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.

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.

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.

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.

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.

Human arylamine N-acetyltransferase 2 polymorphism and susceptibility to allergic contact dermatitis.

BACKGROUND: N-acetyltransferase 2 (NAT2) polymorphism may be involved in the pathogenesis of allergic contact dermatitis. OBJECTIVE: The present study was designed to evaluate whether acetylation polymorphism plays a role in the susceptibility to p-Phenylenediamine (PPD) sensitization. METHODS: The frequencies of seven NAT2 point mutations, namely G191A, C282T, T341C, C481T, G590A, A803G, and G857A, and genotypes were determined by PCR/RFLP in a total of 70 patients with allergic contact dermatitis to PPD and 100 control subjects with no history of allergy, atopy, lung disease, diabetes mellitus and cancer. RESULTS: Genotypes coding rapid acetylation were detected in 52.9% and 37.0% of patients with contact dermatitis and control subjects, respectively (P = 0.04). The frequency of the NAT2*4 allele and NAT2*4/*4 genotype, coding for rapid acetylation, were also significantly higher in the contact dermatitis patients than in the control subjects (P = 0.003). CONCLUSION: Our results suggest an association between rapid acetylation polymorphism and susceptibility to PPD sensitization.

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.

Successful treatment of antifungal- and cryotherapy-resistant subcutaneous hyalohyphomycosis in an immunocompetent case with topical 5% imiquimod cream.

Hyalohyphomycosis is an unusual opportunistic mycotic infection where the tissue morphology of the causative organism is mycelial. Etiological agents, which are not responsible for the otherwise-named infections like aspergillosis, are the species of non-dematiaceous hyaline hyphomycetes including Penicillium, Paecilomyces, Acremonium (formerly known Cephalosporium), Beauveria, Fusarium, and Scopulariopsis. Several cases of Acremonium infection have been described in immunocompromised patients; however it can cause invasive disease in an immunocompetent person very rarely. Optimum therapy of Acremonium infection is unclear because of the limited number of reported cases and conflicting results of therapies. Imiquimod, an imidazoquinoline with potent antiviral, antitumor and immunoregulatory properties, is currently approved for the topical treatment of external anogenital warts and actinic keratosis. Imiquimod has also been found to be effective for other virus-associated dermatologic lesions, including common and flat warts, molluscum contagiosum, and herpes simplex virus type-2 as well as for some cases of cutaneous leishmaniasis. We report herein, for the first time, a case of unusually recalcitrant hyalohyphomycosis of the face due to Acremonium strictum successfully treated with topical 5% imiquimod in an immunocompetent patient, who had failed to respond to various antifungals, including itraconazole, and cryotherapy.

A prospective epidemiologic survey on the prevalence of onychomycosis and dermatophytosis in male boarding school residents.

Dermatophyte infections and onychomycosis are not usually serious in term of mortality; however, they may have significant clinical consequences such as secondary bacterial infections, chronicity, therapeutic difficulties and esthetic disfigurement in addition to serving as a reservoir of infection. Our aim was to determine the prevalence of onychomycosis and dermatophytosis in a selected high risk group, consisting of male boarding school residents. A total of 410 males inhabiting two houses were evaluated by two dermatologists. In cases of clinical suspicion, appropriate samples were taken for direct microscopy and culture. The results showed that the prevalences of tinea pedis (athlete's foot) and pure pedal onychomycosis were 51.5% (n:211) and 4.4% (n:18), respectively. Thirty cases of those with tinea pedis were complicated by toenail onychomycosis. Tinea cruris was present only in five cases with tinea pedis. Interestingly 71.1% of those with tinea pedis and 45.8% of those with onychomycosis, associated with or without tinea pedis were unaware of their diseases. The most common fungal isolate was Trichophyton rubrum (76.6%) followed by Epidermophyton floccosum (11.6%), T. interdigitale (10.55%). Approximately one third of the cultures from nail specimens yielded pure growths of nondermatophyte moulds or Candida albicans. In conclusion, we found unexpectedly high prevalences of occult athlete's foot and toenail onychomycosis among the male residents of student houses. Our results indicate that health-care workers of such common boarding-houses should be more aware of clinical and subclinical dermatophyte infections and onychomycosis, and have more active approaches to educational measures and management strategies to prevent further infections. To our knowledge, this is the first epidemiologic study on the prevalences of dermatophytosis and onychomycosis in boarding-houses from Turkey.

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.

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.

Severe monilethrix associated with intractable scalp pruritus, posterior subcapsular cataract, brachiocephaly, and distinct facial features: a new variant of monilethrix syndrome?

Monilethrix is a rare developmental hair shaft defect characterized by small elliptical node-like deformities with increased hair fragility resulting in partial or diffuse alopecia. The disorder is usually transmitted in an autosomal dominant fashion with incomplete penetrance and variable expressivity, but autosomal recessive inheritance has also been reported. It is thought to be without systemic involvement, whereas keratosis pilaris and follicular papules are almost invariably associated features. We describe an instance of monilethrix in a 9-year-old boy from consanguineous parents, characterized by universal dystrophic alopecia associated with intractable scalp pruritus, diffuse keratosis pilaris, and bilateral posterior subcapsular cataracts. His disease was further characterized by physical underdevelopment and distinct features of hypertelorism, a wide-based nose, long philtrum, relatively large mouth with thick lower lip, enlarged forehead, small, receding chin, short neck, and rounded (ultrabrachycranial) skull. The findings in our patient suggest that "monilethrix syndrome" is an appropriate term for defining the instances of monilethrix associated with other abnormalities. We conclude that our patient may represent a new and severe, autosomal recessive variant of monilethrix syndrome.

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.

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.