Stephan Lautenschlager

Eosinophilic fasciitis 30 years after - what do we really know? Report of 11 patients and review of the literature.

Background: Eosinophilic fasciitis (EF) is a rare fibrosing disorder associated with peripheral eosinophilia and scleroderma-like induration of the distal extremities which affects substantially quality of life. Although the disease has been described 30 years ago, the etiology and pathomechanisms are still obscure, and consensus for therapy is lacking. Numerous case reports of patients with EF exist but series are scarce. Patients and Methods: Eleven patients with EF from the Department of Dermatology, Kantonsspital Aarau, the University Hospital Basel and the Outpatient Clinic of Dermatology, Triemli Hospital Zurich, Switzerland, were retrospectively studied. Results: In 4 patients the initial diagnosis was not recognized by the referring nondermatologists. The median age was 55 years, excluding the youngest patient ever diagnosed with EF (age = 1 year). All patients showed an induration of the skin, which led to painful contractures in the joints in 3 cases. All but 2 patients demonstrated edema. A slight predominance of the upper extremities was observed. Sclerodactyly was noticed in 1 patient. Three patients reported an initial trauma at the affected site. Two patients were tested positive for borreliosis. One patient subsequently developed aplastic anemia and Hashimoto thyroiditis. Visceral or extracutaneous involvement was absent. Eight patients had a full or partial recovery under corticosteroids whereas in 2, improvement could be achieved only with cyclosporine, azathioprine or cyclophosphamide. Conclusions: The diagnosis of EF can be established by clinical, laboratory and histological findings. In general, corticosteroids are highly efficacious in EF and only a minority of patients need other immunosuppressive or cytostatic drugs.

Aquagenic Syringeal Acrokeratoderma (Transient Reactive Papulotranslucent Acrokeratoderma)

In 1996, English and McCollough described an unusual entity in 2 sisters characterized by a transient and recurrent keratoderma exclusively on the palms after water exposure. The condition developed 3–5 min after exposure to water and resolved within a short time after drying. This finding was associated with a tightening sensation. Yan et al. coined the term ‘aquagenic palmoplantar keratoderma’, and the designation ‘aquagenic syringeal acrokeratoderma’ was suggested by MacCormack et al. Until now, a total of 8 cases have been reported. We documented 2 new cases with acquired aquagenic syringeal acrokeratoderma. A 25-year-old female had observed within the last 3 months a burning sensation on the palms after some minutes of water contact. Physical examination revealed a perfectly normal skin on the palms. Three minutes after water immersion of 20°C, a whitish discoloration appeared on the palms and a thickening of the palmar skin was visible. In addition, the eccrine pores were much more prominent. Few minutes after drying the skin, the situation returned to a normal state. The second patient, a 33-year-old female noticed a painful whitish discoloration of the skin on the palms after a short period of water immersion. Sometimes the white skin could be peeled off. In the last year, hyperhidrosis developed, and a more reddish aspect of the palms appeared. In our office after rinsing the hands with water at room temperature, a whitish discoloration in the center of the palms appeared which was associated with a painful sensation. After drying, the whitish lesions disappeared almost completely within 30 min. Aquagenic palmar keratoderma describes an acquired and transient condition which occurs after brief exposure to water and disappears after drying within minutes to an hour. Only rarely may a slight hyperkeratosis remain for a longer time. The possible pathophysiology and treatment options are discussed.

Cutaneous manifestations of syphilis : recognition and management.

After a marked decline in the number of syphilis cases in the context of AIDS prevention campaigns, a significant increase has been observed in states of the former Soviet Union since 1994. In recent years, outbreaks have also been reported in the US, Canada, and several European countries. The current epidemic in the US and in different parts of Europe has largely involved men who have sex with men, many of whom are infected with HIV. Since a misdiagnosis of syphilis can have serious consequences for the patient and also for pregnancies and newborns, clinicians should be aware of the many manifestations of syphilis and difficulties in the diagnosis and management of the disease. Younger clinicians in particular are no longer familiar with the diverse clinical symptoms and the complex diagnostics of syphilis. Patients co-infected with HIV may present with atypical clinical manifestations and laboratory test results. Furthermore, through its association with an increased risk of HIV infection, syphilis has acquired a new potential for morbidity and mortality, and the diagnosis of syphilis should be routinely considered in patients with uveitis, sudden deafness, aortic thoracic aneurysm, or pregnancy.Only a minority of syphilis infections are detected in the primary stage. This may be because of atypical locations and, occasionally, atypical morphology of the lesions; however, it may also be because of the difficulty of detecting the pathogen. In the secondary stage, which is clinically extremely diverse, the diagnosis is confirmed serologically. There is a need for increased awareness of the symptoms and signs of acute infections, together with a willingness to consider the diagnosis of syphilis in patients with vague symptoms. An increasing number of diagnostic tests (both specific and nonspecific) are now available. However, in the absence of clinical symptoms or in cases with a low titer or inconsistent test results, diagnosis of syphilis can be difficult or even impossible. Treatment and follow-up should follow current guidelines designed for the involved area.In this article, the cutaneous manifestations of syphilis and their diagnostic and therapeutic management are described in detail.

Cowden Syndrome – Diagnostic Skin Signs

Cowden syndrome is a rare autosomal dominant familial cancer syndrome with a high risk of breast cancer. The most important clinical features include carcinomas of the breast and thyroid, and hamartomatous polyps of the gastrointestinal tract. There are characteristic mucocutaneous features which allow early recognition of the disease and are generally present before internal malignancies develop. We report on a woman in whom the diagnosis of Cowden syndrome was first made after she had been treated for both breast cancer and melanoma.

Assessment of a real-time PCR test to diagnose syphilis from diverse biological samples

Objectives: To investigate the contribution of a real-time PCR assay for the detection of Treponema pallidum in various biological specimens with the secondary objective of comparing its value according to HIV status. Methods: Prospective cohort of incident syphilis cases from three Swiss hospitals (Geneva and Bern University Hospitals, Outpatient Clinic for Dermatology of Triemli, Zurich) diagnosed between January 2006 and September 2008. A case–control study was nested into the cohort. Biological specimens (blood, lesion swab or urine) were taken at diagnosis (as clinical information) and analysed by real-time PCR using the T pallidum 47 kDa gene. Results: 126 specimens were collected from 74 patients with primary (n  =  26), secondary (n  =  40) and latent (n  =  8) syphilis. Among primary syphilis, sensitivity was 80% in lesion swabs, 28% in whole blood, 55% in serum and 29% in urine, whereas among secondary syphilis, it was 20%, 36%, 47% and 44%, respectively. Among secondary syphilis, plasma and cerebrospinal fluid were also tested and provided a sensitivity of 100% and 50%, respectively. The global sensitivity of T pallidum by PCR (irrespective of the compartment tested) was 65% during primary, 53% during secondary and null during latent syphilis. No difference regarding serology or PCR results was observed among HIV-infected patients. Specificity was 100%. Conclusions: Syphilis PCR provides better sensitivity in lesion swabs from primary syphilis and displays only moderate sensitivity in blood from primary and secondary syphilis. HIV status did not modify the internal validity of PCR for the diagnosis of primary or secondary syphilis.

Diagnosis of syphilis: Clinical and laboratory problems

JDDG | 12 ̇2006 (Band 4)

Localized scleroderma associated with Borrelia burgdorferi infection: Clinical, histologic, and immunohistochemical observations

BACKGROUND Recent reports have implicated Borrelia burgdorferi infection as a possible cause of localized scleroderma (LS). OBJECTIVE Our purpose was to describe the clinical, histologic, and immunopathologic features of patients with LS who had serum antibodies to B. burgdorferi. METHODS Ten patients were examined clinically and by routine microscopy. Biopsy specimens from seven patients were studied immunohistochemically with monoclonal antibodies. The proliferative response of peripheral blood mononuclear cells to B. burgdorferi was investigated in seven patients by lymphocyte proliferation assay. RESULTS Seven patients had plaque-type morphea, and three patients had linear scleroderma. Two patients had a history of previous erythema migrans. One patient had coexistent acrodermatitis chronica atrophicans, and in two patients lichen sclerosus et atrophicus was observed. Histologically, a prominent inflammatory phase with sclerosis of the connective tissue was shown in all patients. Immunohistochemical studies revealed that the inflammatory infiltrates consisted of both B and T lymphocytes, predominantly of the CD4+ subset. All 10 patients had strongly elevated serum antibodies to B. burgdorferi. Patients with LS showed significantly elevated lymphoproliferative responses to B. burgdorferi when compared with healthy control subjects. CONCLUSION Our findings suggest that some cases of LS are linked to Borrelia infection.

Viral Lesions of the Mouth in HIV-Infected Patients

Viral lesions of the mouth in patients with HIV infection are common and these diseases any be a marker for HIV and disease progression. We review the spectrum of oral viral manifestations and discuss treatment modalities. The most common Epstein-Barr virus (EBV)-induced disorder in HIV-infected patients is oral hairy leukoplakia. EBV-related oral B-cell and T-cell lymphoma in AIDS patients has been described repeatedly. Herpes virus type 1 and rarely type 2 may lead to painful and resistant oral ulcers, and systemic treatment with acyclovir, valaciclovir or famciclovir is indicated. In acyclovir-resistant cases foscarnet is the treatment of choice. In recent years it has been documented that Kaposis sarcoma, which often affects oral mucosa, is probably induced by herpesvirus type 8. Cytomegalovirus was found in 53% of cases with herpesviridae-induced mucosal ulcers as the only ulcerogenic viral agent in AIDS patients. In severe cytomegalovirus infection treatment with ganciclovir is helpful. Viral warts induced by different HPV may occur in the mouth. Several physical treatment modalities are possible in the oral mucosa. In AIDS patients mollusca contagiosa may occur as large and atypical lesions in the face and lips and rarely in the oral cavity. Cryotherapy is a bloodless treatment in such patients.

Oral manifestations in HIV-infected patients: diagnosis and management.

Oral lesions have been observed since the beginning of the AIDS epidemic. The number of HIV-infected patients is still increasing, especially in the heterosexual population. Oral diseases in HIV-infected patients are often more difficult to diagnose because the clinical presentations may differ from the same diseases in HIV-negative patients. HIV-associated oral lesions have diagnostic, prognostic, and therapeutic impact. Approximately 10% of the HIV-infected population will have oral manifestations as a first sign of their disease. In HIV-infected men oral hairy leukoplakia and oral candidiasis are useful markers for disease progression. This article summarizes the oral manifestations and the management of oral health in persons with HIV infection.

The Heterogeneous Clinical Spectrum of Genital Herpes

Background and Objective: Most data of genital herpes have been collected in STD clinics in the USA where unrecognized forms accounted for 80% of HSV-2 infections. Our aim was to study the clinical features in an outpatient clinic of dermatology. Methods: The charts of 170 patients, previously monitored prospectively for a HIV prevalence study, with culture-confirmed genital herpes or herpetic infection with HSV-2 at any other site presenting between 1995 and 1999 were analyzed. Results: 111 (65%) men and 59 (35%) women were identified with a mean age of 44 years. Only 49% had a typical cluster of genital lesions. Eighty-six (51%) presented with either lesions at extragenital sites [mostly the buttocks 33/170 (19%), thigh 10/170 (6%), anal region 9/170 (5%) and fingers 8/170 (5%)] or showed morphologically atypical forms of isolated genital lesions [single ulcer 16/170 (9%), erosion 6/170 (4%), crust 3/170 (2%) and fissure, edema or erythema each 1/170 (1%)]. Women significantly presented more often with extragenital infections of HSV-2 [36/59 (61%)] than men [18/111 (16%)]. Conclusions: More than half of the patients with genital herpes of a mainly immunocompetent population presented with atypical manifestations. The underdiagnosis of genital herpes seems largely due to misinterpretation of atypical genital and extragenital lesions.