The disease is very rare in children, and most cases occur in school-aged children (generally, those older than 8 years).1 Mucous membrane involvement seems to be more frequent in childhood BP, as well as involvement of the hands and feet in infants younger than 1 year.2 As in adult BP, antibodies against the 180-kDa BP antigen (BP180, also known as type XVII collagen) seem to be involved in the pathogenesis of the disease.3 However, to date, the target antigen of the autoantibodies has been studied by immunoblot assay or enzyme-linked immunosorbent assay (ELISA) in only a small number of children with BP. Herein, we describe a series of 4 infants with BP seen at a single institution (Hospital Sant Joan de Deu, Barcelona, Spain). The serum samples of 3 of them were tested against the 2 main antigenic domains of BP180 (recombinant NC16A and carboxyterminal domains) both by immunoblot assay and ELISA.
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AUTHOR INFORMATION
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REFERENCES
CASE 1
A 5-month-old boy with a sudden eruption of blisters and bullae on his hands and feet was referred to our institution. He also had small oral erosions that had been noted by the pediatrician. The cutaneous lesions quickly generalized to involve the trunk, face, and lower limbs. On physical examination, the patient had an eruption of tense vesicles and blisters with surrounding erythema on these sites. We were not able to see mucosal lesions. Histologic examination of a perilesional skin biopsy sample revealed a subepidermal blister with abundant eosinophils. Direct immunofluorescence (IF) examination showed linear deposits of IgG, IgA (faint deposits), and C3 (intense deposits) along the basement membrane zone (BMZ). Treatment with 2 mg/kg per day of oral prednisolone stearoyl glycolate was started, and remarkable clinical improvement was noted within a few days; therefore, the dosage was quickly tapered and finally discontinued when complete remission was achieved after 3 months of therapy. The patient was still in remission 2 years after follow-up.
CASE 2
A 5-month-old boy was referred to our institution for evaluation of a vesicular eruption affecting his hands, feet, and trunk. On physical examination, his general condition was good, but he exhibited vesiculobullous lesions arising from inflamed skin on his hands and feet (Figure 1) and isolated lesions on the anterior side of his trunk. Mucous membranes were not affected. Results from laboratory studies were all within reference range, except for a finding of slight leukocytosis with eosinophilia (14%). Findings from histopathologic examination revealed spongiosis, subepidermal vesicles, and mixed inflammatory infiltrates in the papillary dermis consisting predominantly of eosinophils. Direct IF examination of perilesional skin showed linear deposits of IgG, IgA (faint deposits), and C3 (intense deposits) along the BMZ. Treatment with topical mometasone propionate was tested for 1 week, without improvement, so treatment with 1 mg/kg per day of oral prednisolone stearoyl glycolate was begun. This therapy was continued for 2 weeks, when complete remission was achieved. It was then gradually tapered and finally stopped after 3 months, with no recurrence of lesions after 6 months of follow-up.
Place holder to copy figure label and captionFigure 1.Case 2. Pompholyxlike appearance at presentation with tense vesicles and blisters on the hands (A) and feet (B).Grahic Jump Location+View Large |
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CASE 3
A 12-month-old boy was sent to our institution for evaluation of a 3-month history of blisters, erosions, and crusted lesions on his hands, feet, abdomen, and scalp. An initial clinical diagnosis of chronic bullous dermatosis of childhood had been made at another institution, and treatment with 1 mg/kg per day of dapsone was started. Despite an apparent initial response to treatment, the lesions quickly spread on his trunk and lower limbs. On physical examination, the patient was found to have crusted lesions on his scalp as well as on his trunk and limbs, with tense blisters on his palms and soles. Subepidermal bullae were found on histologic examination, and direct IF of perilesional skin detected linear deposits of IgG and intense deposits of C3 along the BMZ. No blood samples were drawn for indirect IF or further studies in this case. A diagnosis of BP was then made, and treatment with dapsone was stopped when therapy with 1 mg/kg per day of oral prednisolone stearoyl glycolate was prescribed. Complete remission was achieved in 1 month while tapering the dosage of corticosteroids. Ten months after therapy ended, the patient was still in remission.
CASE 4
A 4-month-old boy was referred for a widespread eruption of blisters affecting his abdomen, limbs, scalp, palms, and soles that had started a few days previously. On physical examination, he had tense blisters with surrounding erythema on these sites (Figure 2), without mucosal involvement. Histopathologic examination showed subepidermal bullae with a mixed infiltrate in papillary dermis, with abundant eosinophils. Topical therapy with betamethasone diproprionate was started. Results from routine laboratory testing were within reference range except for a slight leukocytosis with a normal differential. Direct IF showed linear deposits of IgG and intense deposits of C3 along the BMZ. Therapy with 1 mg/kg per day of oral prednisolone stearoyl glycolate was started, with immediate improvement. Complete remission was achieved during the next 2 weeks. The dosage of prednisolone was then gradually tapered and finally stopped after 4 months, and 1 year later the patient was still in remission.
Place holder to copy figure label and captionFigure 2.Case 4. A, Widespread eruption of bullae arising on erythematous base. B, Detail of lesions on the thigh.Grahic Jump Location+View Large |
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Archives of Dermatology 143(2):215-220. · 3.89 Impact Factor
