Eruptions in life-threatening rheumatologic diseases.

Dermatologic changes occur in a variety of rheumatic diseases. Skin can be the initial site of involvement, thus providing important clues for an accurate diagnosis based on cutaneous findings. Dermatologic findings can also be an indicator of systemic involvement and prognostic outcome; however, many connective tissue disorders have a wide variety of cutaneous manifestations, with significant overlap between different diseases. These skin signs often precede systemic clinical manifestations. Careful attention to characteristic dermatologic findings in Behçet's disease, systemic lupus erythematosus, rheumatoid arthritis, and various vasculitis can provide prompt therapeutic approaches in the case of life-threatening complications of systemically involved rheumatologic diseases.

Assessment of treatment efficacy of diphenylcyclopropenone (DPCP) for alopecia areata

Background/aim: Alopecia areata (AA) is an inflammatory disease with a genetic and autoimmune basis. Herein, it was aimed to study the efficacy and safety of an immunomodulatory therapeutic agent, diphenylcyclopropenone, while manifesting its association with histopathological features, prognostic factors, and side effects. Materials and methods: In this retrospective study, 98 patients (60 males, 38 females) with alopecia, who were referred to the Hair Disease Polyclinic at the Department of Dermatology, between 2011 and 2015, were included. Together with medical histories and dermatological examinations, a skin biopsy for histopathological examination was conducted for all of the patients prior to therapy. Therapeutic success was evaluated on the basis of the hair regrowth percentage. Results: Regarding the overall treatment success, 33 (34%) patients had complete response, 16 (16%) had partial response (between 50% and 99%), 27 (28%) had minimal response (between 1% and 49%), and 22 (22%) were nonresponders. Both sexs were equally represented in the outcome. Conclusions: There was a significant relation between the severity of alopecia and the treatment outcome (P = 0.038). Patients with AA had significantly better response when compared to those with alopecia totalis and universalis. There was no statistically significant relation with other parameters, such as disease duration, age, sex, atopy history, age of onset, and histopathological features.

Behçet's disease as a systemic disease.

Behçet's disease usually begins with cutaneous manifestations, such as recurrent aphthous stomatitis, genital ulcers, erythema nodosum-like lesions, papulopustular findings, and pathergy phenomenon. Recurrent aphthous stomatitis is generally the first sign, and other findings may develop in the course of the disease. There is no specific diagnostic available for Behçet's disease. It is most prevalent among patients along the ancient Silk Road. The high frequency of HLA-B51 among a wide range of ethnic populations favors the role of genetic factors. Behçet's disease usually appears in the third to fourth decade of life, and is rarely seen in children and adults over 50 years of age. It affects both genders equally, but the course of the disease is more severe in men. Eye involvement leading to loss of vision, plus vascular, articular, and central nervous system involvement are more commonly observed among men. Behçet's disease is a systemic inflammatory disorder. A complex genetic background, coupled with innate and adaptive immune system activation, causes the diverse clinical manifestations that characterize the clinical picture.

Photodermatoses, including phototoxic and photoallergic reactions (internal and external).

Photodermatoses are caused by an abnormal reaction mainly to the ultraviolet component of sunlight. Photodermatoses can be broadly classified into four groups: immunologically mediated photodermatoses, chemical- and drug-induced photosensitivity, photoaggravated dermatoses, and DNA repair-deficiency photodermatoses. In this review, we focus mainly on chemical- and drug-induced photosensitivity, namely, phototoxicity and photoallergy.

Rosai-Dorfman disease: a case report with nodal and cutaneous involvement and review of the literature.

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a benign proliferative disorder of histiocytes with an unknown etiology. It is a rare disease characterized by overproduction and accumulation of histiocytes within lymph node sinuses and many other extranodal sites, including skin, oral and nasal cavities, respiratory tract, eyelid, and periorbital area. In this case, a 44-year-old woman with diagnosis of Rosai-Dorfman disease, with xanthelasma-like cutaneous lesions on facial area, extending to her neck and acneiform papules on her back, cervical lymph node involvement, and concomitant presence of diabetes insipidus was presented. Histopathological examination of the lesions demonstrated diffuse lymphocyte, plasmocyte, eosinophil, and sparse neutrophil infiltration, together with histiocytes showing phagocytosed inflammatory cells (emperipolesis). Histiocytes demonstrated immunoreactivity with the antibodies for CD68 and S100, whereas they were negative for CD1a and Langerin. Laboratory tests were normal, except mild immunoglobulin G hypergammaglobulinemia. Systemic methylprednisolone therapy was effective for cutaneous lesions.

Behçet's syndrome: facts and controversies.

Behçet's syndrome is a systemic vasculitis of small and large vessels affecting both veins and arteries. Almost all patients with Behçet's syndrome have recurrent oral aphthae, followed by genital ulcers, variable skin lesions, such as erythema nodosum and papulopustuler lesions, arthritis, uveitis, thrombophlebitis, and gastrointestinal and central nervous system involvement. Recent epidemiologic works suggest that genetic factors are more important than environmental factors in its pathogenesis. European League Against Rheumatism guidelines were recently published for the treatment of Behçet's syndrome. Although these are quite useful for the management of mucocutaneous, eye, and joint involvement, treatment of vascular, neurological, and gastrointestinal involvement are still problematic as there are no controlled studies for these manifestations. This contribution addresses the epidemiology, mucocutaneous manifestations, diagnostic criteria, and evidence-based therapies, including biologic agents.