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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.

Stevens-Johnson syndrome is a severe mucocutaneous disease following drugs or infections. We present a 7-year-old boy with mucous membrane lesions (stomatitis, conjunctivitis, and urethritis) but without skin lesions. The diagnosis of acute Mycoplasma pneumoniae infection strongly suggests a concomitant Fuchs syndrome.

Pyemotes mites, of which the grain itch mite is the most important for human medicine, are ectoparasites of many insect species. Human infestation occurs only accidentally and temporally. The source materials are most often grain, straw and plant-based foods. Bites produce an intensely itching urticarial wheal capped by pin-point vesicles, which often are destroyed by scratching. Pyemotes dermatitis favours the back, the abdomen and the flexor side of the forearms. The mites are barely visible to the naked eye and reside only briefly on humans and thus are not found by physicians during clinical examination. When itching starts, the parasites have usually left their hosts. Therefore it is important to investigate the patient's environment and to identify the infested materials. We describe an outbreak of Pyemotes dermatitis in at least 16 persons, most of them warehouse workers in a hobby mail-order house. Infested decorative wheat was the source of the problem. Successful eradication was achieved by fumigation of the storage facilities with the insecticide methylbromide. Our cases demonstrate the importance of examination of environmental specimens for ectoparasites in cases of unexplained dermatoses. Biological agriculture avoiding insecticides may cause reappearance of this almost forgotten skin disease.

Besides the inherited forms of mottled and reticulate pigmentation, a vast number of diseases and trigger mechanisms can lead to acquired pigmentation of the neck. Nonhereditary variants of reticulate and mottled pigmentation can affect the neck as a typical site and therefore may give a diagnostic clue or it can occur sporadically on the neck as well as on other sites. A well-known and important factor in the pathogenesis is exposure to sunlight. Sun-induced pigmentation often presents on the neck and may result from phototoxic, photoallergic and cumulative actinic damage. Frequent forms comprise berloque dermatitis, Riehl’s melanosis, poikiloderma of Civatte and tanning bed lentigines. Different infections may also lead to this distinct skin alteration as pediculosis capitis, pityriasis versicolor and syphilis II. Treatment-induced irregular pigmentations may occur after applications of topical agents (e.g. diphenylcyclopropenone), systemic medication (e.g. 5-fluorouracil, chlorpromazine), as a complication of laser resurfacing or as a chronic graft-versus-host reaction. Different neoplasms may also involve the neck. Widespread pigmented basal cell carcinoma, cutaneous T-cell lymphoma, syringolymphoid hyperplasia and histiocytic diseases may lead to reticulated pigmentation. Various other infrequent conditions as connective tissue diseases, malnutrition, lichen planus pigmentosus and others are summarized. The neck, a readily accessible site to medical inspection, may have an underestimated value for the diagnosis of different skin diseases.

Background CD30 is expressed in aggressive and Epstein–Barr virus–associated forms of B-cell non-Hodgkin lymphomas, but is rarely expressed by the majority of tumor cells in primary cutaneous B-cell lymphomas (CBCLs). The expression of CD30 in CBCLs may be at risk for misinterpretation as an unequivocal indicator of a highly aggressive form of the disease. Objective We report 4 cases of low malignant primary cutaneous follicle center lymphoma (PCFCL) with diffuse and strong expression of CD30 by the majority of neoplastic cells. Results The patients included 3 men and 1 woman with tumors on the scalp (3 patients) and chest wall (1 patient). The histologic examinations revealed a mixed, diffuse, and follicular growth pattern with CD20 + , bcl-6 + , and bcl-2 − tumor cells. Seventy percent to 90% of the tumor cells expressed CD30. Clonal rearrangement of immunoglobulin heavy chain genes was found in 1 of 4 cases. None of the 3 cases yielded positivity for Epstein–Barr virus RNA. Limitations The study is limited by the small number of patients. Conclusions This rare variant of CD30 + PCFCL needs be distinguished from CD30 + aggressive B-cell lymphomas. CD30 in this variant of CBCLs may serve as a therapeutic target for anti-CD30 antibody–based strategies.

Die Seropravalenz von HSV-1 betragt etwa zwei Drittel, diejenige von HSV-2 ein Funftel. Atypische Manifestationen bezuglich Morphologie und Lokalisation sind haufig., und der Anteil an genitalen HSV-1-Infektionen nimmt zu. Ausgepragte oder haufige Rezidive werden zunehmend mit hochdosierter Kurzzeitbehandlung oder Dauersuppression angegangen.

Hande und Nagel haben eine wesentliche Bedeutung in der sozialen Interaktion. Gesund aussehende Nagel sind wesentlicher Bestandteil des Korperbilds. Dementsprechend gross ist die Morbiditat bei Nagelveranderungen, sowohl in funktioneller als auch ‒ durch die kosmetische Entstellung ‒ in sozialer Hinsicht

The incidence of sexually transmitted infections is rising in Europe and in Switzerland since the beginning of the third millenium. Many organisms may affect the perianal skin and the anorectum. While some of these infections are a result of contigous spread from genital infection, most result from receptive anal intercourse affecting males who have sex with males but is seen increasingly in females as well since there is evidence of the increasing popularity of anal sex among heterosexuals. The symptoms of specific infections are largely dependent on the route and site of inoculation. Organisms that cause typical genital symptoms - such as syphilis, chancroid, herpes simplex or HPV-infection - result in similar symptoms when the perianal skin, the anoderm or the distal anal canal are the site of infection. Patients with proctitis may have unspecific signs in various degrees including mucous discharge, rectal bleeding, anorectal pain, superficial ulcers and sometimes generalized lymphadenopathy and fever. It is of utmost importance to include STIs (e. g. lymphogranuloma venereum, gonorrhea, non-LGV-chlamydia and herpes simplex) in the differential diagnosis in these patients. Unfortunately rectal infection with chlamydia and gonorrhea is asymptomatic in the majority of cases of men having sex with men and in high-risk females. A careful history and physical examination is essential in establishing a correct diagnosis, usually supported by proctoscopy, culture, PCR, serology and histology. Certain organisms, more commonly thought of as food- or water-borne disease may be sexually transmitted by direct or indirect fecal-oral contact from various sexual practices.Seit über 10 Jahren sind wir in der Schweiz mit steigenden Zahlen von Geschlechtskrankheiten konfrontiert. Zahlreiche Erreger können neben genitalen auch anorektale Veränderungen verursachen. Während es sich hierbei gelegentlich um eine Ausbreitung der Infektion per contingentatem handelt, findet überwiegend eine direkte Inokulation nach rezeptivem Analverkehr häufig bei homosexuellen Männern aber auch bei Frauen statt. Perianal und teilweise endoanal ist die klinische Präsentation von Geschlechtskrankheiten wie Syphilis, Herpes, Kondylome oder Ulcus molle vergleichbar mit derjenigen in der Genitalregion. Bei Patienten mit Proktitis findet sich bei zahlreichen Erregern eine ähnliche Symptomatik in variierender Intensität, weshalb der Erregeridentifikation im Labor eine große Bedeutung zukommt. Neben einer Sexualanamnese ist eine Untersuchung der Analregion unter Zuhilfenahme eines Proktoskopes, Abstrichen für PCR und Kultur sowie serologischen und allenfalls histologischen Abklärungen für eine korrekte Diagnose von STI unabdingbar.

In spite of advances in the local treatment of malignant skin tumours and other skin diseases, radiotherapy remains highly relevant as a therapeutic alternative or under certain conditions even as the treatment of choice. Hardly any other procedure is capable of treating superficial as well as deep skin tumours with such precision according to their depth and without causing serious damage to healthy tissue. In particular, curative radiotherapy is most often used to treat basal cell carcinomas, squamous cell carcinomas, extensive precancerous lesions, lentigo maligna and lentigo maligna melanoma. Ideally, moderately to larger-sized non-melanoma skin cancers in the facial region in patients over the age of 60 are treated with radiotherapy when surgery is not feasible. Even for inflammatory skin conditions such as chronic eczema or psoriasis, radiotherapy can be performed in cases of failure of other treatment methods. Palliative radiotherapy may be indicated for Kaposi’s sarcoma and T- and B-cell lymphoma; even primary cutaneous B-cell lymphoma can be treated curatively. The radiation quality mostly used in dermatology lies between 12 and 50 kV. Knowledge of physical and radiobiological parameters is important such as half-value depth, inverse square law and absorption law. Fractionated radiotherapy is an effective and well-tolerated outpatient treatment modality which should be considered according to the exact diagnosis, age of the patient, comorbidities, goals of treatment, lesion characteristics including location and not least patient preference.

Although there have been significant advances in diagnosis, treatment, and education, the occurence of STI continues to increase worldwide as well as in Switzerland. The most common STI include human papillomavirus (HPV), chlamydia, herpes, simplex virus types 1 and 2, gonorrhea, syphilis, hepatitis B and trichomoniasis. Diagnosis and treatment continue to be major public health challenges because of the asymptomatic nature of many of these infections. Recent studies showed favorable epidemiologic trends for HPV infection in vaccinated females as well as in some males. On the other hand we are confronted with rising incidences e.g. in urethritis with various causative agents and in syphilis cases. Alarmingly during the last 3 years gonococci have become less susceptible to our remaining antibiotics, necessitating strict adherence to updated treatment guidelines. Other trends in STIs and their consequences are discussed.

In einer Multicenterstudie wurden insgesamt 100 Patienten mit chronischer Urtikaria mittels eines standardisierten Personlichkeitstests (Giesen-Test), eines standardisierten Beschwerdebogens (Giesener Beschwerdebogen) und eines selbstentwickelten Fragebogens zu Symptomatik, Krankengeschichte und Verhalten im Krankheitsschub befragt. Ein knappes Drittel der untersuchten Patienten zeigte erhohte Depressionswerte und einen hohen Beschwerdedruck, der sich aus Korpersymptomen zusammensetzt, die im Rahmen einer Depression auftreten konnen. Die vorliegenden Ergebnisse erlauben es nicht, eine chronische Urtikaria per se als Somatisierungsstorung aufzufassen. Da bei einem Drittel der untersuchten Patienten hinsichtlich der Depressivitat Abweichungen von der Normalbevolkerung zu finden sind, wird fur eine dermatologisch-psychosomatische Simultandiagnostik pladiert, um den Urtikariapatienten eine der unterschiedlich individuellen Gesamtproblematik entsprechende Behandlung zukommen zu lassen.

Zosteriform metastasis is a rare form of tumor spread to the skin that most often arises from an internal carcinoma or a hematologic malignancy. We describe a 29-year-old woman with malignant melanoma of the back in whom zosteriform metastases developed along the fifth thoracic dermatome.

Der 68-jahrige, bislang gesunde Patient wurde wegen seit zwei Tagen bestehendem Fieber, Nausea und Emesis sowie einer scharf begrenzten Rotung mit Blasenbildung am rechten Hemiabdomen zugewiesen.

Objective To systematically review and estimate pooled sensitivity and specificity of the polymerase chain reaction (PCR) technique compared to recommended reference tests in the diagnosis of suspected syphilis at various stages and in various biological materials. Design Systematic review and meta-analysis. Data sources Search of three electronic bibliographic databases from January 1990 to January 2012 and the abstract books of five congresses specialized in the infectious diseases' field (1999-2011). Search key terms included syphilis, Treponema pallidum or neurosyphilis and molecular amplification, polymerase chain reaction or PCR. Review methods We included studies that used both reference tests to diagnose syphilis plus PCR and we presented pooled estimates of PCR sensitivity, specificity, and positive and negative likelihood ratios (LR) per syphilis stages and biological materials. Results Of 1160 identified abstracts, 69 were selected and 46 studies used adequate reference tests to diagnose syphilis. Sensitivity was highest in the swabs from primary genital or anal chancres (78.4%; 95% CI: 68.2-86.0) and in blood from neonates with congenital syphilis (83.0%; 55.0-95.2). Most pooled specificities were ∼95%, except those in blood. A positive PCR is highly informative with a positive LR around 20 in ulcers or skin lesions. In the blood, the positive LR was

The estimation of bite force and bite performance in fossil and extinct animals is a challenging subject in palaeontology and is highly dependent on the reconstruction of the cranial myology. Furthermore, the morphology and arrangement of the adductor muscles considerably affect feeding processes and mastication and thus also have important dietary and ecological ramifications. However, in the past, the reconstruction of the (cranial) muscles was restricted to the identification of muscle attachment sites or simplified computer models. This study presents a detailed reconstruction of the adductor musculature of the Cretaceous therizinosaur Erlikosaurus andrewsi based on a stepwise and iterative approach. The detailed, three-dimensional models of the individual muscles allow for more accurate measurements of the muscle properties (length, cross-section, attachment angle and volume), from which muscle and bite force estimates are calculated. Bite force estimations are found to be the lowest at the tip of the snout (43–65 N) and respectively higher at the first (59–88 N) and last tooth (90–134 N) position. Nevertheless, bite forces are comparatively low for E. andrewsi, both in actual numbers as well as in comparison with other theropod dinosaurs. The results further indicate that the low bite performance was mainly used for leaf-stripping and plant cropping, rather than active mastication or chewing processes. Muscle and thus bite force in E. andrewsi (and most likely all therizinosaurs) is considerably constrained by the cranial anatomy and declines in derived taxa of this clade. This trend is reflected in the changes of dietary preferences from carnivory to herbivory in therizinosaurs.

Skin cancers remain the most commonly diagnosed neoplasms among organ transplant recipients (OTRs), comprising nearly 40% of all posttransplant malignancies. The occurrence of skin tumors has a considerable impact on the quality of life and the overall survival of OTRs. Ultraviolet radiation (UVR) is the major environmental cause of nonmelanoma skin cancers, and it is therefore pivotal to protect the skin from UVR in order to prevent de novo and progression of preinvasive and invasive malignancies. The present chapter delivers background information on UVR, photoprotection and accompanying concerns regarding vitamin D, as well as educational and behavioral aspects of photoprotection in OTRs; some common misconceptions and mistakes regarding photoprotection are addressed as well.

Serology is the mainstay for syphilis diagnosis and treatment monitoring. We investigated serological response to treatment of syphilis according to disease stage and HIV status.A retrospective cohort study of 264 patients with syphilis was conducted, including 90 primary, 133 secondary, 33 latent, and 8 tertiary syphilis cases. Response to treatment as measured by the Venereal Disease Research Laboratory (VDRL) test and a specific IgM (immunoglobulin M) capture enzyme-linked immunosorbent assay (ELISA; Pathozyme-IgM) was assessed by Cox regression analysis.Forty-two percent of primary syphilis patients had a negative VDRL test at their diagnosis. Three months after treatment, 85%-100% of primary syphilis patients had reached the VDRL endpoint, compared with 76%-89% of patients with secondary syphilis and 44%-79% with latent syphilis. In the overall multivariate Cox regression analysis, serological response to treatment was not influenced by human immunodeficiency virus (HIV) infection and reinfection. However, within primary syphilis, HIV patients with a CD4 count of500 cells/μL had a slower treatment response (P = .012). Compared with primary syphilis, secondary and latent syphilis showed a slower serological response of VDRL (P = .092 and P.001) and Pathozyme-IgM tests (P.001 and P = .012).The VDRL should not be recommended as a screening test owing to lack of sensitivity. The syphilis disease stage significantly influences treatment response whereas HIV coinfection only within primary syphilis has an impact. VDRL test titers should decline at least 4-fold within 3-6 months after therapy for primary or secondary syphilis, and within 12-24 months for latent syphilis. IgM ELISA might be a supplement for diagnosis and treatment monitoring.

Skin cancers remain the most commonly diagnosed neoplasms among organ transplant recipients (OTRs), comprising nearly 40% of all posttransplant malignancies. The occurrence of skin tumors has a considerable impact on the quality of life and the overall survival of OTRs. Ultraviolet radiation (UVR) is the major environmental cause of nonmelanoma skin cancers, and it is therefore pivotal to protect the skin from UVR in order to prevent de novo and progression of preinvasive and invasive malignancies. The present chapter delivers background information on UVR, photoprotection and accompanying concerns regarding vitamin D, as well as educational and behavioral aspects of photoprotection in OTRs; some common misconceptions and mistakes regarding photoprotection are addressed as well.

We report a 6-year-old boy who developed depigmentation within a congenital melanocytic nevus at the age of 3 years. During the following months a halo phenomenon and vitiligo-like lesions distant from the nevus appeared. A thorough search for malignant melanoma was negative. A second patient, a 45-year-old woman, had a large congenital nevus on the trunk with marked satellitosis. At the age of 20 years, partial regression of the large nevus occurred and, in addition, halos developed around almost all smaller nevi. Repeated searches for associated malignant melanoma were negative. We review the rare cases of halo congenital nevi and emphasize that depigmentation is not necessarily associated with malignant degeneration.

Corynebacterium ulcerans is mainly known for its ability to cause animal infections. Some strains of C. ulcerans produce diphtheria toxin, which can cause life-threatening cardiopathies and neuropathies in humans. Human cutaneous C. ulcerans infection is a very rare disease that mimics classical cutaneous diphtheria. We present a very rare case of a C. ulcerans skin infection caused by a non-diphtheria toxin-producing strain of C. ulcerans that resolved after 3 weeks of therapy with amoxicillin-clavulanate. A pet cat was the probable source of infection. The presence of C. ulcerans in the mouth of the cat was confirmed by 16S rRNA gene analysis and the API Coryne system. In cases of human infection with potentially toxigenic corynebacteria, it is important to determine the species and examine the isolate for diphtheria toxin production. If toxigenicity is present, diphtheria antitoxin should be administered immediately. Carriers and potential infectious sources of C. ulcerans include not only domestic livestock but also pet animals. For the primary prevention of disease caused by diphtheria toxin-producing corynebacteria, vaccination with diphtheria toxoid is recommended.

Chancroid is a sexually acquired disease caused by Haemophilus ducreyi. The infection is characterized by one or more genital ulcers, which are soft and painful, and regional lymphadenitis which may develop into buboes. The infection may easily be misidentified due to its rare occurrence in Europe and difficulties in detecting the causative pathogen. H. ducreyi is difficult to culture. Polymerase chain reaction (PCR) can demonstrate the bacterium in suspected cases. Antibiotics will usually be efficient for curing chancroid.

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.

Stevens-Johnson syndrome is a severe mucocutaneous disease following drugs or infections. We present a 7-year-old boy with mucous membrane lesions (stomatitis, conjunctivitis, and urethritis) but without skin lesions. The diagnosis of acute Mycoplasma pneumoniae infection strongly suggests a concomitant Fuchs syndrome.

A 19-year-old man presented with phimosis and painful swelling of the penis four weeks after augmentation with silicone in Thailand. Histology revealed a foreign body reaction to silicone. Infectious causes were ruled out. Granulomatous foreign body reactions to silicone are common, but there are few case reports on reactions following silicone injection for penis enlargement. Foreign body reactions should be included in the differential diagnosis of penis swelling.

Une concentration adequate de vitamine D est sans aucun doute essentielle a notre sante. De tres nombreuses publications et articles scientifiques controverses ont paru ces derniers temps dans la presse grand public sur les questions de l’importance de la vitamine D, de sa concentration souhaitable et de la meilleure maniere de l’atteindre. Comme il n’y a que 3 sources de cette vitamine D consideree comme etant de plus en plus importante – soleil, alimentation et complements dietetiques – il faut consciencieusement examiner ce que cela implique, sur la base des donnees actuelles.

BACKGROUND: The diagnosis of parapox virus infections relies primarily on a history of contact with infected animals. The clinical presentation is usually a non-specific necrotic ulcer. The histology may also be non-specific, especially with older lesions. Negative-staining electron microscopy (EM) is a fast and reliable diagnostic tool, but is not widely available. Serological tests and the time-consuming viral culture are also rarely used in Europe. PATIENTS AND METHODS: The diagnostic procedure in two patients with ecthyma contagiosum and milker's nodule using polymerase chain reaction specific for orthopox, parapox and Orf virus is explained. Diagnostics included bacterial culture, viral culture, histology and EM. In addition to these, a polymerase chain reaction (PCR) was performed in both cases. RESULTS: The patient with ecthyma contagiosum was negative for ortho-, parapox-, and orf-virus on PCR, whereas the patient with milker's nodule had a PCR positive for parapoxvirus. CONCLUSIONS: PCR is a simple, fast, and standardized method of diagnosis that can distinguish between the subgroups of parapoxviruses. A diagnosis can be made even in cases of ambiguous history or unspecific clinical presentation. The method is limited by the necessity to sample native material or to use neutrally buffered formalin in case of PCR from paraffin material.

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.

An increasing number of syndromes with palmoplantar keratoderma (PPK) with associated diseases are being identified, representing a wide spectrum of distinct entities. At present only one case report has described the combination of marked anogenital leukokeratosis with diffuse PPK evolving in a collodion baby. We report a patient with a diffuse, nonprogressive PPK in combination with an intermittently pruritic, slowly progressive anogenital leukokeratosis. Hyperkeratosis of the perineal area was most pronounced and extended to the distal portion of the anal mucosa. The opalescent lesion was also visualized at the margin of the major labia. Vulvar structures were not otherwise involved or dystrophic. There were no signs or symptoms of ectodermal dysplasia. Specifically, the nails were normal and showed no signs of pachyonychia congenita. Other differential diagnoses included dyskeratosis congenita and white sponge nevus, which may be associated with anogenital leukokeratosis, but a keratoderma is not associated with these entities. Keratin immunocytochemistry showed marked expression of suprabasal K17 and absence of K6 and K16. Further examination of the initial case described by Itin and Rufli demonstrated the same expression pattern and supports the contention that these two cases represent the same entity.