Fitzpatrick's Dermatology in General Medicine.

Guideline for the diagnosis, treatment and long-term management of cutaneous lupus erythematosus

Behcet's syndrome (BS) is a multi-systemic vasculitis of unknown aetiology. More than one mechanism seems to be operative in the pathogenesis of Behcet's syndrome, including genetic and environmental factors, causing different manifestations of the syndrome. There are several clues to the role of environmental factors and especially micro-organisms in the pathogenesis. These include clinical findings such as a decrease in the frequency of a positive pathergy reaction with surgical cleaning of the skin before the procedure, the acne–arthritis association carrying similar features to acne-associated reactive arthritis, a higher rate of tonsillectomy, cold sores, late birth order, higher number of siblings, history of travel to countries with a high incidence of BS and earlier age at first sexual intercourse. Moreover, basic research on both viruses and bacteria suggests that micro-organisms may be playing a role, possibly through heat shock proteins and T-cell hypersensitivity.

Behçet's syndrome and micro-organisms.

Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus erythematosus. It tends to heal with scarring, hair loss, and pigmentary changes if treatment is not initiated in the early phase of the disease. Classic DLE lesions are initially red-purple macules, papules, or small plaques that rapidly acquire a hyperkeratotic appearance. Only a minority of patients with DLE progress to develop systemic lupus erythematosus (SLE). A small percentage of patients with SLE have concomitant DLE. However, generalized DLE is more frequently associated with systemic involvement than classic DLE. The diagnosis of DLE is usually based on clinical features, although in some cases histopathological examination may be required to confirm the diagnosis. Standard therapy for cutaneous lupus erythematosus includes broad-spectrum sunscreens, topical and intralesional glucocorticoids, and antimalarial agents. A 63-year-old man presented with erythematous scaly patches that he had on the face for approximately eight months. Although the face was the main affected site, lesions were also noted on the scalp, neck, chest, shoulder, upper arms, and trunk. Histopathological examination verified the diagnosis of DLE. Laboratory examination and consultation with other departments did not reveal any systemic involvement. Imiquimod cream 5% was applied three times a week, every other week. After 24 applications over a period of two months, an almost complete recovery was achieved. Topical imiquimod may be an alternative treatment for generalized DLE.

/pdf/xanthoma-disseminatum-case-report-and-mini-review-of-the-xr7lqb3dn6.pdf

Xanthoma Disseminatum: Case Report and Mini-Review of the Literature

Importance Nonsexual acute genital ulceration (NAGU) is a rare vulvar skin condition typically affecting girls and young women, characterized by acute onset of singular or multiple painful vaginal ulcers. The etiology of this ulcerative dermatosis has not been identified, although it has been associated with systemic infections. To our knowledge, this is the first report of an association with Lyme disease. Observations A case of a woman with early disseminated Lyme disease presenting with NAGU is reported. A thorough workup ruled out other causes of genital ulceration, and the ulcers completely resolved after treatment with topical steroids and oral doxycycline. Conclusions and Relevance Although the etiology of NAGU is unknown, the vulvar ulcers may result from an exuberant immune response to infection. Most patients with NAGU exhibit nonspecific symptoms such as myalgias and fever, suggesting an infectious agent, but the majority have no identifiable pathogen. In addition to previously reported associations with systemic infection, which are reviewed herein, Lyme disease should be considered in women presenting with acute-onset genital ulcers.

https://jamanetwork.com/journals/jamadermatology/articlepdf/1899258/dcr140017.pdf

Disseminated Lyme Disease Presenting With Nonsexual Acute Genital Ulcers

Cutaneous metastasis of neuroendocrine carcinoma

A case is presented of a 27-year-old Caucasian woman that presented with an oval purplish patch with defined borders on her right cheek. It was swollen and tense, with a mottled surface due to scarring. Histologic examination indicated discoid lupus erythematosus and the patient was initially treated with medical therapy. The lesion relapse and cosmetic results convinced us to propose surgical option with aggressive medical treatment and follow up. In our patient, this combination therapy proved effective.

/pdf/medical-and-surgical-treatment-for-discoid-lupus-s95qe7rzh6.pdf

Medical and Surgical Treatment for Discoid Lupus Erythematosus

Introduction : Nailfold videocapillaroscopy (NVC) is a non-invasive imaging technique widely used to investigate microvascular abnormalities in different connective tissue diseases (CTDs). Methods : We conducted a retrospective study where we analysed 415 patients submitted to NVC. Patients with scleroderma-like pattern were selected to investigate if there are specific NCV changes, which discriminate among the different CTDs. Ninety-one patients met this requirement and had a diagnosis of CTD. For each patient the following abnormalities were observed: enlarged and giant capillaries, oedema, loss and rarefaction of capillaries, long loops and minor dystrophies. Results : Multivariate analyses did not reveal any specific modification among the analysed co-variables for scleroderma (SS) and dermatomyositis (DM). For the others CTDs analysed in this study, logistic regression revealed that some of the capilloroscopic features could be indicative of specific diseases. Of note, the presence of megacapillaries with long loops in a scleroderma-like pattern seems to be highly indicative for a diagnosis of systemic lupus erythaematosus (SLE). Conclusions : Our data showed that in CTDs with a scleroderma-like pattern, the NVC variables alone are not able to discriminate for a specific diagnosis of CTD. Nevertheless, there are some NVC features, which could strongly address the differential diagnosis toward a specific CTD.

Is Scleroderma Pattern Able to Address a Specific Diagnosis of Connective Tissue Diseases

Is scleroderma pattern able to address a specific diagnosis of connective tissue

this case, we think that any perianal lesion in a patient who suffers from intestinal CD should be suspected as perianal CD and a biopsy should be conducted. If perianal CD occurred as the first manifestation of CD, it could be difficult to diagnose. In such cases, the relative lack of pain, multiplicity of lesions, oedema of skin tags, and eccentricity of fissures can be important clinical indicators.

Adamantiades Behçet Disease triggered by a tick bite and or borrelia infection.

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