Irma Korom

Necrolytic migratory erythema

Background:  Necrolytic migratory erythema is considered to be a paraneoplastic dermatosis. The classical symptoms are associated with α‐cell pancreatic islet cell tumor or ‘glucagonoma’. Generally, extracutaneous hallmarks of this disease include weight loss, diabetes, anaemia and diarrhoea.

Neglected Basal cell carcinomas in the 21st century.

Although tumors on the surface of the skin are considered to be easily recognizable, neglected advanced skin neoplasms are encountered even in the 21st century. There can be numerous causes of the delay in the diagnosis: fear of the diagnosis and the treatment, becoming accustomed to a slowly growing tumor, old age, a low social milieu, and an inadequate hygienic culture are among the factors leading some people not to seek medical advice. The treatment of such advanced neoplasms is usually challenging. The therapy of neglected cases demands an individual multidisciplinary approach and teamwork. Basal cell carcinoma (BCC), the most common cutaneous tumor, usually develops in the elderly, grows slowly, and has an extremely low metastatic potential; these factors are suggesting that BCCs might well be the “ideal candidates” for neglected tumors. Five neglected advanced cases of BCC were diagnosed in our dermatological institute between 2000 and 2009. The clinical characteristics and treatment modalities of these neoplasms are discussed, together with the possible causes of the neglect.

CYCLOSPORIN FOR DYSTROPHIC EPIDERMOLYSIS BULLOSA

A 65-year-old woman with epidermolysis bullosa since childhood was first admitted to our clinic in September, 1987. At first we treated the patient with high doses of vitamin E, phenytoin, prednisolone, and erythromycin but without sucess. When we read a report of the use of cyclosporin in a patient with epidermolysis bullosa acquisita, we began treatment with the above drug combination plus cyclosporin 1 mg/kg daily. Soon after the interruption of cyclosporin therapy, the blisters and erosions reappeared again. Cyclosporin was reintroduced and significant improvement was noted one month later

Collagen XVII/BP180 protein expression in squamous cell carcinoma of the skin detected with novel monoclonal antibodies in archived tissues using tissue microarrays and digital microscopy

Collagen XVII/BP180, a hemidesmosomal adhesion protein, is lost during normal keratinocyte maturation; however, it may be reexpressed upon malignant transformation. In this work, highly sensitive monoclonal antibodies 6D1 and 9G2 were produced, characterized, and used for the detection of collagen XVII in a tissue microarray series of archived samples of nonmelanocytic epithelial neoplasias, including 5 verruca vulgaris, 14 seborrheic keratosis, 38 actinic keratosis, 38 basal cell carcinoma (BCC), 15 basosquamous carcinoma, 58 squamous cell carcinoma (SCC), and 9 normal skin. Digital microscopy and a new tissue microarray software linking image and patient data allowed easy and validated evaluation and quality archiving of stained samples. In normal skin and benign epidermal lesions, collagen XVII protein was restricted to basal keratinocytes. However, possibly as a sign of undifferentiated/transformed state, it was widely expressed in SCC showing elevated levels around invasive tumor fronts with some staining in tumor adjacent stroma, endothelium, and histiocytes. Collagen XVII immunostaining of atypical keratinocytes in most actinic/solar keratosis supports the view of their malignancy and common origin with SCC. Squamous component of basosquamous carcinoma showed moderate reaction, whereas islets of BCC were mainly negative reflecting the diverse genotype and phenotype, and pathogenesis of SCC and BCC. These results suggest that collagen XVII neoexpression may be associated with early atypia/malignant transformation of keratinocytes. Further investigations are under way to analyze the potential of these antibodies for tracing progression and metastatic potential of skin tumors.

Melanoma and melanocytic nevi in decorative tattoos: three case reports.

Background: In response to the demands of style and fashion, the number of decorative tattoos has been increasing worldwide. This has been paralleled by a rising incidence of melanocytic proliferations, including melanoma. The coincidence of various dermatological diseases and skin tumors with tattoos has been documented with some frequency, but reports of melanoma associated with tattoos are exceedingly rare. To date, only 13 cases have been documented in the English language literature. The possibility of an association between melanocytic proliferations and tattoos remains an area for further study.

Juvenile pemphigus foliaceus

A 7‐year‐old girl with generalized erythematous, scaling plaques and vesiculobullous lesions on the extremities was diagnosed as having pemphigus foliaceus. Lesional direct immunofluorescence revealed intercellular IgG, IgA and C3 deposition. The patients serum gave positive reactions against one epitope of desmoglein 3 and the epitope of desmoglein 1 in enzyme‐linked immunosorbent assays, but the blood sample for indirect immunofluorescence did not display any circulating antibodies. The patient was successfully treated systemically with prednisolone and dapsone. Currently, she is taking dapsone, 12.5 mg daily. She has been free of lesions for the last 3 years.

Primary lymphoedema associated with xanthomatosis, vaginal lymphorrhoea and intestinal lymphangiectasia

Primary lymphoedema associated with chylous reflux is a very rare clinical entity. We report a 3‐year‐old girl with unilateral lymphoedema, xanthomatosis and vaginal lymphorrhoea. Biopsy also revealed intestinal lymphangiectasia. This paper also presents a brief review of the literature and draws attention to the significance of the xanthomatous eruption in the diagnosis of a chylous reflux.

BRAFV600E mutation in cutaneous lesions of patients with adult Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is characterized by the proliferation of pathologic Langerhans cells. The disease can develop in any age and can affect almost any organ. Cutaneous involvement is frequent in LCH. The recent demonstration of the activating, oncogenic BRAFV600E gene mutation in LCH samples strongly supports the neoplastic origin of the disease.

Strontium ranelate-induced DRESS syndrome with persistent autoimmune hepatitis

A relatively new drug used in the treatment of osteoporosis, strontium ranelate has been associated with several side effects, including increased relative risk of venous thromboembolism (including pulmonary embolism), transient increases in creatine kinase levels, mild gastrointestinal, nervous system and muscular disorders, and drug-induced hypersensitivity syndrome, also called DRESS syndrome (1). DRESS syndrome is a severe, acute drug reaction defined by the presence of fever, skin eruptions and systemic symptoms, including enlarged lymph nodes, abnormal liver function, renal impairment, and pulmonary and cardiac infiltrates, as well as haematological abnormalities, primarily hypereosinophilia and lymphocytosis (2, 3). We report here a case of a patient with strontium ranelate-induced DRESS who developed persistent autoimmune hepatitis.

Periocular Dermatitis: A Micropapular Sarcoid-Like Granulomatous Dermatitis in a Woman

A case is reported of a woman with periorbital micropapular dermatitis, which had a histology of sarcoid-like granulomata. It is an important problem to make a differential diagnosis from Boeck sarcoid or other granulomatosis, mainly by histopathology.