PURPOSE:

To report a first case of bullous pemphigoid (BP) following intravenous fluorescein for fundus angiography. Clinical Features: A 70-year-old male patient was admitted to the intensive care unit with BP and sepsis. He reported a history of fundus fluorescein angiography with a pre-diagnosis of senile macular degeneration 2 months prior to presentation. At that time, fluorescein extravasated at the antecubital region. Following the procedure, pruritus and erythema began at the wrists bilaterally, and quickly spread to the entire body. The patient also reported a history of allergy to human albumin solution (Plamasteril(R); Abbott) 15 years before, during bypass surgery. On dermatologic examination, erythematous patches were present on the scalp, chest and anogenital region. Vesicles and bullous lesions were present on upper and lower extremities. On day 2 of hospitalization, tense bullae appeared on the upper and lower extremities. The patient was treated with oral methylprednisolone 48 mg (Prednol(R); Mustafa Nevzat), topical clobetasol dipropionate 0.05% cream (Dermovate(R); Glaxo SmithKline), and topical 4% urea lotion (Excipial Lipo(R); Orva) for presumptive bullous pemphigoid. Skin punch biopsy provided tissue for histopathology, direct immunofluorescence examination, and salt extraction, which were all consistent with BP. After 1 month, the patient was transferred to the intensive care unit with sepsis secondary to urinary tract infection; he died 2 weeks later from sepsis and cardiac failure.

CONCLUSIONS:

To our knowledge, this is the first reported case of BP following fundus fluorescein angiography in a patient with known human albumin solution allergy. Consideration should be made to avoid fluorescein angiography, change administration route, or premedicate with antihistamines in patients with known human albumin solution allergy. The association between fundus fluorescein angiography and BP should be further investigated.

Lichen sclerosus (LS) is a chronic skin and mucosa inflammatory disease. It affects mainly the female anogenital area especially in postmenopausal period. The main symptoms include pruritus, burning, pain, sometimes urinary problems, or difficulties in defecation. Usually, porcelain-white plaques are seen in the skin and mucosa. The etiology and pathogenesis of LS are still uncertain. There are some research studies on possible genetic predisposition, yet autoimmune, hormonal, or infectious factors are not excluded. The typical treatment of LS is mainly pharmacological, although the alternative treatment method used in LS is photodynamic therapy (PDT), which is noninvasive technique based on selective destruction of lesions. Our study is focused on molecule markers of vascularisation (CD34), nervous cell function (myelin basic protein [MBP]), keratinocyte function (CD44), and proliferation index (Ki67) in cases treated with photodynamic method.A group of 100 patients treated in our department was included in the study. All 100 women had LS on the basis of clinical and histological criteria. All the subjects underwent PDT. In all cases, skin biopsies were taken before and after treatment, and samples were analyzed with CD34, CD44, MBP, and Ki67 antibodies using immunohistochemical staining.The study shows the high efficacy of PDT in LS treatment including beneficial changes to CD34, CD44, and MBP immunostained molecules. The Ki67 proliferation index did not change significantly. A significant increase of CD34 (microvessel density), MBP, and CD44 expression was confirmed in the histological images and in the partial or full remission of clinical objective and subjective symptoms.The PDT is a very effective therapeutic method in LS treatment.

Repeated courses of potent topical corticosteroids and maintenance therapy with moderately potent topical corticosteroids are frequently needed to treat various forms of vulvar dermatoses, which are often characterized by an abnormal proliferation or activation of T lymphocytes. Because such therapeutic regimen is associated with an increased risk of potential side effects, particularly skin atrophy, an anti-inflammatory alternative to topical corticosteroids is desirable. The two non-steroid topical calcineurin inhibitors pimecrolimus and tacrolimus are immunomodulators that block the release of inflammatory cytokines from T lymphocytes in the skin while promoting cutaneous innate host defences. They are currently approved in Europe and in the United States of America as second-line anti-inflammatory agents for the treatment of atopic dermatitis. We provide a comprehensive summary of existing case reports, series of cases, and open-label prospective studies concerning the use of topical pimecrolimus and tacrolimus for the treatment of anogenital lichen sclerosus, genital lichen planus, vulvar lichen simplex chronicus and related pruritic vulvar dermatoses (chronic vulvar pruritus and allergic contact dermatitis of the vulva). The available data suggest that both topical calcineurin inhibitors may be effective and well tolerated in these vulvar dermatoses, although topical pimecrolimus may exhibit a better long-term tolerability profile. Being devoid of steroid-related side effects, they may represent a useful second-line therapeutic option for patients who are intolerant of, or resistant to topical corticosteroids. Controlled clinical trials and comparative studies are warranted to substantiate the promising findings summarized in this review.

Hidradenoma papilliferum (HP) of the anogenital region, which was previously thought to be an apocrine tumour, is now believed to be derived from anogenital mammary-like glands (MLG) and is more accurately termed MLG adenoma. We sought to explore any ramifications that may have resulted from the perceived change in histogenesis of this tumour.We performed a clinicopathological audit of 46 cases.The mean age was 52 years and the range 31-90 years. Symptoms occurred in 23%, comprising nodules of increasing size 9%, pruritus 9% and bleeding 6%. Tumours occurred in the known distribution of mammary-like glands. The labia minora accounted for 50%, labia majora 40%, fourchette 7% and clitoris 3%. Tumours were described clinically as cystic in 42%, ulcerated 33% and solid 25%. Histologically, adjacent normal MLG were often present. There was striking diversity in histology. Tubular, papillary, cystic and solid areas were seen in various combinations. Two cell types, epithelial and myoepithelial, were present. The most common epithelial cell, the ductal cell, was seen alone in 43% or associated with apocrine metaplasia (57%) and/or foam cells (13%) and/or squamous cells (13%). Myoepithelial cells were usually flattened, but were prominent and clear cell in type in 11%. Stroma was variable in amount and either desmoplastic or sclerotic. Inflammatory cells were particularly associated with tumours involving the surface. Unusual architectural patterns resembled breast lesions such as erosive adenomatosis, sclerosing adenosis and ductal adenoma. No recurrence or association with malignancy was recorded.MLG adenomas demonstrate a marked diversity in histological pattern and cell morphology. The ductal cell and a site compatible with and/or the presence of adjacent normal MLG are the most characteristic features. Unusual vulvar tumours, which have been previously reported as erosive adenomatosis, sclerosing adenosis, papillary adenofibroma, syringocystadenoma papilliferans, etc., are variants of MLG adenomas.

Brachioradial pruritus is characterized by the presence of pruritus on the lateral aspect of the arms. The etiology of this enigmatic entity is the subject of some debate some authors claim that brachioradial pruritus is a photodermatosis whereas others attribute it to the presence of underlying cervical radiculopathy. In these case reports, we present our experience with brachioradial pruritus and discuss the role of underlying neuropathy in its etiology and that of other types of localized pruritus such as notalgia paresthetica, anogenital pruritus, and burning mouth syndrome.

Lichen sclerosus is a chronic mucocutaneous inflammatory condition affecting both adults and children in a bimodal age distribution. Children can present as young as 6 months of age but average around 5 years. Females present with vulvar itching, soreness, dysuria, or gastrointestinal complaints, while males tend to have difficulty retracting the foreskin leading to phimosis. On examination, white smooth atrophic plaques are found in the anogenital region with atrophy and possible distortion of anatomy. LS in children has been commonly misdiagnosed as sexual abuse, leading to delay in appropriate diagnosis and unnecessary turmoil for families. It is a chronic relapsing and remitting condition possibly due to autoimmunity. When evaluating patients, a complete history can help guide which patients may benefit from a systemic evaluation for autoimmune disease. Although some patients have spontaneous resolution during puberty, many do not. There is a significant risk of squamous cell carcinoma developing in genital LS in adults possibly from chronic inflammation, delay in diagnosis, and delay in appropriate treatment. The risk of squamous cell carcinoma in pediatric onset LS is undefined. It is also unclear if effective control of cutaneous inflammation can decrease the risk of malignant transformation. Treatment is aimed at decreasing symptoms and returning involved skin to its normal appearance. Relapses are common. Ultrapotent topical corticosteroids are first line for the treatment of LS and can be used intermittently for years for flares. Topical tacrolimus or pimecrolimus are also good treatment options at controlling inflammation. Patients need to be monitored every 6 to 12 months even when asymptomatic because of the potential for development of malignancy. Given the distressing nature of LS, support groups and a multidisciplinary approach are recommended.

HIV-positive patients have unpredictable local immune responses even with severe systemic immunosuppression and data reported to date is insufficient to predict the effect of imiquimod in HIV-positive patients.To evaluate the efficacy of 5% topical imiquimod in HIV-positive male patients with anogenital warts (AGW) and to elucidate its effect on recurrence.Open-label clinical trial.Of the 43 patients enrolled, 86% completed treatment. Patients' mean age was 34 years (range: 19-50). Thirty-one patients were receiving highly active antiretroviral therapy (HAART) therapy. At week 16, 10 patients completely cleared lesions and 21 patients had a wart size reduction > or =50%. At 20 weeks of therapy, 17 patients achieved total clearance whereas 14 patients had a >50% wart reduction. Clearance was not influenced by CD4-counts, HIV-viral load, previous therapy, or wart localization. Of the patients who experienced a complete clearance, five (29%) had a recurrence. Mean time of recurrence was 14.4 weeks. Erythema, pruritus, and burning sensation were the most frequent local skin reactions.Topical 5% imiquimod is safe and may benefit HIV-positive patients with anogenital warts particularly when it is used for up to 20 weeks. It is also useful to decrease wart recurrence.

The topical calcineurin inhibitors (TCIs) pimecrolimus and tacrolimus are approved for atopic dermatitis but have additional potential in other inflammatory skin diseases. This article reviews their clinical use in non-atopic dermatitis diseases. In seborrheic dermatitis, asteatotic eczema, and contact dermatitis, TCIs are of great benefit and can compete with topical corticosteroids. In psoriasis, TCIs have shown clinical efficacy and safety in facial and intertriginous lesions. Further investigations into possible combinations of TCIs with other established treatments such as UVB irradiation in this disorder are necessary. Initial studies in cutaneous lupus erythematosus have been promising, whereas the response in rosacea and rosacea-like eruptions has been mixed. TCIs have been associated with good clinical responses in oral lichen planus and anogenital lichen sclerosus et atrophicus. In vitiligo, TCIs are associated with some degree of repigmentation, with better results being seen in children and in facial and neck areas. TCIs have a synergistic effect with UVB irradiation in vitiligo. There is a long list of small series and case reports documenting use of TCIs in various other skin conditions that warrant further validation. Although the established mode of action of TCIs is T-cell control, other effects also need to be considered. Specifically, TCIs reduce pruritus and erythema, which cannot be explained by T-cell interactions, and further investigations are needed in these fields.

Lichen sclerosus is a chronic inflammatory autoimmune disease causing significant sclerosis, atrophy and pruritus. Treatment remains unsatisfactory, with potent corticosteroids being the most effective therapy.To conduct a multicentre, phase II trial to assess the safety and efficacy of tacrolimus ointment 0.1% for the treatment of lichen sclerosus with a follow-up period of 18 months at 10 university and teaching hospitals in Germany and Austria.Eighty-four patients (49 women, 32 men and three girls) aged between 5 and 85 years with long-standing, active lichen sclerosus (79 with anogenital and five with extragenital localization) were treated with topical tacrolimus ointment 0.1% twice daily for 16 weeks. Computerized analysis of the lesional area was performed. The primary endpoint was clearance of active lichen sclerosus. Secondary endpoints were time to optimal response, reduction of sclerosis and duration of remission.The primary endpoint (clearance of active lichen sclerosus) was reached by 43% of patients at 24 weeks of treatment. Partial resolution was reached in 34% of patients. Maximal effects occurred between week 10 and 24 of therapy. Treatment led to a significant reduction of the total lesional area (P < 0.01) and to a significant decline in the total symptom score (P < 0.005). Symptoms (e.g. itching) and findings (erythema, erosions and induration) showed significant improvement. No serious adverse events were observed. There were three (9%) recurrences during the follow-up period.Topical tacrolimus ointment 0.1% was safe and effective for the treatment of long-standing active lichen sclerosus.

Lichen sclerosus et atrophicus is a chronic inflammatory disorder that mainly affects girls of premenarchial age and women in their fifties. Besides the anogenital region, lichen sclerosus (LS) may also affect extragenital or mucosal areas. Symptoms include soreness and pruritus, but other less common symptoms are not rare. An increased activity of fibroblasts causes increased sclerosis of the affected skin. Latest studies have identified LS as a chronic inflammatory dermatosis. Auto-antibodies against the extracellular membrane protein-1 are present in up to 80% of the patients. Moreover, in the widely accepted therapy with potent corticosteroids promising results have been presented using calcineurin antagonists in the treatment of LS. An interdisciplinary management and a continued care of patients with LS will improve the clinical manifestations and quality of life.