Therapeutic use of topical corticosteroids in the vesiculobullous lesions of incontinentia pigmenti.

Incontinentia pigmenti (IP) is a rare genodermatosis caused by a mutation of nuclear factor kappa B essential modulator gene. There is no specific treatment for IP, therefore it has been claimed that there is no effective treatment to hasten resolution of any of the phases of IP. However, the initial vesiculobullous stage of IP is characterized histopathologically by eosinophilic inflammation, which is expected to respond to corticosteroids. An 18-day-old female neonate was seen, with vesicles on her trunk and limbs diagnosed as the vesiculobullous stage of IP. The patient was treated with a double-compound cream containing a potent corticosteroid (difluocortolone valerate 0.1%) and an antiseptic (chlorquinaldol 1%), to be applied to the lesions twice daily. Five days later, resolution of the lesions was almost complete. As chlorquinaldol has no known anti-inflammatory activity, we attribute this improvement to difluocortolone valerate. This case shows that early lesions of IP with eosinophilic inflammation are treatable.

Eruptive vellus hair cysts: an effective extraction technique for treatment and diagnosis.

BACKGROUND: Eruptive vellus hair cysts are uncommon developmental anomalies of vellus hair follicles that are observed in young patients. Two patients were evaluated for asymptomatic flesh-coloured papules appearing on the chest, abdomen and axillae. Lesions of both patients were diagnosed histologically as eruptive vellus hair cyst, which is a disorder with no standard treatment option. OBJECTIVE: To introduce an alternative treatment and diagnostic technique for eruptive vellus hair cysts and to evaluate this technique's effectiveness histopathologically. MATERIALS AND METHODS: We treated the lesions by using cautery and a standard dissecting forceps under topical anaesthesia. After puncturing the overlying skin in the centre using the sharp-tipped cautery point, we grasped the base of the cyst using a standard dissecting forceps and we extracted the cysts out. In patient 1, extracted cysts were sent for histopathological examination; in patient 2, microscopic examination of the extracted material was performed in potassium hydroxide preparations. RESULTS: Over 150 cysts were extracted with this technique in two patients. The technique was simple, quick and tolerated well. No lesion recurrence was observed after 4 months. On histopathological examination, extracted cysts were observed to have cyst walls. In patient 2, the diagnosis was made within minutes by microscopic examination of the potassium hydroxide preparations of the extracted material. CONCLUSION: Our extraction technique is a simple and effective treatment option for these cysts. It can be regarded as curative since the cyst walls were also extracted. Microscopic examination of the potassium hydroxide preparation of the extracted cysts suggests that this technique can also be used as a simple diagnostic procedure.

Apolipoprotein E polymorphism and lipoprotein compositions in normolipidaemic xanthelasma patients.

BACKGROUND: Apolipoprotein E (apoE) phenotypes and lipoprotein compositions in xanthelasma patients have been reported in different series. OBJECTIVE: To investigate the apoE polymorphism and lipoprotein compositions in xanthelasma patients by using rapid polymerase chain reaction, and searched for an association between apoE polymorphism and the lipoprotein levels in xanthelasma patients. DESIGN: ApoE polymorphism and the different types of serum lipoproteins were studied in 25 patients with xanthelasma and compared with 27 normal subjects. RESULTS: All of patients were found to be normolipidaemic. The patients had significantly higher concentrations of total cholesterol and apolipoprotein B, and lower concentrations of apolipoprotein A. There was no difference in serum triglyceride, low-density lipoprotein (LDL) and high-density lipoprotein (HDL) cholesterol concentrations. The distribution of apoE genotypes and alleles was the same in both groups. CONCLUSIONS: The apoB, apoA and cholesterol levels did show statistically significant differences in the direction of an increased risk of atherosclerosis. Patients with xanthelasma demonstrated slight differentiations in the apoE polymorphism and metabolism of lipoproteins that require further clarifications.

Idiopathic guttate hypomelanosis: idiopathic or ultraviolet induced?

Idiopathic guttate hypomelanosis (IGH) is a common but often unrecognized skin disease with obscure etiopathogenesis. As the lesions mostly develop on the exposed parts of the extremities, ultraviolet has been hypothesized to induce these lesions. However, previous statistical studies did not confirm any association between ultraviolet and IGH. We describe a 72-year-old woman with mycosis fungoides in whom widespread IGH occurred during narrow band ultraviolet B (UVB) therapy. The lesion development in the trunk following narrow band UVB therapy suggests that these lesions may not be idiopathic and they may be associated with UV exposure although controversy exist in the literature. This case is particular as it reveals strong evidence on this relationship.

A simple open comedone extraction technique for Favre-Racouchot disease.

Favre-Racouchot disease (FRD) is a common disease characterized by solar elastosis and large open comedones and cysts. This disorder mainly affects elderly Caucasian men who have had a great deal of exposure to the sun. Patients having FRD continuously develop numerous large open comedones that could cause a cosmetic problem. We performed a simple and effective extraction technique for these open comedones using a standard dissecting forceps. This technique is well tolerated and no complications were observed. We suggest this comedone extraction technique as a simple alternative treatment for this common cosmetic problem.

Oxidant/antioxidant status in patients with recurrent aphthous stomatitis.

Recurrent aphthous stomatitis (RAS) is recognized as one of the most common oral mucosal diseases worldwide. The aim of this study was to determine the oxidant/antioxidant status in erythrocyte and plasma samples from patients with RAS in comparison with healthy controls. Twenty-two patients with RAS and 23 healthy controls were recruited. Superoxide dismutase, glutathione peroxidase (GSHPx) and catalase (CAT) activities, and malondialdehyde (MDA) and antioxidant potential (AOP) levels were measured in plasma and erythrocytes from patient with RAS and controls. We found decreased CAT and GSHPx activities and AOP levels in the erythrocytes, and decreased AOP and increased MDA plasma levels in patients with RAS in comparison with control subjects. In summary, this study demonstrated that enzymatic and nonenzymatic antioxidant defence systems are impaired in patients with RAS.

Neutrophilic dermatosis of the hands (localized Sweet's syndrome) in association with chronic hepatitis C and sarcoidosis.

We report a case of recurrent localized Sweet's syndrome (SS) with pulmonary sarcoidosis and hepatitis C virus infection. Hepatitis C may be the triggering factor for both Sweet's syndrome and sarcoidosis through stimulation of T helper 1 immune responses involved in the pathogenesis of both diseases.

Severe erythema nodosum due to Behçet's disease responsive to erythromycin.

A patient with severe erythema nodosum due to Behçet's disease is reported on here. Erythema nodosum lesions did not respond to classical treatments; however, they cleared after erythromycin treatment, which was prescribed for the treatment of coincidental erythrasma. Erythromycin treatment appears to be an effective treatment option in erythema nodosum. The hypothetical anti-inflammatory effects of erythromycin, besides its antibiotic properties, are reviewed and discussed to explain such a clinical improvement.

Uveitis associated with granuloma annulare.

PURPOSE: To report a case of uveitis associated with granuloma annulare (GA). GA is a benign, usually self-limited dermatosis of unknown origin, causing necrobiotic dermal and subcutaneous papules. So far, ocular involvement has not been reported in GA. METHODS: We describe a case of uveitis with GA. Ocular examination findings were consistent with uveitis. Histopathologic studies on lower extremity lesions revealed features consistent with GA. RESULTS: The patient was successfully treated with systemic and topical corticosteroids. After two months, however, the skin and eye lesions relapsed. When the same treatment was restarted, her lesions regressed within four weeks. CONCLUSIONS: This case provides an example of concomitant uveitis and GA. To our knowledge, this is the first report of this combination.

Surgical approach to benign small papular and dome-shaped melanocytic naevi on the face.

Patients frequently request removal of benign papular and dome-shaped naevi for cosmetic or functional reasons. Melanocytic naevi can be removed by elliptical, round, punch or shave excision or destroyed using electrodessication or cryotherapy. Total elliptical excision is probably the most widely used method of removal. If malignancy is suspected, adequate specimens for histological interpretation are required. When malignancy is not suspected, the cosmetic result becomes the first priority. Smaller incisions minimize tissue trauma and so give cosmetically superior results. Round excision has been recommended for the removal of moles but has not been widely practised. Round excision and punch excision may be better alternatives than conventional fusiform excision of benign dome-shaped or papular naevi of the face, as more tissue is preserved. Shave excision of naevi may be preferable to elliptical excision in sites where the incidence of hypertrophic scarring is high, as preservation of some thickness of the dermis may result in a more acceptable scar or even avoid a scar entirely. Expedient and simple surgery with excellent cosmetic results can be accomplished by the use of punches. Cryotherapy with cutting or curetting and electrodesiccation combined with shaving have been described. Round excision may be a better alternative to conventional fusiform or shave excision of benign papular or dome-shape nevus of the face because it leaves an almost imperceptible scar. In this technique, less skin is excised and histopathological examination can be done.

Association of class I HLA antigens with the clinical manifestations of Turkish patients with Behçet's disease.

Genetic factors appear to be important in the pathogenesis of Behçet's disease. Although it is known to be strongly associated with HLA-B 51, the association of HLA class I antigens with specific clinical findings of the disease has not been studied extensively and the few studies are conflicting. The aim of this study was to investigate the association of HLA class I alleles with the manifestations of Behçet's disease in Turkish patients. Eighty-five patients with Behçet's disease were typed for HLA-A, B, and C antigens with the serologic, standard microlymphocytotoxicity technique. Possible associations of the HLA complex with clinical findings of Behçet's disease were examined. Statistically significant findings are as follows (P < 0.05): increased HLA-B 51 and decreased HLA-B35 frequency in patients with thrombophlebitis, increased HLA-A29 and decreased HLA-Bw6 frequency in patients with ocular involvement, decreased HLA-Cw2 frequency in patients with erythema nodosum, and decreased HLA-Cw 7 frequency in patients with genital ulceration. Of particular note, the results of this study suggest that the presence of HLA-B 51 and the absence of HLA-B35 can be regarded as laboratory risk factors of venous thrombosis in patients with Behçet's disease.