Nail changes in diabetes.

Diabetes is a common condition that is increasing in incidence worldwide. Although the skin manifestations of this condition are well described, there is scant literature on the associated nail changes. In this review, we describe the various clinical features of nail changes associated with diabetes, which can be broadly divided into infections, vascular changes, neuropathic manifestations and miscellaneous changes, although there is overlap between them. There is no pathognomonic nail alteration, but it is important for clinicians to be aware of the potential nail manifestations in diabetes as they can facilitate investigations and thereby early diagnosis of diabetes, resulting in holistic management of the patient.

Inflammatory nail conditions. Part 1: nail changes in psoriasis.

Nail changes are visible in a variety of inflammatory dermatoses. The commonest dermatological condition with nail manifestations is chronic plaque psoriasis. This two-part article reviews the nail signs in psoriasis in Part 1, and the nail changes in cutaneous lichen planus and alopecia areata in Part 2. It provides a brief summary of the salient points in the clinical features, management and prognosis of these entities, with practical recommendations that may be beneficial to all dermatologists.

Case reports and narrative fallacies: the enigma of black swans in dermatology.

Narrative fallacy is a problematic heuristic that leads us to make inaccurate cause-effect relationships. They are a particular issue in case reports because of the limited scope of these papers, the perpetuated biases they proffer and the misperception of 'black swan' events. This article highlights the negative effects of these fallacies in dermatological practice through three case studies: the use of epinephrine with lignocaine at distal sites, the difference between once-daily and twice-daily application of topical steroids, and the effect of sterile gloves for skin surgery on infection rates. Awareness of the biases in case reports and the employment of metacognition may help us to avoid falling victim to narrative fallacies. Given the potential problems with this heuristic, Clinical and Experimental Dermatology (CED) utilizes case reports to further medical education and offer different clinical perspectives, rather than as a driver of medical knowledge.

Inflammatory nail conditions. Part 2: nail changes in lichen planus and alopecia areata.

Nail changes are frequently seen in patients with cutaneous lichen planus and alopecia areata. This manuscript provides an updated overview on the clinical features, management and prognosis of both conditions. Searches of electronic databases PubMed and EMBASE were conducted and eligible articles were accessed. Practical management principles relevant to these two conditions are also included.

Disseminated Mycobacterium simiae infection in a patient with adult-onset immunodeficiency due to anti-interferon-gamma antibodies - a case report.

BACKGROUND: Mycobacterial species other than Mycobacterium tuberculosis and Mycobacterium leprae are generally free-living organisms and Mycobacterium simiae is one of the slowest growing Non-tuberculous mycobacteria. This is the first case report of Mycobacterium simiae infection in Sri Lanka and only very few cases with extrapulmonary manifestation reported in the literature. CASE PRESENTATION: A 24-year-old, previously healthy Sri Lankan male presented with generalized lymphadenopathy with discharging sinuses, evening pyrexia, weight loss, poor appetite and splenomegaly. Lymph node biopsies showed sheets of macrophages packed with organisms in the absence of granulomata. Ziehl Neelsen, Wade Fite and Giemsa stains revealed numerous red coloured acid-fast bacilli within foamy histiocytes. Slit skin smear for leprosy was negative and tuberculosis, fungal and bacterial cultures of the lymph node and bone marrow did not reveal any growth. Later he developed watery diarrhea and colonoscopy revealed multiple small polyps and ulcers throughout the colon extending up to the ileum, Which was confirmed to be due to cytomegalovirus confirmed by PCR and successfully treated with ganciclovir. Positron emission tomography scan guided biopsies of the gut and lymph nodes confirmed presence of mycobacterial spindle cell pseudo-tumours and PCR assays revealed positive HSP65. The culture grew Mycobacterium Simiae. Flow cytometry analysis on patient's blood showed extremely low T and B cell counts and immunofixation revealed low immunoglobulin levels. His condition was later diagnosed as adult onset immunodeficiency due to anti- interferon - gamma autoantibodies. He was initially commenced on empirical anti-TB treatment with atypical mycobacterial coverage. He is currently on a combination of daily clarithromycin, ciprofloxacin, linezolid with monthly 2 g/kg/intravenous immunoglobulin to which, he had a remarkable clinical response with complete resolution of lymphadenopathy and healing of sinuses. CONCLUSIONS: This infection is considered to be restricted to certain geographic areas such as mainly Iran, Cuba, Israel and Arizona and this is the first case report from Sri lanka. Even though the infection is mostly seen in the elderly patients, our patient was only 24 years old. In the literature pulmonary involvement was common presentation, but in this case the patient had generalized lymphadenopathy and colonic involvement without pulmonary involvement.

Management of mucous membrane pemphigoid in a joint oral medicine-dermatology clinic.

BACKGROUND: Mucous membrane pemphigoid (MMP) comprises a group of immunobullous diseases involving the mucosa and skin. Potential sequelae include painful mucosal erosions, vision loss and laryngeal stenosis. AIM: To characterize the features of patients with MMP seen within an Oral Medicine setting, including clinical features, immunofluorescence results and response to treatment. METHODS: A retrospective case note analysis was undertaken. Treatment effect was divided into response and nonresponse using predetermined adjective terms. RESULTS: In total, 42 cases of MMP were identified (18 men, 24 women), mean age 65 years (range 36-85 years). Oral involvement was most common on the gingivae (n = 38; 90.5%) while the most common extraoral sites involved were ocular (n = 13; 31.0%) and skin (n = 12; 28.6%). Features of MMP were found in 21 of 34 (61.8%) of routine biopsies, 31 of 34 (91.2%) direct immunofluorescence samples and 8 of 25 (32.0%) indirect immunofluorescence samples. Topical corticosteroids provided effective symptom control in 9 of 42 cases (21.4%), while systemic therapy was used in 31 of 42 patients (73.8%). Dapsone was prescribed for 25 patients, of whom 18 (72.0%) responded. Mycophenolate mofetil was used in 13 cases and had a response rate of 46.2%. Overall, 27 of 42 patients (64.3%) achieved a response using a tolerable topical or systemic treatment. CONCLUSION: This series demonstrates that MMP has a female predominance and is a disease of older age, with a predilection for specific oral sites. Direct immunofluorescence has a high sensitivity in detecting features of MMP. Although some patients achieve adequate symptom control with topical corticosteroids, many require systemic therapy.

Novel compound heterozygous mutations in the desmoplakin gene cause hair shaft abnormalities and culminate in lethal cardiomyopathy.

A 2-month-old white girl born to nonconsanguineous parents presented to the dermatology department with hair loss that had commenced a few months after birth. Although her hair loss later stabilized, it remained sparse. By the age of 2 years, she was noted to have developed focal keratoderma over pressure points of the soles. Aged 5 years, she was admitted to hospital with a chest infection, and investigations at that point revealed that she had a dilated cardiomyopathy. Subsequent genetic investigations identified compound heterozygous mutations in the 3' end of the desmoplakin (DSP) gene (7567delAAGA and 6577G>A), explaining the cardiocutaneous phenotype.

Imiquimod use in the genital area and development of lichen sclerosus and lichen planus.

Imiquimod has immune-stimulant properties that can precipitate autoimmune conditions like eczema, psoriasis and lichenoid conditions. We report two cases here where imiquimod induced florid lichen sclerosus in one patient and lichen planus in another. In both patients the condition was so aggressive and unresponsive to steroid treatment that circumcision was necessary.

Behçet's disease.

Behçet's disease (BD) is a chronic, relapsing multisystem vasculitis with predominant involvement of the oral and genital mucosa. It has a worldwide distribution, but the prevalence is highest in Central Asia and the Far East (along the ancient 'Silk Route'). Genetic, environmental, immunological and haemostatic factors play a role in the aetiopathogenesis. The International Study Group for BD proposed criteria for the diagnosis of this condition, the essential feature being recurrent oral ulceration. Genital ulcers and skin manifestations are common, while ocular changes are the most important cause of morbidity. Almost any organ in the body can be involved, and systemic involvement may portend a poorer prognosis. There is no pathognomonic test for BD and the diagnosis is made on clinical findings. Treatment of BD would require multidisciplinary cooperation, and early referral to an ophthalmologist is advisable to prevent ocular morbidity. Topical and systemic agents (colchicine, dapsone and thalidomide) are useful in controlling exacerbation of the oral and genital ulcers. Severe disease may require immunosuppressive agents and, more recently, biological agents have been used successfully. It tends to follow an unpredictable course, and the eventual prognosis depends on the systemic involvement.

Lichen sclerosus.

Lichen sclerosus (LS) is a chronic inflammatory disorder of the skin and mucosa, presenting to genitourinary physicians and dermatologists. It affects both sexes and all age groups. Although the exact aetiology is uncertain, genetic predisposition, infections and autoimmune factors have been implicated in its pathogenesis. Symptoms include pruritus and soreness, but asymptomatic presentations are not uncommon. The classical clinical picture is of atrophic white plaques in the anogenital region. Histopathology is specific with basal cell degeneration, upper dermal oedema, homogenization of collagen and a chronic inflammatory infiltrate. Short courses of potent topical corticosteroids form the mainstay of treatment. The condition tends to be remitting and relapsing, with spontaneous regressions reported in a few. In men, the term balanitis xerotica obliterans is sometimes used to describe late and severe LS of the penis. Scarring and progression to squamous cell carcinomas can occur in chronic LS, resulting in significant morbidity. A multidisciplinary approach to care and the need for long-term monitoring cannot be overemphasized.