Etoricoxib challenge in patients with chronic urticaria with NSAID intolerance

Patients with chronic urticaria frequently experience flares of hives following the ingestion of chemically unrelated nonsteroidal anti-inflammatory drugs (NSAIDs). The pathogenic mechanism of these reactions is based on cyclooxygenase-1 (COX-1) enzyme inhibition. In most cases, nonselective NSAIDs, which inhibit both COX-1 and COX-2, are responsible for such adverse reactions; in contrast, analgesic and anti-inflammatory drugs exerting limited inhibition on COX-1 are generally better tolerated by these patients. This study aimed to detect tolerability of etoricoxib, a selective COX-2-inhibiting drug, in patients with chronic urticaria with a history of NSAID intolerance. Single-blind, placebo-controlled oral challenges with increasing doses of etoricoxib were carried out in 17 adult patients with chronic urticaria exacerbated by NSAID. All patients tolerated the drug at therapeutic doses. The study suggests that etoricoxib, with its favourable COX-1/COX-2 ratio, is well tolerated by patients with chronic urticaria exacerbated by NSAID intolerance.     

Extensive Nevus Comedonicus with Inflammatory Nodules and Cysts Controlled with Adalimumab

Nevus comedonicus is a very rare skin disorder characterized by the presence of comedo-like dilated pores with keratinous plugs, rarely resulting in painful recurrent inflammatory nodules or cysts. It presents as localized or extensive form. It displays unilaterally or bilaterally segmental distribution. Histopathologically, it is characterized by keratin-filled epidermal invagination with bulbous proliferation of keratinocytes. The condition may be caused by fibroblast growth factor receptor 2 mutation. Although it may be controlled by a variety of therapeutic modalities, it is difficult to achieve complete resolution. We report a case of extensive nevus comedonicus with inflammatory nodules and cysts controlled with adalimumab.     

Factors associated with patient satisfaction with care among dermatological outpatients

BACKGROUND: It has been shown that poor patient satisfaction can lead to poor adherence to treatment with consequently poor health outcomes. In order to improve the quality of care perceived by the patient and thus the health outcome, it is important to understand which are the main factors influencing patient satisfaction. OBJECTIVES: To examine factors associated with patient satisfaction with care among dermatological out-patients. METHODS: This longitudinal study is based on a sample of dermatology out-patients. The independent effects on patient's satisfaction of patient characteristics (sociodemographic characteristics, disease severity, quality of life) and of specific aspects of provided health care (the time the physician spent with patients, physician's interpersonal skills, etc.) were examined by multiple logistic regression. RESULTS: A total of 1389 out-patients were selected at random and invited to participate. Of the 722 patients who agreed to participate, 424 fulfilled the inclusion criteria and 396 of these patients (93.4%) completed the study. Overall satisfaction was reported by 60.0% of patients. The likelihood of overall satisfaction was found to be significantly and independently increased by the physician's ability to give explanations and to show empathy for the patient's condition, and by the older age of patients. The likelihood of satisfaction also increased with increasing disease severity, but decreased with symptom-related poor quality of life. The lowest level of satisfaction was found among patients whose symptom-related quality of life was worse than the clinical severity rated by the dermatologist. CONCLUSIONS: Improving the physician's interpersonal skills can increase patient satisfaction, which is likely to have a positive effect on treatment adherence and health outcomes. Dermatologists succeeded better in establishing a good relationship with clinically more severely affected patients than with patients who were clinically mildly affected despite their quality of life being impaired. Thus, the inclusion of a patient-rated quality of life can be a useful measure in dermatology, as it enables clinicians to perceive the patients' perception of their health status.     

Knemometry in Children with Atopic Dermatitis Treated with Topical Glucocorticoids

Abstract: Recently the knemometer, a lower leg length measuring device, has been introduced for sensitive assessment of systemic activity of exogeneous glucocorticoids in children. The aim of this study was to assess by means of knemometry whether the topical glucocorticoid budesonide affects short-term growth in children with atopic dermatitis. Fourteen children 5 to 12 years old were studied in an open longitudinal trial with three periods of 2 weeks duration. In periods 1 (run-in) and 3 (run-out), the children were treated with emollient. In period 2, budesonide cream 0.025% was followed by emollient twice daily to all of the body except the face. Eczema was evaluated according to a score based on extent and activity. Knemometry was performed twice weekly. Compared to the run-in and run-out periods the mean growth rate during budesonide treatment was reduced by 0.11 mm/wk (p > .05) and 0.40 mm/wk (p < .05), respectively. The mean growth rate during run-out was increased by 0.29 mm/wk as compared to run-in (p < .05). Compared to run-in the mean severity indices during budesonide treatment and runout were reduced by 1.55 (p < .05) and 1.55 points (p <.05), respectively. The concomittant variations in lower leg growth rate and disease activity suggest that short-term treatment with topical glucocorticoids may provide a better growth potential during the weeks after withdrawal of the treatment. Whether this is due to improved disease control needs further study. Being a noninvasive method, knemometry may be useful for comparing different topical glucocorticoids and administration regimens in children in whom vasoconstrictor assays are difficult.     

Treatment of angiolymphoid hyperplasia with eosinophilia with the carbon dioxide laser

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon, idiopathic condition that presents with isolated or grouped plaques or nodules in the periauricular region, forehead, or scalp. ALHE is marked by a proliferation of blood vessels with distinctive large endothelial cells accompanied by a characteristic inflammatory infiltrate that includes eosinophils. The lesion is benign but may be persistent and difficult to eradicate. Various therapeutic modalities that have been tried for its treatment include intralesional and oral corticosteroids, cryotherapy, oral retinoids, vinblastine, surgical excision, laser therapy, and INFalpha2a. We report two cases with this rare condition: one patient, treated with cryotherapy, did not improve, while the second patient was successfully treated with the CO(2) laser.     

Characteristic features of Japanese women's hair with aging and with progressing hair loss

BACKGROUND: There have been few studies of the features of hair with aging and hair loss in Japanese women. OBJECTIVE: Features of Japanese women's hair with aging and with progressing hair loss were investigated. METHODS: Japanese women with hair loss (n=46) or with no or less hair loss (n=113), aged 14-68 years, were studied. Severity of hair loss was rated by visual comparison with six standard photographs. Hair density, hair growth rate, and hair diameter were analyzed by phototrichogram. Follicular units were deduced by a non-invasive method using tree-view analysis on scalp imaging. RESULTS: Hair loss in Japanese women is commonly characterized by a diffuse central pattern occurring after approximately 40 years of age. Hair density declines with age after the 40s. The reduction resulted from an increase in the number of one-haired follicular units and a reduction of three- and more-haired follicular units. Both the ratio and the growth rate of anagen hair also declined with age after the 40s. Mean hair diameter and the ratio of thick hairs increased with age from about 10 to 40 years, and decreased with progressing hair loss. There were few vellus-like hairs in women with hair loss, in comparison with male-pattern baldness. CONCLUSION: In Japanese middle-aged women, hair density declined with age without the appearance of hair loss. Hair loss appeared after approximately 40 years of age. The major causes might be reduction of hair density and the ratio of thick hairs, but not an increase of vellus-like hairs.     

Congenital Insensitivity to Pain with Anhidrosis Presenting with Palmoplantar Keratoderma

Abstract: Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal‐recessive disease caused by mutations in the NTRK1 gene. The disease is characterized by insensitivity to pain and absence of thermal perception. Herein a 6‐year‐old boy is presented with a large ulcer on the sole of his right foot and a thick, hyperkeratotic appearance of his palms and soles; there was also a medical history of hyperthermia, anhidrosis, recurrent bone fractures, osteomyelitis, injuries, mental retardation, dry and exfoliative skin, insensitivity to pain, and lack of thermal sensation. Genetic studies revealed a homozygote mutation in the NTRK1 gene. Although the patient initially presented with palmoplantar keratoderma, genetic studies confirmed the diagnosis of CIPA.     

Multifocal haemangioma with extracutaneous involvement associated with hypergalactosaemia

Neonatal haemangiomatosis, characterized by multiple haemangiomas, is a rare disease that develops during the neonatal period with or without visceral involvement. We report a 1‐month‐old Japanese boy with multifocal haemangiomas with extracutaneous involvement. A haemangioma on his left lower eyelid, present at birth, increased in size during the first postnatal month and more lesions developed during the same period. Neonatal mass screening showed hypergalactosaemia. Laboratory investigations found raised total bile acid and ammonia. Computed tomography and abdominal ultrasonography studies showed multiple hepatic haemangiomas and intrahepatic portovenous shunts. The child’s cutaneous and hepatic haemangiomas disappeared spontaneously with normalization of laboratory data, and galactose accumulation improved with the feeding of lactose‐free milk. There were no complications and the child has had no recurrence of the symptoms. Our case implies a possible association of multiple haemangioma and hypergalactosaemia, suggesting the necessity for visceral investigation.     

Dermatomyositis treated with high-dose intravenous immunoglobulins and associated with panniculitis

Polymyositis and dermatomyositis are idiopathic inflammatory myopathies characterized by subacute symmetrical weakness of proximal limb and trunk muscles. Dermatomyositis is distinguished from polymyositis by the presence of rash.^1,2 We describe an adult patient with treatment-resistant childhood-type dermatomyositis who made a good response to high dose intravenous immunoglobulins. Additionally, there was evidence of panniculitis which is an unusual histopathological finding in dermatomyositis.     

Impetigo herpetiformis with hyperparathyroidism

Impetigo herpetiformis, first described by Hebra, is a rare pustular disorder that primarily affects pregnant women and it is often complicated by an increased risk of spontaneous abortion. A commonly associated hypocalcemia often appears with hypoparathyroidism. Here we report a case of complicated impetigo herpetiformis without hypocalcemia paired with a compensatory hyperparathyroidism.     

Neurologic Abnormalities in Two Patients with Facial Hemiatrophy and Sclerosis Coexisting with Morphea

Abstract: Progressive facial hemiatrophy or Parry-Romberg syndrome is a rare entity characterized by unilateral atrophy of the skin, subcutaneous tissue, and the underlying bony structures. This syndrome has many features of linear scleroderma en coup de sabre but is distinguished by more extensive involvement of the lower face and by only slight cutaneous sclerosis. We describe two unusual children with both atrophic and sclerotic changes of half of the face coexisting with multiple plaques of typical morphea. Both children developed neurologic disturbances with cranial magnetic resonance imaging (MRI) abnormalities 2 years and 15 years, respectively, after the onset of cutaneous lesions. Thus considering that it may not be possible to correlate impairment in neurologic function and cutaneous disease, as illustrated by our patients, we emphasize the importance of an accurate follow-up.     

Hailey-Hailey disease with dissemination and eczema herpeticatum in therapy with etretinate

We report a 39-year-old patient with Hailey-Hailey disease localized mainly in the genito-femoral and scrotal areas. During hospital treatment the condition worsened due to superinfection with Candida albicans and gram-negative bacteria. Treatment with systemic steroids followed by aromatic retinoids was tried: eczema herpeticum developed in the affected areas, and dissemination of Hailey-Hailey disease ultimately occurred with papulo-vesicular and histologically characteristic lesions.     

Acrokeratosis paraneoplastica with bullous lesions associated with esophageal squamous cell carcinoma

Acrokeratosis paraneoplastica (Bazex syndrome) is characterized by an acral eruption with a psoriasiform appearance, which usually presents simultaneously with an underlying neoplasm. We describe the case of a 64-year-old male who presented with a two-month history of pruritic, flaky, erythematous lesions on the palms, backs of the fingers and toes and pinnae, accompanied by bullous lesions. The patient also reported progressive dysphagia in the last six months, and general wasting with a loss of 15 kg. The digestive endoscopy revealed a squamous cell carcinoma in the proximal esophagus. The histopathological study of the bullous lesions showed the presence of a subepidermal bulla, and direct immunofluorescence revealed granular deposits of IgG, IgA and C3 in the basal membrane of the healthy perilesional skin. These clinical/pathological findings support the existence of an immunological pathogenic mechanism related to this entity.     

Hand-foot syndrome with sclerodactyly-like changes in a patient treated with capecitabine

Capecitabine, a fluoropyrimidine carbamate with antineoplastic activity, is an oral agent that was developed as a prodrug of 5-fluorouracil and is used in the treatment of metastatic colorectal and breast cancers. Multiple cutaneous adverse effects had been described with the use of this drug, but to our knowledge, specific association with capecitabine and hand-foot syndrome with sclerodactyly-like changes has been described only once. We report a patient with a colon adenocarcinoma who presented with hand-foot syndrome, with sclerodactyly-like changes induced by capecitabine.     

Bowenoid papulosis in a patient with AIDS treated with imiquimod: case report

A 53-year-old male patient with acquired immunodeficiency syndrome (AIDS) was treated with topical immunomodulator imiquimod for bowenoid papular. Clinically the lesions presented as condilomatous and papulous changes with color varying from skin color to grayish. The lesions were located in the glans and in the dorsum of the penis. Clinical diagnosis was confirmed by histopathological examination, and the polymerase chain reaction (PCR) demonstrated the presence of human papilloma virus (HPV) 16. It was decided to apply a topical treatment with imiquimod 5% cream three times a week for 16 weeks. Almost complete regression was obtained; the residual lesions were treated with a combined chemical cauterization by using 50% trichloroacetic acid followed by 25% podophylin. Although it is not a definitive treatment, the use of topical immunomodulator is one more therapeutic option in the selected HPV cases.     

Acrodermatitis enteropathica with anorexia nervosa

Acrodermatitis enteropathica is a rare hereditary or acquired disorder of hypozincemia. It is characterized by acral and periorificial dermatitis, alopecia, diarrhea and growth retardation. Anorexia nervosa is characterized by low body weight, body image distortion with an obsessive fear and is also associated with various cutaneous findings including acrodermatitis enteropathica. We report a 37‐year‐old female with acrodermatitis enteropathica showing acquired zinc deficiency with anorexia nervosa.     

Axillary hyperhidrosis. Local treatment with aluminium-chloride hexahydrate 25% in absolute ethanol with and without supplementary treatment with triethanolamine

In a randomized, double-blind, half-sided experiment, 30 volunteers were treated in both armpits with aluminium chloride hexahydrate 25% in ethanol. In order to neutralize pH and thus reduce the skin irritation, post-treatment was performed in one armpit with triethanolamine 50% in ethanol. The sweat production was measured after physical labour by means of a combined colorimetric/gravimetric method. The combined treatment with aluminium chloride hexahydrate and triethanolamine was found to be statistically significantly (p less than 0.01) less irritating to the skin, but also statistically significantly (p less than 0.01) less effective than treatment with aluminium chloride hexahydrate alone. However, the reduction in the effect of the treatment was not of a sufficient extent as to be noticed by the volunteers themselves.     

Mutilating keratoderma with deaf-mutism

A 30 year old woman presented with typical lesions of mutilating keratoderma. The patient was deaf and dumb. Hyperkeratosis of palms and soles was present since infancy. Constriction of digits started by the age of 5 years. The clinical diagnosis was supported by histopathological examination.     

Multicentric reticulohistiocytosis with pulmonary involvement

Multicentric reticulohistiocytosis (MRH) is a rare and possibly devastating systemic disorder characterized by tissue infiltration by histiocytes and multinucleated giant cells. The disease commonly involves the skin, joints, and mucous membranes, with the rare involvement of other organ systems. We describe a patient with MRH presenting with papules and nodules on both hands and a rapidly progressive arthritis who may have had pulmonary involvement of the disease.     

Metabolic syndrome associated with toenail onychomycosis in Taiwanese with diabetes mellitus

Background Onychomycosis is a complication of diabetes mellitus (DM), which has a deleterious impact on the quality of life. Aim To explore the prevalence of onychomycosis amongst Taiwanese diabetics, and to analyze the factors associated with onychomycosis after adjusting for age and sex. Methods A total of 1245 Taiwanese diabetics were enrolled, and a nested case–control study was performed by onychomycosis outcome and the exposures were compared. Results The overall prevalence of onychomycosis among DM patients was 30.76% (383/1245), with a significantly higher prevalence in men than in women (P = 0.024). The factors associated with onychomycosis in matched pairs by gender and age were analyzed in 375 pairs. It was found that metabolic syndrome, obesity, triglyceride (TG) levels, and glycosylated hemoglobin (HbA1c) were associated with onychomycosis (P < 0.05). Conclusion Higher prevalence rates of onychomycosis were found in men and older DM patients. Metabolic syndrome, obesity, high TG levels, and poor glycemic control were associated with onychomycosis.