An unusual case of hydroa vacciniforme with ocular involvement.

Hydroa vacciniforme (HV) is a sporadic, rare and idiopathic chronic photodermatosis characterized by recurrent vesicles and crust formation on a sun-exposed skin, typically resulting in vacciniform or varioliform scarring. Herein, we report on a 18-year-old boy who presented with rare ocular involvement in HV.

Analysis of myeloperoxidase promotor polymorphism and enzyme activity in Turkish patients with vitiligo.

Vitiligo is an acquired depigmentary disorder characterized by white areas of the skin due to loss of epidermal melanocytes. Oxidative stress and free radicals are suggested as important phenomena in the pathogenesis of vitiligo. Myeloperoxidase is a lysosomal enzyme of polymorphonuclear leukocytes and acts as a catalyst in the production of hypochlorous acid, a powerful oxidant. In this study we analysed enzyme activity and gene polymorphism of myeloperoxidase in patients with vitiligo. Fifty-four patients with vitiligo and 58 healthy controls were enrolled to this study. Patient groups were subdivided according to localization of the lesions; generalized, acrofacial and local. Plasma myeloperoxidase enzyme activity was determined with ELISA and G-463A gene (-463) polymorphism with the PCR-RFLP (AciI) method. The plasma MPO level was significantly lower in vitiligo patients than in the healthy controls (p = 0.005), however, it was not significantly different among subtypes of vitiligo (p = 0.8). A significant difference was not observed for G-463A genotype and allele distribution in patients with vitiligo. In conclusion, the present study is the first study investigating MPO G-463A polymorphism and enzyme levels, which warrants further studies with higher patient numbers and broader polymorphism panels.

Childhood bullous pemphigoid in association with hyperimmunoglobulin E syndrome.

Bullous pemphigoid is an acquired immunobullous disorder affecting predominantly the elderly. It is very rare in children and exceptional in infants. Hyperimmunoglobulin E syndrome is a rare primary immunodeficiency characterized by a triad of high serum levels of polyclonal immunoglobulin E with peripheral eosinophilia, recurrent staphylococcal infections of the skin and lungs, and pruritic dermatitis. Variable associated features include coarse facies, cold cutaneous abscesses, and osteopenia. This report describes, to the best of my knowledge, the first instance of childhood bullous pemphigoid associated with hyperimmunoglobulin E syndrome in a 6-month-old boy.

Association of angiotensin-converting enzyme gene insertion/deletion polymorphism with allergic contact dermatitis.

Angiotensin-converting enzyme (ACE) plays an important role in the physiological control of blood pressure and inflammation. Insertion/deletion (I/D) polymorphism of the gene for ACE was investigated in relation to cardiovascular, cerebrovascular, neurodegenerative and inflammatory diseases. The purpose of the present study was to investigate the possible association between allergic contact dermatitis and insertion/deletion polymorphism of the ACE gene. A total of 90 patients with allergic contact dermatitis and 160 control persons were enrolled in the present study. ACE I/D genotypes were determined by the polymerase chain reaction. Allelic frequencies and genotype distribution of the ACE I/D polymorphism in the patient group were significantly different from control group (ACE II genotype 30.0% versus 17.5%, P = 0.022; ACE I allele 51.7% versus 39.4%, P = 0.008). Our data suggest that the ACE polymorphism could be a risk factor for patients with allergic contact dermatitis.

Erythrokeratodermia variabilis with adult onset: report of a sporadic case unresponsive to systemic retinoids.

Erythrokeratodermia variabilis is a rare autosomal-dominant genodermatosis characterized by migratory and erythematous patches changing over the course of hours to days, and fixed keratotic plaques. The disease begins mostly at birth or within the first year of life; it very rarely starts after childhood. We present herein a sporadic case with adult onset that was unusually resistant to both isotretinoin and acitretin.

Tuberculosis verrucosa cutis in a patient with long-standing generalized lichen planus: improvement of lichen after antitubercular polychemotherapy.

Tuberculosis verrucosa cutis (TBVC) is a rare form of skin tuberculosis in Western communities, and is generally seen in certain risk groups. A 52-year-old housewife with recalcitrant generalized cutaneous and mucosal lichen planus (LP) of more than a 20-year duration presented with an erythematous-edematous plaque with a warty surface containing deep hemorrhagic fissures and numerous orifices draining purulent material on the back of her left hand. On the basis of clinical, histological and bacteriological findings, TBVC was diagnosed. The patient was successfully treated with isoniazid, rifampicin, ethambutol and pyrazinamide. Interestingly, all lichen lesions improved completely with this therapy. To the best of our knowledge, this is the first documented case of TBVC associated with LP, which was cured by antitubercular polychemotherapy. The case is presented because it reflects a possible reciprocal causal relationship between two cutaneous conditions of different natures.

Successful treatment of porokeratosis with topical imiquimod in 2 immunosuppressed cases.

Porokeratosis is a group of cutaneous disorders of keratinization characterized by a predisposition to malignant transformation. The condition, which may be associated with immune suppression, is usually resistant to therapy and has a high frequency of recurrence. Imiquimod, a potent topical immune response modifier with antiviral, antitumor, and immunoregulatory properties, is currently approved for the treatment of external anogenital warts and actinic keratosis. However, there have been also several reports demonstrating its efficacy in a variety of premalignant and malignant conditions. We report on 2 cases with immunosuppression-associated porokeratosis successfully treated with 5% topical imiquimod application.

Serum adenosine deaminase activity in monitoring disease activity and response to therapy in severe psoriasis.

OBJECTIVES: Activity of Serum Adenosine Deaminase (ADA), a main enzyme in purine degradation and considered as a marker for non-specific T cell activation, in psoriasis has been investigated in a few studies with conflicting results. DESIGN AND METHODS: To evaluate the significance of serum ADA activity in psoriasis, and analyze whether ADA activity may be related to disease activity, we performed a prospective study with 38 cases of psoriasis and 24 healthy volunteers. Patients were divided into two groups as cases with local and stable lesions (Group i, n: 20) and severe cases with extensive involvement (Group ii, n: 18). Serum ADA activity was determined by modified Guisti procedure. RESULTS: When taken into consideration of all patients--regardless of the severity of the disease--the mean serum ADA activity of psoriatics did not differ significantly from that of controls (p > 0.05). However, it was higher in Group ii than in Group i and healthy controls (respectively p < 0.001 and p < 0.05). A significant decrease was observed also after therapy only in Group ii (p < 0.001). CONCLUSION: Serum ADA activity may be correlated to the disease activity of severe psoriasis. We suggest that it might be a serologic marker for follow-up of in such cases. It could be used in predicting relapses before clinical findings as well as in deciding to stop or decrease systemic therapies at the right time, which have potential to cause severe systemic side effects when given for a long period. Further studies with larger case populations are required to support our findings.

Rapid improvement of human orf (ecthyma contagiosum) with topical imiquimod cream: report of four complicated cases.

Orf is a zoonosis caused by an epitheliotropic DNA parapox virus. Human orf is a generally benign, self-limiting condition that usually regresses in 6-8 weeks without specific treatment. However, it may be accompanied by local symptoms including pain, pruritus, lymphangitis and axillary adenitis, or less frequently by systemic symptoms such as fever or malaise. Furthermore, it may be complicated by erythema multiforme, Stevens-Johnson syndrome, erysipelas, generalized mucocutaneous eruption, toxic erythema, eyelid oedema and giant, persistent or recurrent lesions in immunocompromised patients. Imiquimod, a potent topical immune response modifier, enhances both the innate and acquired immunity by stimulation of immune system cells resulting in local antiviral, antitumour and immunoregulatory activity. We present, for the first time, four complicated cases of orf successfully treated by topical imiquimod resulting in rapid regression of both orf and associated lesions. Two of the cases were complicated with erythema multiforme, one with recurrent eyelid oedema, and another had giant orf associated with axillary lymphadenitis. We suggest that topical imiquimod may be an effective and safe therapy for complicated orf cases.

Prevalence of skin diseases among male adolescent and post-adolescent boarding school students in Turkey.

Skin disease is a common problem in boarding schools and may account for significant morbidity. To document the prevalence and patterns of skin diseases among male adolescent and post-adolescent boarding school residents, a cross-sectional epidemiologic survey was performed. A total of 682 students were examined for evidence of any skin disease, and subjects with skin disease(s) were also asked to fill in a questionnaire. Of the study population, 378 (55.42%) had at least one skin disease. The most prevalent diseases were tinea pedis (32.5%), acne vulgaris (28.6%), onychomycosis (8.06%), androgenetic alopecia (5.6%), common warts (5.3%), unguium incarnatus (4.1%), irritant hand dermatitis (3.6%), foot callosities (3.6%), and pitted keratolysis (2.6%). Of those with skin problem(s), 245 (65%) were not aware of their disease(s) and 45% of the remaining 133 subjects who were aware of their disease(s) had not sought medical help. We concluded that skin diseases, especially foot problems, are very common among male boarding school students. In additional to monitoring the epidemiology of skin diseases, intermittent medical education programs for both health-care workers and residents living in these communities would be useful for enhancing knowledge of available and effective treatments and implementing appropriate preventive measures.

Progressive pigmentary purpura related to raloxifene.

Progressive pigmentary purpura is a rare condition characterized by lymphocytic capillaritis histologically causing various clinical entities which are also named as persistent pigmented purpuric dermatoses. It is generally idiopathic; however, rare cases secondary to drugs and various diseases have been reported. In this report we describe a case of progressive pigmentary purpura induced by raloxifene, a selective estrogen receptor modulator which is primarily used in the treatment and prevention of postmenopausal osteoporosis. As far as we know, no case of progressive pigmentary purpura has previously been reported as an adverse effect of raloxifene.

Topical therapy for dermatophytoses: should corticosteroids be included?

Dermatophytoses, commonly known as ringworm or tinea, represent superficial fungal infections caused by dermatophytes, which are among the most common infections encountered in medicine. The use of corticosteroid-containing combinations in dermatophyte infections that are usually treated with topical medications is still a much-debated issue. The addition of a corticosteroid to local antifungal therapy may be of value in reducing local inflammatory reaction and thus carries the theoretical advantage of rapid symptom relief in acute dermatophyte infections associated with heavy inflammation. However, the use of such combinations requires caution as they have some potential risks, especially with long-term use under occlusive conditions. Corticosteroid-induced cutaneous adverse effects have been reported primarily in pediatric patients due to inappropriate application of these preparations on diaper areas. Additionally, the corticosteroid component may interfere with the therapeutic actions of the antifungal agent, or fungal growth may accelerate because of decreased local immunologic host reaction, such that underlying infection may persist, and dermatophytes may even acquire the ability to invade deeper tissues. Analysis of the literature documenting clinical study data and adverse reactions related to combination therapy, drew the following conclusions: (i) combination products containing a low potency nonfluorinated corticosteroid may initially be used for symptomatic inflamed lesions of tinea pedis, tinea corporis, and tinea cruris, in otherwise healthy adults with good compliance; (ii) therapy should be substituted by a pure antifungal agent once symptoms are relieved, and should never exceed 2 weeks for tinea cruris and 4 weeks for tinea pedis/corporis; and (iii) contraindications for the use of these combinations include application on diaper or other occluded areas and facial lesions, as well as in children <12 years of age and in immunosuppressed patients for any reason.

Ocular anterior segment pathologies and tear film changes in patients with psoriasis vulgaris.

Ocular manifestations in patients with psoriasis vulgaris have been investigated in only a small number of studies. Our purpose was to identify tear film function and ocular pathologies associated with psoriasis vulgaris in patients who had received neither oral retinoids nor phototherapy. We examined 62 eyes of 31 patients with psoriasis and 60 eyes of 30 age-and-sex matched healthy volunteers. In addition to complete ocular and dermatological examination, tear film function (i.e., tear secretion and tear film stability) were assessed by the Schirmer-I test, as well as by tear film break-up time. None of the controls had any ocular abnormalities, whereas 67.74% of patients with psoriasis had various anterior segment pathologies (P<0.00009). The most prevalent finding was chronic blepharoconjunctivitis (64.5%), as the only pathology (n=9) or in association with other findings, including nonspecific corneal opacities (n=4), cataract (n=3), both corneal opacities and cataract (n=2), and corneal pigment dispersion (n=2). The Schirmer-I test results revealed comparable mean values in the patient group (9.8+-4.2 mm) and in the controls (11.2+-3.7 mm; P=0.078). However, mean tear film break-up time was significantly shorter in the patients (7.2+-2.5 sec) than in the healthy persons (11.7+-3.1 sec; P=0.001). In agreement with some previous reports, our findings clearly demonstrated that early ocular involvement occurs in patients with psoriasis vulgaris, irrespective of the history of previous therapeutic modalities (e.g., retinoid therapy and phototherapy). Thus, the present findings are suggestive of the contributory role of primary etiologic factors of psoriasis in the pathogenesis of ocular changes in patients with psoriasis vulgaris.

Multiple NSAID intolerance in chronic idiopathic urticaria is correlated with delayed, pronounced and prolonged autoreactivity.

Autologous serum skin test (ASST) reactivity is positive in up to 60% of patients with chronic idiopathic urticaria (CIU). About 21 to 30% of patients with CIU have intolerance to acetyl salicylic acid (ASA) and/or other chemically unrelated non-steroidal anti-inflammatory drugs (NSAIDs). To investigate the relationship between ASA/NSAID intolerance and ASST reactivity, a case-control study was performed in 110 patients with CIU and 60 healthy controls. A positive ASST was defined as an erythematous wheal with a diameter of > 5 mm more than the saline-induced response. Patients were assessed at 10-minute intervals for a minimum of three hours. ASA/NSAID intolerance was ascertained by a placebo controlled-provocation test with offending drug (s). Forty-two patients with CIU (38.2%) had autoreactivity whereas only two of the controls (3.3%) displayed early and weak skin responses (P<.0001). ASA/NSAID intolerance was demonstrated in 30 (27.3%) patients with CIU. The prevalences of autoreactivity were 93.3% (28/30) and 17.5% (14/80) in patients with and without ASA/NSAID intolerance, respectively (P<.001). Thirteen of the 25 ASST-positive patients (52%) who had single (n: 7) or multiple (n: 6) NSAID intolerance showed early (before or at 30 min) and mild autoreactivity of short duration, whereas 15 of the remaining 17 ASST-positive patients (88.2%) who all had multiple NSAID intolerance showed delayed (later than 30 min) and prolonged autoreactivity (P<.05). These findings suggest that a common mechanism may be responsible for the pathogeneses of both delayed autoreactivity and multiple NSAID intolerance in CIU. It might be further speculated that delayed, prolonged, and pronounced autoreactivity may be a possible predictor for multiple NSAID sensitivity in CIU.

Childhood bullous pemphigoid following hepatitis B immunization.

Bullous pemphigoid (BP) is an acquired autoimmune bullous disorder which predominantly affects the elderly. It is very rare in children. There are approximately 50 reported cases of childhood BP. Although the cause of childhood BP is unknown, drug intake and vaccination have been incriminated in some cases. A total of 13 patients with BP (10 adults and 3 infants) have been described to be related to various vaccines and tetanus toxoid booster. However, no case related to hepatitis B vaccination has previously been reported. Our case of childhood BP developed one week after hepatitis B immunization in a Turkish caucasian child. This case suggests that the hepatitis B surface antigen can function as the triggering factor for BP by inducing a nonspecific immune reactivation which unmasks subclinical BP or by stimulating a specific antibody production that may cross-react with BP antigens.

Cutaneous hyalohyphomycosis and onychomycosis caused by Onychocola canadensis: report of the first case from Turkey.

We present the first Turkish case of skin and nail infection due to Onychocola canadensis in an otherwise healthy farmer who frequently worked barefoot on soil. Cutaneous involvement consisted of scaly and hyperkeratotic lesions resembling tinea pedis, erythematous plaques, and dermal papulonodules of various sizes simulating Majocchi's granuloma. Repeated cultures from nail plates, skin scrapings and needle aspiration materials from papules or nodules all yielded the same mold on Sabouroud dextrose media with and without cycloheximide, trichophyton agar, and potato dextrose agar at 26 degrees C. The causal isolate was identified as Onychocola canadensis Sigler gen. et sp. nov., a slow-growing arthroconidial hyphomycete, on the basis of its colonial and microscopic morphology. While skin lesions were responsive to daily itraconazole in a dose of 200 mg for three months, the onychomycosis was resistant to therapy. To our knowledge, this is the first presentation of O. canadensis as the cause of cutaneous hyalohyphomycosis to date.

Cutaneous involvement in Hodgkin's lymphoma: report of two cases.

Hodgkin's lymphoma (HL) comprises 20 to 30% of all lymphomas. Skin involvement is almost always secondary to visceral or nodal involvement. Secondary cutaneous HL is rare, occurring in only 0.5 to 3.4% of the cases. Herein we report two cases of skin involvement in Hodgkin's disease. One was a 25-year-old female admitted with a draining sinus in the neck. The other was a 19-year-old female admitted with a neck mass and skin papules on her chest and arm. Skin involvement may antecede or can be seen during the course of HL. Better understanding of such cutaneous involvement, which occasionally may be the initial sign of HL, is needed, and a biopsy of any suspicious skin lesion should be considered.

Amlodipine associated hyperpigmentation.

Amlodipine is a potent peripheral and coronary vasodilator with high selectivity for vascular smooth muscle, and is widely used in mild to moderate hypertension, chronic stable angina and vasospastic angina. Its most prevalent side effects are peripheral edema, flushing and headache. Cutaneous adverse reactions associated with amlodipine have been rarely reported. Herein, a male patient is described to develop oral mucosal and cutaneous hyperpigmentation one year after starting amlodipine, which became more noticeable with time. Although cutaneous hyperpigmentation was most prominent on the photoexposed areas, there was no history of previous photosensitivity, pruritus or flushing. To our knowledge, no case of oral and cutaneous hyperpigmentation associated with amlodipine has been formally reported up to date.

Unilateral nystagmus in an infant with acrocallosal syndrome.

Acrocallosal syndrome (ACS), is an extremely rare disorder characterized by the absence of corpus callosum (CC), macrocephaly, hypertelorism, pre- and postaxial polydactyly and severe motor and mental retardation. There are only 3 reports of ACS associated with ocular findings, including optic atrophy, esotropia and anophthalmus. We report on the first known Turkish case of ACS associated with unilateral nystagmus in addition to several neurologic abnormalities such as absence of the adhesio interthalamica and many others. A physically and mentally underdeveloped one year-old girl was evaluated for macrocephaly, polydactyly and left-sided nystagmus, which was not recognized until the fourth month. Magnetic resonance imaging revealed external hydrocephaly, triventricular hydrocephaly, midline brain abnormalities including partial agenesis of the CC, cavum septi pellucidi, cavum vergae, and absence of the adhesio interthalamica. The following anomalies were also noted; high arched palate, short nose with broad nasal bridge and anteverted nostrils, macrocephaly, frontal bossing, open and down turned angles of the mouth, hypertelorism, postaxial polydactyly of the left foot, hypertrichiasis, and hypertrichosis. On the basis of these findings, a diagnosis of ACS was made. In addition to neuroimaging, systemic research is needed in all patients presenting with asymmetric nystagmus as such a nystagmus may be associated with various external developmental abnormalities in addition to central nervous system involvement. Our case indicates that asymmetric nystagmus and midline brain abnormalities may also be included in the diagnostic criteria of ACS.