Full article available at: http://www.ncbi.nlm.nih.gov/pubmed/23138121?dopt=Abstract
Introduction: Though oral aphthosis is common, it has a significant impact on the quality of life in the patients. It is the most common oral ulcerative condition encountered in clinical practice. This study describes the characteristics and patterns of oral aphthosis seen at a tertiary dermatological centre in Singapore, with emphasis in evaluating the management gaps and in identifying underlying systemic diseases and nutritional deficiencies. Materials and Methods: This is a retrospective review of medical records over a 10-year period between June 2000 and June 2010. Two hundred and thirteen patients were identified using the search terms ‘oral ulcers’, ‘aphthous ulcers’, ‘oral aphthosis’, and ‘Behcet’s disease’. Patients with Behcet’s disease without oral ulcers and other diagnoses such as pemphigus vulgaris, lichen planus and herpes simplex were
excluded. The remaining patients were evaluated with regard to demographic characteristics, characteristics of oral ulcers, associated connective tissue disorders and nutritional deficiencies, diagnostic tests results, treatment response as well as follow-up duration. Results: One hundred and seventy-five patients were included in this study. One hundred and one patients had recurrent oral aphthosis, with 77 having simple aphthosis and 24 having complex aphthosis. Fourteen patients (8%) fulfilled the International Study Criteria (ISG) for Behcet’s disease, of which, 85.71% had complex aphthosis. The therapeutic ladder for such patients ranged from topical steroids and colchicine through to oral corticosteroids and/or dapsone therapy. Conclusion: Recurrent oral aphthosis is a niche condition in which dermatologists are well-poised to manage. This study demonstrates that a more definitive management and therapeutic algorithm for oral aphthosis are needed for better management patients in the future. In particular, complex aphthosis needs to be monitored for progression onto Behcet’s disease.
Full article available at: http://www.ncbi.nlm.nih.gov/pubmed/23122017?dopt=Abstract
Full article available at: http://www.ncbi.nlm.nih.gov/pubmed/23106586?dopt=Abstract
Rosacea is a common dermatosis that can involve the bald area of the scalp. We report the case of a man presenting clinical symptoms of rosacea of the forehead and the scalp, but with a histological picture of familial chronic benign pemphigus.
PATIENTS AND METHODS:
A 47-year-old man with a history of Hailey-Hailey disease had been presenting facial dermatosis for 5 years. The clinical features were erythema with pustules and scales located on the mid-forehead and the androgenic bald area of the frontal scalp. The histological aspect of the skin biopsy showed suprabasilar clefting and ancantholysis at all levels of the epidermis and sparse perivascular infiltrate. Direct immunofluorescence was negative. These findings were typical of Hailey-Hailey disease. Based on clinical findings, and without taking account of the skin biopsy results, treatment with doxycycline and a topical antifungal was administered for 3 months, leading to remission of symptoms.
The site of rosacea on the bald area of the scalp in males is described in the literature, and when present, is probably enhanced by exposure to UV radiation. In this patient, the histological features were interpreted as histopathologically equivalent to Köbner phenomenon.
full article available at: http://www.ncbi.nlm.nih.gov/pubmed/23122375?dopt=Abstract
The present report details the clinical and immunological features of a 53 year old Asian female who developed symptoms and signs of oesophageal PV during therapy with azathioprine and decreasing prednisolone dosage. Oesophageal involvement occurred during stable oral disease.
Oesophageal involvement can occur without significant oro-cutaneous lesions and immunological evidence of PV. This suggests that immunological targets for oesophageal disease may differ from those of other mucocutaneous areas, and that conventional first-line systemic therapy may not be effective for oesophageal lesions.
Full article available at: http://www.ingentaconnect.com/content/ubpl/wlmj/2012/00000004/00000002/art00001
Inherent to some immunobullous disorders is potential for intraepidermal or dermal–epidermal junction fragility, a phenomenon that may compromise biopsy specimen integrity and direct immunofluorescence (DIF) interpretation. In these situations, cutaneous adnexal structures (e.g. hair follicles, sweat apparatus) usually remain intact. Whether periadnexal DIF findings are reliable in diagnosing immunobullous conditions is unknown.
We evaluated 56 cutaneous specimens with diagnostic immunoglobulin (Ig) deposition patterns that contained adnexal structures. In a corollary study, we examined 145 hematoxylin-eosin-stained frozen specimens to determine biopsy factors associated with the presence of adnexal structures.
Periadnexal DIF findings offered diagnostic sensitivity in conditions with linear or cell-surface Ig deposition or lupus band. Periadnexal DIF findings were unreliable in dermatitis herpetiformis. Biopsy specimens from scalp and genitalia were most likely to contain folliculosebaceous units and sweat duct apparatus, respectively. Relative depth of biopsy correlated directly with the likelihood of identifying sweat duct apparatus but not folliculosebaceous units.
Periadnexal DIF findings may add diagnostic sensitivity in DIF evaluation of pemphigoid, pemphigus and lupus erythematosus. Pathologists can guide clinicians to biopsy certain anatomic sites and to obtain sufficient biopsy depth to increase the probability of capturing adnexal structures and, therefore, diagnostic yield from DIF specimens.
Aim. To characterize the clinical and immunological profile of patients with PF or PV with umbilical involvement.
Methods. In total, 10 patients (7 women, 3 men; age range 24–70 years, disease duration 3–16 years) diagnosed with either PV (n = 5) or mucocutaneous PF (n = 5) were assessed according to their clinical features, histopathology and immunological findings .
Results. Erythema, erosions, crusts and vegetating skin lesions were the main clinical features of the umbilical region. DIF of the umbilical region gave positive results for intercellular epidermal IgG and C3 deposits in eight patients and for IgG alone in the other two. Indirect immunofluorescence with IgG conjugate showing the typical pemphigus pattern was positive in all 10 patients, with titres varying from 1 : 160 to 1 : 2560. ELISA with recombinant Dsg1 gave scores of 24–266 in PF and 0–270 in PV. Reactivity to recombinant Dsg3 was positive in all five patients with PV (ELISA 22–98) and was negative in all PF sera.
Conclusions. All 10 patients with pemphigus with umbilical presentation had the clinical and immunopathological features of either PF or PV. This peculiar presentation, not yet completely elucidated, has rarely been reported in the literature. A possible explanation for this unique presentation may be the presence of either novel epitopes or an association with embryonic or scar tissue located in the umbilical-cord region.
Full article available at: http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2230.2012.04468.x/abstract
Full article available at: http://www.ncbi.nlm.nih.gov/pubmed/23073990?dopt=Abstract