Epidermolysis bullosa pruriginosa, a genetic mechanobullous disease, manifests at birth or late in life and is characterised by intense pruritus, resulting in lichenified or nodular prurigo-like lesions and scarring most prominent on the shins. Treatment is unsatisfactory. We report a patient treated with success using a combination of topical and systemic agents.
View Article and Find Full Text PDFAllergic contact dermatitis (ACD) is a type IV, delayed-type reaction caused by skin contact with low-molecular-weight organic chemicals and metal ions that activate antigen-specific T cells, primarily T-helper 1 (Th1), in a sensitized individual, leading to skin eczema.First-line treatments are based on avoidance of causal agents and topical corticosteroids/immunomodulators. In recalcitrant cases, chronic oral immunosuppressive agents may be used, but they may have serious adverse effects and do not address the immunological disfunction.
View Article and Find Full Text PDFPyodermatitis vegetans (PV) is a rare inflammatory dermatosis of unknown etiology and difficult diagnosis characterized by vesiculopustular, exudative, and vegetating plaques usually localized in the axillary, genital, and oral region. It bears similarities to pemphigus vegetans but clinical history, histology, and immunofluorescence help confirm the diagnosis. Pyodermatitis vegetans is often associated with an underlying gastrointestinal disorder, especially ulcerative colitis.
View Article and Find Full Text PDFActa Dermatovenerol Alp Pannonica Adriat
January 2017
Pityriasis rubra pilaris is a chronic inflammatory dermatosis of unknown etiology and great clinical variability. It has been divided into six categories. Types III, IV, and V occur in childhood and are distinguished by their clinical presentation, age of onset, and course.
View Article and Find Full Text PDFBrachioradial pruritus is a chronic sensory neuropathy of unknown etiology which affects the skin of the shoulders, arms and forearms on the insertion of the brachioradialis muscle. We describe the case of a 60-year old woman recently diagnosed with multiple myeloma who refers paresis, severe pruritus and itching lesions on the right arm with 6 months of evolution. Investigation led to a diagnosis of Brachioradial pruritus consequent to the presence of cervical disc herniation and Parsonage-Turner syndrome.
View Article and Find Full Text PDFCrohn's disease is a multisystem chronic granulomatous inflammatory disease that primarily affects the gastrointestinal tract. In the majority of the cases, the cutaneous manifestations follow the intestinal disease, but occasionally dermatological lesions are the inaugural event and may constitute the only sign of the disease. Vulvoperineal involvement is rare, may precede bowel symptoms by months to years and may go unrecognized.
View Article and Find Full Text PDFAntiphospholipid syndrome is a relatively recent systemic autoimmune disorder defined by thrombotic events and/or obstetric complications in the presence of persistent elevated antiphospholipid antibodies. It\'s characterized by a wide spectrum of clinical presentations and virtually any organ system or tissue may be affected by the consequences of vascular occlusion. Diagnosis is sometimes difficult and although classification criteria have been published and revised there remain ongoing issues regarding nomenclature, expanding clinical features, laboratory tests and management and much still has to be done.
View Article and Find Full Text PDFA 50-year-old man presented with a scaly erythema of the face, upper chest, forearms, and dorsum of the hands. He has been treated with cyamemazine for 6 months. Photopatch tests were performed and the patient was diagnosed with photoallergic reaction to cyamemazine.
View Article and Find Full Text PDFWe report a clinical case of a rare variant of pemphigus - pemphigus herpetiformis - which combines the clinical features of dermatitis herpetiformis with the immunological findings of pemphigus. Due to its atypical presentation, it is frequently misdiagnosed as dermatitis herpetiformis. It is basically characterized by the herpetiform pattern of skin lesions, severe pruritus and by the presence of eosinophilic spongiosis confirmed on histopathology.
View Article and Find Full Text PDFVesico-bullous subacute cutaneous lupus erythematosus is an uncommon and severe presentation. The authors report an exuberant case of vesico-bullous subacute cutaneous lupus erythematosus successfully treated with dapsone and hydroxychloroquine.
View Article and Find Full Text PDFStevens-Johnson syndrome and toxic epidermal necrolysis are potentially life-threatening adverse cutaneous drug reactions. Clinically, it manifests as a rash, often morbilliform, or atypical target lesions that evolve to epidermal detachment. There is mucosal involvement in almost all patients.
View Article and Find Full Text PDFIntroduction: Recently, the tumor necrosis factor-α inhibitors (anti-TNF-α) have been extensively used in clinical practice, in the treatment of several immune-mediated disorders, such as inflammatory bowel diseases, rheumatoid arthritis and more recently in psoriasis.
Case Report: A 35-year-old woman, suffering from Crohn's disease, diagnosed in 1994, successfully treated with infliximab at the dosage of 5 mg/Kg every 8 weeks, since 10 months ago, was referred to our Dermatology Department due to the development of erythematic-scaling confluent plaques on the scalp, back and umbilical fold. The skin biopsy confirmed the clinical diagnosis of psoriasis.
Pellagra is a nutritional disease caused by the deficiency of niacin. We describe a case of pellagra as the initial presentation of Crohn disease, which has been rarely described in the literature.
View Article and Find Full Text PDFGranuloma annulare is a benign, usually self-limited, dermatosis of unknown cause. Generalized lesions occur in approximately 15 percent of patients with GA and may cause mild to severe cosmetic disfigurement. The treatment of generalized granuloma annulare can be challenging.
View Article and Find Full Text PDFKaposi sarcoma remains an important cause of morbidity in HIV-infected patients. Regardless of the recent pharmacological progress, treatment of this malignancy is still disappointing. We report the case of a patient with Kaposi sarcoma in an unusual localization, the penis, which completely resolved with highly active antiretroviral therapy alone.
View Article and Find Full Text PDF