ABSTRACT
Epidermolysis bullosa acquisita (EBA) is a chronic, subepidermal blistering disease characterized by the presence of autoantibodies to type VII collagen, located below the lamina densa of the basement membrane zone (BMZ). There is a large clinical and histological overlap between EBA and other subepidermal autoimmune bullous diseases, therefore, complex immunological techniques are required to make an accurate diagnosis. Therapy of EBA is also a difficult issue. Most patients do not respond to several common immunosuppressive agents. We describe a patient who has shown a good response to high-dose intravenous immunoglobulin therapy.
Subject(s)
Epidermolysis Bullosa Acquisita/diagnosis , Immunoglobulins, Intravenous/administration & dosage , Immunologic Factors/administration & dosage , Antibodies/analysis , Basement Membrane/immunology , Drug Administration Schedule , Epidermolysis Bullosa Acquisita/drug therapy , Female , Fluorescent Antibody Technique, Direct/methods , Humans , Immunoglobulins, Intravenous/immunology , Immunologic Factors/immunology , Middle Aged , Treatment OutcomeABSTRACT
La amiloidosis cutánea nodular primaria es la forma clínica menos frecuente de las amiloidosis cutáneas. Es posible su asociación a trastornos mieloproliferativos, así como a amiloidosis sistémica. Se ha descrito recientemente la asociación a otras entidades, como el síndrome de Sjögren. Se presenta el caso de una paciente con síndrome de Sjögren que desarrolló una amiloidosis cutánea nodular primaria
Nodular primary cutaneous amyloidosis is the least frequent clinical form of the cutaneous amyloidoses. It may be associated with myeloproliferative disorders, as well as with systemic amyloidosis. Its association with other entities, such as Sjögren's syndrome, has recently been described. We present the case of a female patient with Sjögren's syndrome who developed nodular primary cutaneous amyloidosis
Subject(s)
Female , Aged , Humans , Sjogren's Syndrome/complications , Amyloidosis/complications , Amyloidosis/physiopathology , Amyloid/classification , Amyloidosis/classificationABSTRACT
Se presentan 2 casos de lupus eritematoso sistémico (LES) que se manifestaron con síntomas cutáneos subagudos. Tanto el lupus cutáneo como el lupus sistémico son el resultado de interacciones entre genes de susceptibilidad y factores del medio, como la radiación ultravioleta, que provoca una respuesta inmunitaria anómala con una hiperreactividad de linfocitos T y B. Más del 50 % de los lupus cutáneos subagudos tienen o tendrán un LES, mientras que sólo del 16 al 61 % de los LES tienen lesiones de lupus cutáneo agudo
We present two cases of systemic lupus erythematosus (SLE) which began with subacute clinical manifestations. Both cutaneous and systemic lupus are the result of interactions between susceptibility genes and environmental factors such as ultraviolet radiation, giving rise to an anomalous response with hyperreactivity of T and B lymphocytes. Over 50 % of subacute cutaneous lupus cases have or will have SLE, while only 16 to 61 % of SLE cases have acute cutaneous lupus lesions
Subject(s)
Male , Female , Adult , Humans , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/therapy , Echocardiography, Doppler , Ultraviolet Rays , Lupus Erythematosus, Cutaneous/diagnosis , Lupus Erythematosus, Cutaneous/therapy , Biopsy , Hyperkeratosis, Epidermolytic/diagnosis , Exanthema/complications , Exanthema/diagnosis , Lupus Erythematosus, Systemic , Antibodies, Antiphospholipid , Dermatomyositis/diagnosis , Dermatomyositis/therapy , Polymyositis/diagnosis , Polymyositis/therapyABSTRACT
Nodular primary cutaneous amyloidosis is the least frequent clinical form of the cutaneous amyloidoses. It may be associated with myeloproliferative disorders, as well as with systemic amyloidosis. Its association with other entities, such as Sjögren's syndrome, has recently been described. We present the case of a female patient with Sjögren's syndrome who developed nodular primary cutaneous amyloidosis.
Subject(s)
Amyloidosis/etiology , Sjogren's Syndrome/complications , Skin Diseases/etiology , Aged , Female , HumansABSTRACT
We present two cases of systemic lupus erythematosus (SLE) which began with subacute clinical manifestations. Both cutaneous and systemic lupus are the result of interactions between susceptibility genes and environmental factors such as ultraviolet radiation, giving rise to an anomalous response with hyperreactivity of T and B lymphocytes. Over 50 % of subacute cutaneous lupus cases have or will have SLE, while only 16 to 61 % of SLE cases have acute cutaneous lupus lesions.
Subject(s)
Lupus Erythematosus, Cutaneous , Lupus Erythematosus, Systemic , Adult , Female , Humans , Lupus Erythematosus, Cutaneous/complications , Lupus Erythematosus, Cutaneous/pathology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/pathology , MaleABSTRACT
La infección por dermatofitos del cuero cabelludo o tinea capitis es una patología relativamente infrecuente en adultos.Se presenta el caso clínico de una tiña del cuero cabelludo inflamatoria grave en una mujer de 70 años por Microsporum gypseum, único dermatofito geofílico patógeno para el ser humano y responsable del 1,4% de las dermatofitosis de nuestro país (AU)
Subject(s)
Aged , Female , Humans , Microsporum/isolation & purification , Tinea Capitis/microbiology , Tinea Capitis/drug therapy , Microsporidiosis/diagnosis , Microsporidiosis/drug therapy , Severity of Illness Index , Itraconazole/therapeutic use , Antifungal Agents/therapeutic useABSTRACT
La elastosis perforante serpiginosa (EPS) es una dermatosis crónica e infrecuente, que pertenece al grupo de las dermatosis perforantes primarias. Se han descrito tres formas principales: formas idiopáticas, formas reactivas asociadas a enfermedades hereditarias del tejido conjuntivo, o al síndrome de Down, y formas relacionadas con los tratamientos crónicos con penicilamina. En los pacientes con síndrome de Down se ha observado una prevalencia de EPS del 1%, y parece existir una predisposición a desarrollar lesiones más generalizadas y más resistentes al tratamiento. El desencadenante de la EPS es la producción de un exceso de fibras elásticas alteradas morfológica y bioquímicamente, situadas en la dermis papilar. Estas fibras actúan como un material extraño provocando una reacción por la cual son eliminadas a través de conductos transepidérmicos (AU)
Subject(s)
Adult , Male , Humans , Down Syndrome/complications , Skin Diseases/etiology , Erythema/pathology , Elastic Tissue/physiopathologyABSTRACT
Hepatitis C virus (HCV) replicates in salivary glands of chronic hepatitis C patients and is released into the saliva, suggesting that HCV may replicate in other exocrine glands. The presence of positive and negative HCV RNA strands was demonstrated by in situ hybridization, and of HCV core protein by immunohistochemistry, in sweat glands and keratinocytes in healthy skin biopsies from 15 patients with chronic hepatitis C and 10 anti-HCV negative patients with chronic liver disease. Positive and negative HCV RNA strands were detected in 9.6 +/- 5.2% and 4.2 +/- 3.8%, respectively, of the epithelial cells of eccrine sweat glands. Core protein was detected in 6.0 +/- 3.93% of these cells. HCV RNA resistant to RNase digestion (encapsidated HCV RNA) was detected in 10/10 sweat samples from HCV-infected patients. Positive and negative HCV RNA strands were detected in 6.7 +/- 2.97% and 3.0 +/- 3.08% of the keratinocytes, respectively. HCV core protein was found in 4.5 +/- 2.76% of these cells. No HCV RNA or HCV core protein was detected in the skin biopsies from the 10 anti-HCV negative patients. In conclusion, HCV replicates in eccrine sweat glands cells and keratinocytes in healthy skin and is released into the sweat.
Subject(s)
Hepacivirus/physiology , Hepatitis C, Chronic/virology , Keratinocytes/virology , Sweat Glands/virology , Virus Replication , Adult , Female , Hepacivirus/isolation & purification , Humans , Immunohistochemistry , In Situ Hybridization , Male , Middle Aged , RNA, Viral/analysis , Reverse Transcriptase Polymerase Chain Reaction , Viral Core Proteins/analysisABSTRACT
Cutaneous lichen planus has been associated in patients with chronic hepatitis C virus infection. It is still unknown whether hepatitis C virus infects keratinocytes of lichen planus lesions. In this report we have analyzed the presence of genomic and anti-genomic hepatitis C virus RNA in skin biopsies from 26 patients with chronic hepatitis C and healthy skin and from 24 patients with cutaneous lichen planus (five with and 19 without hepatitis C virus infection) by in situ hybridization. Hepatitis C virus RNA was detected in the keratinocytes of 69% of the patients with healthy skin and chronic hepatitis C, in 100% of the patients with lichen planus and hepatitis C virus infection, and in none of lichen planus patients without hepatitis C virus infection. The percentage of keratinocytes showing genomic or anti-genomic hepatitis C virus RNA was statistically lower (p < 0.01 in all cases) in patients with healthy skin (mean +/- SD: 5.7 +/- 3.5% and 2.7 +/- 3.1% of keratinocytes with genomic or anti-genomic hepatitis C virus RNA, respectively) than in those with lichen planus lesions (31.7 +/- 7.9% and 18.8 +/- 7.4%, mean +/- SD) or the unaffected adjacent skin (24.8 +/- 6.9% and 14.3 +/- 3.8%, mean +/- SD). In conclusion, we have demonstrated that hepatitis C virus infects keratinocytes from patients with lichen planus and hepatitis C virus infection.
