Should we consider hypohidrotic ectodermal dysplasia as a possible risk factor for malignant melanoma?

BACKGROUND: Hypohidrotic ectodermal dysplasia (HED) is a rare multisystem disorder that typically affects hair, teeth, nails, and sweat glands in combination with abnormalities of ectodermal originated tissues. OBJECTIVE: To raise the issue of whether heightened awareness is required in the follow-up of pigmented lesions in patients with HED. METHODS/RESULTS: Two patients with HED are reported. The first had a history of two malignant melanomas, whereas the second presented with a novel malignant melanoma. CONCLUSION: Although a prospective epidemiologic study to determine the incidence of malignant melanoma in patients with HED is virtually impossible and the probable pathogenesis warrants further investigation, these two case reports, in conjunction with other recent reports associating other ectodermal dysplasias with malignant melanoma, suggest that heightened awareness might be prudent in the follow-up of pigmented lesions in patients with HED.     

Shall we continue with nerve trunk decompression in leprosy?

There is a tendency to compare the results of surgery with that of oral corticosteroid therapy in leprous neuritis as if the two are competing methods. Surgery helps by removing the external compressive force and improves circulation so that steroids can reach and effectively act at the site of inflammation, minimizing the ischaemic and compression damage to nerve fibres. Often nerve decompression in leprosy is requested rather late so that the desired results are not always achieved. With emphasis on "elimination of leprosy", the disease is being managed in endemic states by field programmes where individual patient is not the priority unlike in the general hospitals and among practitioners where the welfare of the patient is the priority. It is therefore important to create awareness about compression neuropathy in leprosy and the need for combination therapy so as to bring down the morbidity and disability.     

Bilateral lagophthalmos in leprosy: is it a rare phenomenon?

Lagophthalmos is one of the well known complications of leprosy due to involvement of the facial nerve. Herein, we report three cases of bilateral lagophthalmos due to leprosy which presented to us within a span of just three months. In all these cases, lagophthalmos was not the presenting complaint and it was detected by the treating doctor during examination. This report is being presented to highlight the importance of cranial nerve examination in all cases of leprosy as at times early changes of lagophthalmos may go unnoticed by the patient.     

Pruritus in pregnancy and childhood--do we really consider all relevant differential diagnoses?

During pregnancy and also during childhood, pruritus can have manifold aetiologies and should therefore always be taken seriously. In pregnancy, pruritus is the main dermatological symptom, occurring in 18% of women. Pregnancy-specific dermatological diseases such as polymorphic eruption of pregnancy (PEP), Pemphigoid (Herpes) gestationis, Pruritus gravidarum are accompanied by severe pruritus and scratching. In children, it mainly occurs along with dermatoses but in rare cases with systemic diseases such as renal or liver failure. Mostly, it appears in the setting of atopic dermatitis (AD). Both groups of patients require therapeutic regimens of their own. The use of topical and systemic treatments depends on the underlying aetiology of the pruritus and the stage and status of the skin. Because of potential effects on the fetus, the treatment of pruritus in pregnancy requires prudent consideration of whether the severity of the underlying disease warrants treatment and selection of the safest treatments available. Systemic treatments such as systemic glucocorticosteroids, a restricted number of antihistamines and ultraviolet phototherapy may be necessary in severe and generalized forms of pruritus in pregnancy. In children, the physician has to consider that topically applied drugs may cause intoxication due to the different body volume/body surface proportion. The dosages of systemic drugs need to be adapted in children and ultraviolet phototherapy should be performed with caution due to possible longterm photo damage of the skin. In a two center approach, we wanted to highlight the major aetiologies of pruritus during pregnancy and in children and point out the mainstays of antipruritic therapy in these two challenging groups of patients according to our clinical experiences. For the future, it would be desirable for all disciplines involved (dermatologist, gynaecologist, paediatrician, general practitioner) to cooperate closely to expand the clinical and scientific knowledge of pruritus in these groups of pruritus patients.     

Proteomics for rejection diagnosis in renal transplant patients: Where are we now?

Rejection is one of the key factors that determine the long-term allograft function and survival in renal transplant patients. Reliable and timely diagnosis is important to treat rejection as early as possible. Allograft biopsies are not suitable for continuous monitoring of rejection. Thus, there is an unmet need for non-invasive methods to diagnose acute and chronic rejection. Proteomics in urine and blood samples has been explored for this purpose in 29 studies conducted since 2003. This review describes the different proteomic approaches and summarizes the results from the studies that examined proteomics for the rejection diagnoses. The potential limitations and open questions in establishing proteomic markers for rejection are discussed, including ongoing trials and future challenges to this topic.     

Rudimentary meningocele: remnant of a neural tube defect?

BACKGROUND: Rudimentary meningocele, a malformation in which meningothelial elements are present in the skin and subcutaneous tissue, has been described in the past under a variety of different terms and has also been referred to as cutaneous meningioma. There has been debate as to whether rudimentary meningocele is an atretic form of meningocele or results from growth of meningeal cells displaced along cutaneous nerves OBJECTIVE: We reviewed the clinical, histological, and immunohistochemical characteristics of rudimentary meningocele in an attempt to assess the most likely pathologic mechanism for it. DESIGN: Retrospective study. SETTING: University hospitals. PATIENTS: Thirteen children with rudimentary meningocele. MAIN OUTCOME MEASURES: Medical records were reviewed and histopathologic examination as well as immunohistochemistry studies were performed for each case. A panel of immunoperoxidase reagents (EMA, CD31, CD34, CD57, S-100, and CAM 5.2) was used to assess lineage and to confirm the meningothelial nature of these lesions. RESULTS: Recent evidence indicating a multisite closure of the neural tube in humans suggests that classic meningocele and rudimentary meningocele are on a continuous spectrum. CONCLUSION: Rudimentary meningocele seems to be a remnant of a neural tube defect in which abnormal attachment of the developing neural tube to skin (comparable to that in classic meningocele) could explain the presence of ectopic meningeal tissue. In the majority of cases, no underlying bony defect or communication to the meninges could be detected. However, in light of the probable pathogenesis, imaging studies to exclude any communication to the central nervous system should precede any invasive evaluation or intervention.     

Depression in patients with pemphigus: Is it a major concern?

It is suggested that some dermatological diseases due to their chronicity, impact on the body image, unlikelihood of complete recovery and frequent recurrences are one of the major predisposing factors towards depression. Therefore, we aimed to evaluate the rate and level of depression among pemphigus vulgaris and pemphigus foliaceus patients, two of the most common causes of hospitalization in dermatology units. This research was conducted on 55 patients with active pemphigus vulgaris and pemphigus foliaceus referring to pemphigus clinics or admitted as inpatients to the dermatology ward of Qaem and Imam Reza hospitals, Mashhad, Iran, from April 2008 to September 2009. The research tool was the Beck Depression Inventory. Collected data was analyzed by χ²‐test Student's t‐test. Twenty‐six (47.3%) patients were female and 29 (52.7%) were male. The mean age was 42.34 ± 18.98 years. The prevalence rate of clinical depression was 28% in pemphigus vulgaris and 20% in pemphigus foliaceus cases. Depression prevalence showed no significant difference between these two groups (P = 0.873). In conclusion, pemphigus patients are at risk for mild depression.     

Netherton’s syndrome and lepromatous leprosy: a mere coincidence?

Objectives Netherton’s syndrome (NS) is a rare autosomal recessive condition, first described in 1958, which involves a complex immunological dysfunction, ichthyosiform dermatitis, and erythroderma, characteristic defects of the hair shaft and atopy. Recurrent bacterial infection in the skin of patients with NS is frequent. Methods This paper represents the first case report of leprosy and concurrent NS. Discussion This case merits discussion among doctors in endemic and non‐endemic areas to evaluate the chronic use of systemic corticosteroids as a risk factor for leprosy. The present patient came from an endemic area of leprosy and was treated chronically with systemic corticosteroids for erythroderma. This treatment, along with the immunodeficiency related to the syndrome and caused by a genetic mutation in SPINK5, may be a facilitating factor for the infection.