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SSA autoantibodies
776
Journal of the American Academy of Dermatology May 1997
Pearls of wisdom
review highlights clinical manifestations and management in patients with AIDS and critiques several controversial issues and current research.
lowed by a summary of current knowledge of retroviruses (HTLV-I, HIV) and DNA and RNA tumor viruses (including HPV, hepatitis B and C viruses, herpesvirus-
es). COMMENT: This ambitious and definitive review of cryptococcosis, a resilient and devastating disease, especially in the AIDS pandemic, should be a reference for any clinician who examines and treats immunocompromised patients, especially those with AIDS.
Kenneth J. Tomecki, MD
Chancroid and Haemophilus ducreyi: an update
COMMENT:This article is a thoughtful, objective summary of viral carcinogenesis for the clinician. Viral infection is definitely instrumental in the development of some human cancers. The mechanisms and the extent to which viral infection contributes are unclear. Via molecular analysis, tumor-associated viruses function by encoding proteins that alter pathways of growth arrest and apoptosis. For further insights, read the review (it is only four pages), which is recommended for anyone with an interest in the topic.
Kenneth J. Tomecki, MD
Trees DL, M o r s e SA. Clin Microbiol R e v 1995; 8:357-75. The authors present a "state of the disease" update of chancroid, a major ulcerative disease of the genitals now associated with HIV infection. The update covers epidemiology; clinical features; diagnosis; treatment; susceptibility testing and microbial resistance; growth and nutritional requirements; strain typing; human immune response and serology; in vivo and in vitro models to study pathogenesis; potential virulence factors; genetics and classification; and several unresolved issues (e.g., prevalence of Haemophilus ducreyi in genital lesions, the role of asymptomatic carriers, virulence differences among strains). COMMENT: This article is an expert and definitive summary of chancroid (the first in 7 years), a common disease in Africa, Asia, and Latin America. As a sexually transmitted infection, chancroid may be a cofactor for the heterosexual transmission of HIV infection. As such, this review is recommended reading for any clinician who treats patients with sexually transmitted disease or H1V infection.
Kenneth J. Tomecki, MD
Association between the presence of sequences homologous to gag-pol region of the HIV-1 in DNA from sets of connective disease patients and the detection of autoantibodies against cell nucleus particles Prokop J. Post~py Dermatol 1995;12:455-61. (In Polish) A strong association was found between sequences homologous to the gag-pol region of HIV-1 in DNA and the presence of anti double-stranded DNA and anti single-stranded DNA from patients with systemic lupus erythematosus and disseminated discoid lupus erythematosus. There was also a statistically significant negative correlation between these sequences and anti-Ro and anti-La antibody titers in patients with disseminated discoid lupus erythematosus. COMMENT:Retroviral infections have existed long before the recent HIV-1 epidemic. There is much we need to know about retrovirus-induced disease. This detailed and lengthy work deserves careful scrutiny.
Robert A. Schwart~ MD, MPH
Viral infection and cancer Morris JDH, Eddleston A L W F , Crook T. Lancet 1995;346:754-8. What is currently known about the link between viral infection and cancer? The authors review and clarify the current status of viral carcinogenesis, predicated on three observations prevalence of infection versus incidence of the associated tumor, the presence of virus versus tumor development, onset of cancer years after infection fol-
Development of complete heart block in an adult patient with Sj~igren's syndrome and anti-Ro/SSA autoantibodies L e e LA, Pickrell M B , Reichlin M. Arthritis R h e u m 1996;39:1427-9. A woman had complete heart block more than 20 years after development of Sjtgren' s syndrome. Two years be-
Journal of the American Academy of Dermatology Volume 36, Number 5, Part 1
fore the onset of heart block, arthritis developed. There was no other explanation for her heart block and the authors speculate that the high level of anti-Ro(SS-A) antibodies may be involved in the development of this process. COMMENT: This single case may be unique. However, many of our patients with subacute cutaneous lupus erythematosus have these antibodies. Thus, if syncope or dyspnea suddenly develops, it would be wise to examine such a patient for heart block.
Jeffrey P. Callen, MD
Epidermolysis bullosa acquisita in 2 children Rosifiaka-Borkowska D, Nie£,u~wska G, Kowalewski C. Przegl Dermatol 1995;82:172-7. (in Polish) Two children with a bullous eruption clinically resembling bullous pemphigoid, linear IgA bullous dermatosis, and impetigo were shown to have epidermolysis bullosa acquisita. Direct immunofluorescence studies showed IgG anti basement membrane zone antibodies against an antigen within the sub lamina densa region. This diagnosis was confirmed by direct and indirect immunofluorescence studies performed on salt-split skin. One child responded to prednisone, whereas the other reqtdred the addition of dapsone to facilitate cleating of all lesions. COMMENT: The distinction between bullous pemphigoid and epidermolysis bullosa acquisita is not always easy to determine, even with the aid of routine immtmofluorescence. These studies need to be performed on salt-split skin, or other specialized techniques need to be employed.
Robert A. SchwartL MD, MPH
Juvenile xanthogranuloma: forms of systemic disease and their clinical implications Freyer DR, K e n n e d y R, Bostrom BC, et al. J Pediatr 1996;129:227-37. The vast majority of juvenile xanthogranulomas (JXGs) are cutaneous only, but extracutaneous involvement by JXGs may be life-threatening. The authors review 34 previously reported children with systemic involvement and describe two additional affected children. The mean age of patients was 4 months, and symptoms usually resuited from the mass or infiltrative effect of lesions. Cu-
Pearls of wisdom
777
taneous lesions were present in fewer than half the patients. The most frequent sites of extracutaneous involvement were, in order of frequency, subcutaneous soft tissue, central nervous system, liver/spleen, lung, and eye/orbit/oropharynx.Whereas most patients were treated with excision or had spontaneous regression, one third of patients received radiation or systemic chemotherapy. COMMENT: Extracutaneous manifestations of JXGs are rare and are not associated with cutaneous involvement in the majority of children. Although the possibility of extracutaneous manifestations must be considered in children with JXGs, the recent study by Chang, Frieden, and Good (J Am Acad Dermatol 1996;34:445-9) shows the incidence to be 0.3% to 0.4% of children with cutaneous lesions. Factors that increase the risk of extracutaneous (particularly ocular) involvement include multiple lesions, recent onset of lesions, and occurrence in infants younger than 2 years of age.
Amy S. Puller, MD
Ocular problems in infantile periocular hemangioma and in the syndrome of SturgeWeber Vittone P, de M a r c o R. Chronica Dermatologica (Roma) 1996;6:509-16. On Italian) The authors retrospectively evaluated 16 children with periocular hemangiomas and 21 young patients with the Sturge-Weber syndrome seen at their institute from 1992 to 1995. In the former group, interferon alfa-2b appeared to be a promising therapy in the four patients in whom it was used, thereby avoiding steroid complications. Each of the four hemangiomas regressed after 4 months; systemic corticosteroid therapy showed regression in 8 of l0 patients after 6 months, with inWalesional steroids working in one of two after 6 months. In the three therapeutic categories, all hemangiomas regressed after 1 year. Patients with the Sturge-Weber syndrome showed ocular complications in 7 of 20 evaluated for it. Five of these patients had secondary glaucoma caused by increased episcleral pressure. Other findings included one with a coroidal hemangioma and one with strabismus. COMMENT:Interferon therapy may represent an important option for children with facial hemangiomas. Patients with the Sturge-Weber syndrome need to be evaluated for early-onset congenital glaucoma and late-onset glaucoma as well.
Robert A. Schwartz, MD, MPH
Journal of the American Academy of Dermatology May 1997
Pearls of wisdom
review highlights clinical manifestations and management in patients with AIDS and critiques several controversial issues and current research.
lowed by a summary of current knowledge of retroviruses (HTLV-I, HIV) and DNA and RNA tumor viruses (including HPV, hepatitis B and C viruses, herpesvirus-
es). COMMENT: This ambitious and definitive review of cryptococcosis, a resilient and devastating disease, especially in the AIDS pandemic, should be a reference for any clinician who examines and treats immunocompromised patients, especially those with AIDS.
Kenneth J. Tomecki, MD
Chancroid and Haemophilus ducreyi: an update
COMMENT:This article is a thoughtful, objective summary of viral carcinogenesis for the clinician. Viral infection is definitely instrumental in the development of some human cancers. The mechanisms and the extent to which viral infection contributes are unclear. Via molecular analysis, tumor-associated viruses function by encoding proteins that alter pathways of growth arrest and apoptosis. For further insights, read the review (it is only four pages), which is recommended for anyone with an interest in the topic.
Kenneth J. Tomecki, MD
Trees DL, M o r s e SA. Clin Microbiol R e v 1995; 8:357-75. The authors present a "state of the disease" update of chancroid, a major ulcerative disease of the genitals now associated with HIV infection. The update covers epidemiology; clinical features; diagnosis; treatment; susceptibility testing and microbial resistance; growth and nutritional requirements; strain typing; human immune response and serology; in vivo and in vitro models to study pathogenesis; potential virulence factors; genetics and classification; and several unresolved issues (e.g., prevalence of Haemophilus ducreyi in genital lesions, the role of asymptomatic carriers, virulence differences among strains). COMMENT: This article is an expert and definitive summary of chancroid (the first in 7 years), a common disease in Africa, Asia, and Latin America. As a sexually transmitted infection, chancroid may be a cofactor for the heterosexual transmission of HIV infection. As such, this review is recommended reading for any clinician who treats patients with sexually transmitted disease or H1V infection.
Kenneth J. Tomecki, MD
Association between the presence of sequences homologous to gag-pol region of the HIV-1 in DNA from sets of connective disease patients and the detection of autoantibodies against cell nucleus particles Prokop J. Post~py Dermatol 1995;12:455-61. (In Polish) A strong association was found between sequences homologous to the gag-pol region of HIV-1 in DNA and the presence of anti double-stranded DNA and anti single-stranded DNA from patients with systemic lupus erythematosus and disseminated discoid lupus erythematosus. There was also a statistically significant negative correlation between these sequences and anti-Ro and anti-La antibody titers in patients with disseminated discoid lupus erythematosus. COMMENT:Retroviral infections have existed long before the recent HIV-1 epidemic. There is much we need to know about retrovirus-induced disease. This detailed and lengthy work deserves careful scrutiny.
Robert A. Schwart~ MD, MPH
Viral infection and cancer Morris JDH, Eddleston A L W F , Crook T. Lancet 1995;346:754-8. What is currently known about the link between viral infection and cancer? The authors review and clarify the current status of viral carcinogenesis, predicated on three observations prevalence of infection versus incidence of the associated tumor, the presence of virus versus tumor development, onset of cancer years after infection fol-
Development of complete heart block in an adult patient with Sj~igren's syndrome and anti-Ro/SSA autoantibodies L e e LA, Pickrell M B , Reichlin M. Arthritis R h e u m 1996;39:1427-9. A woman had complete heart block more than 20 years after development of Sjtgren' s syndrome. Two years be-
Journal of the American Academy of Dermatology Volume 36, Number 5, Part 1
fore the onset of heart block, arthritis developed. There was no other explanation for her heart block and the authors speculate that the high level of anti-Ro(SS-A) antibodies may be involved in the development of this process. COMMENT: This single case may be unique. However, many of our patients with subacute cutaneous lupus erythematosus have these antibodies. Thus, if syncope or dyspnea suddenly develops, it would be wise to examine such a patient for heart block.
Jeffrey P. Callen, MD
Epidermolysis bullosa acquisita in 2 children Rosifiaka-Borkowska D, Nie£,u~wska G, Kowalewski C. Przegl Dermatol 1995;82:172-7. (in Polish) Two children with a bullous eruption clinically resembling bullous pemphigoid, linear IgA bullous dermatosis, and impetigo were shown to have epidermolysis bullosa acquisita. Direct immunofluorescence studies showed IgG anti basement membrane zone antibodies against an antigen within the sub lamina densa region. This diagnosis was confirmed by direct and indirect immunofluorescence studies performed on salt-split skin. One child responded to prednisone, whereas the other reqtdred the addition of dapsone to facilitate cleating of all lesions. COMMENT: The distinction between bullous pemphigoid and epidermolysis bullosa acquisita is not always easy to determine, even with the aid of routine immtmofluorescence. These studies need to be performed on salt-split skin, or other specialized techniques need to be employed.
Robert A. SchwartL MD, MPH
Juvenile xanthogranuloma: forms of systemic disease and their clinical implications Freyer DR, K e n n e d y R, Bostrom BC, et al. J Pediatr 1996;129:227-37. The vast majority of juvenile xanthogranulomas (JXGs) are cutaneous only, but extracutaneous involvement by JXGs may be life-threatening. The authors review 34 previously reported children with systemic involvement and describe two additional affected children. The mean age of patients was 4 months, and symptoms usually resuited from the mass or infiltrative effect of lesions. Cu-
Pearls of wisdom
777
taneous lesions were present in fewer than half the patients. The most frequent sites of extracutaneous involvement were, in order of frequency, subcutaneous soft tissue, central nervous system, liver/spleen, lung, and eye/orbit/oropharynx.Whereas most patients were treated with excision or had spontaneous regression, one third of patients received radiation or systemic chemotherapy. COMMENT: Extracutaneous manifestations of JXGs are rare and are not associated with cutaneous involvement in the majority of children. Although the possibility of extracutaneous manifestations must be considered in children with JXGs, the recent study by Chang, Frieden, and Good (J Am Acad Dermatol 1996;34:445-9) shows the incidence to be 0.3% to 0.4% of children with cutaneous lesions. Factors that increase the risk of extracutaneous (particularly ocular) involvement include multiple lesions, recent onset of lesions, and occurrence in infants younger than 2 years of age.
Amy S. Puller, MD
Ocular problems in infantile periocular hemangioma and in the syndrome of SturgeWeber Vittone P, de M a r c o R. Chronica Dermatologica (Roma) 1996;6:509-16. On Italian) The authors retrospectively evaluated 16 children with periocular hemangiomas and 21 young patients with the Sturge-Weber syndrome seen at their institute from 1992 to 1995. In the former group, interferon alfa-2b appeared to be a promising therapy in the four patients in whom it was used, thereby avoiding steroid complications. Each of the four hemangiomas regressed after 4 months; systemic corticosteroid therapy showed regression in 8 of l0 patients after 6 months, with inWalesional steroids working in one of two after 6 months. In the three therapeutic categories, all hemangiomas regressed after 1 year. Patients with the Sturge-Weber syndrome showed ocular complications in 7 of 20 evaluated for it. Five of these patients had secondary glaucoma caused by increased episcleral pressure. Other findings included one with a coroidal hemangioma and one with strabismus. COMMENT:Interferon therapy may represent an important option for children with facial hemangiomas. Patients with the Sturge-Weber syndrome need to be evaluated for early-onset congenital glaucoma and late-onset glaucoma as well.
Robert A. Schwartz, MD, MPH