Esophageal Lichen Planus: The Efficacy and Safety of Tacrolimus in Reducing Inflammation and Need for Dilation.

BACKGROUND AND AIMS: Esophageal Lichen Planus (ELP) is a rare inflammatory disease most seen in middle-aged Caucasian women, manifested by sloughing mucosa, thick exudate, and proximal strictures. Most case reports and small series highlight using steroids and other immunosuppressants. To our knowledge, oral tablet tacrolimus has not been studied. We aimed to assess the change in ELP after oral tacrolimus treatment. METHODS: The primary outcome was the efficacy of tacrolimus objectively through our scoring system, ELPSS. All consecutive adults with ELP who underwent more than one EGD by two esophagologists and being treated with tacrolimus or other treatment were eligible for inclusion in this retrospective cohort study. Inflammation and fibrostenotic disease were graded using the novel ELP Severity Score (ELPSS). RESULTS: Twenty two patients met the inclusion criteria. Half (11) received tacrolimus (dose 1-2 mg BID) and half (11) received other therapy (i.e. cyclosporine, topical steroids, or none). Mean ELPSS on first EGD, extraesophageal manifestations of disease, presenting symptoms, and baseline characteristics were similar between groups. Among patients on Tac vs No-Tac, there was a statistically significant improvement in ELPSS (mean difference 1.8 pts; 95% CI 0.25-3.38; P=0.02). Response rate was 89% with Tac vs 30% with No-Tac (P=0.025). All 22 patients underwent bougie dilation safely with a mean diameter of 16 mm achieved. Patients on Tac also required less frequent dilation. CONCLUSION: Oral tablet tacrolimus reduced the inflammatory and fibrostenotic components of ELP. Thus, low-dose oral tacrolimus is safe and should be considered in patients with more severe disease.

Lupus Erythematosus Tumidus as a Distinct Uncommon Subtype of Cutaneous Lupus Erythematosus: A Case Report and Review.

Lupus erythematosus tumidus (LET) is an uncommon but distinct photosensitive subtype of cutaneous lupus erythematosus (CLE). It differs from discoid and subacute cutaneous lupus erythematosus (SCLE) clinically and pathologically. LET is marked by extreme photosensitivity and carries a much lower risk of progression to systemic disease. The differential diagnosis of LET includes polymorphic light eruption (PMLE) and Jessner's lymphocytic infiltration of the skin (JLIS) because of subtle alterations in the histopathology and the paucity of immunopathologic markers in LET. We report herein a case of LET with positive immunoglobulin (Ig) deposits on direct immunofluorescence (DIF) testing. LET resolved completely with strict sun avoidance and treatment with topical corticosteroids, without the sequelae of atrophy, scarring, or dyspigmentation.

Demystifying Esophageal Lichen Planus: A Comprehensive Review of a Rare Disease You Will See in Practice.

Lichen planus (LP) is a chronic inflammatory disorder that often affects the skin, hair, nails, and mucus membranes. Although esophageal involvement has traditionally been felt to be rare, recent reports suggest that it is often unrecognized or misdiagnosed. The diagnoses of esophageal lichen planus can be challenging and is suspected based on patients' endoscopic and histologic findings and in the context of their clinical history and physical examination. Physicians must have an index of suspicion, particularly in older white women and in those patients with an atypical esophagitis or stricturing disease, which do not respond to traditional treatment. Currently, there are limited data on esophageal lichen planus patients, and no formal management guidelines for this disease, which all gastroenterologists will see in practice. This article reviews the etiology and histopathology of LP and provides a comprehensive discussion of the clinical features, diagnosis, and management of esophageal disease from the gastroenterologist's perspective. Finally, we address the esophageal complications of LP.

Safety of efalizumab in patients with moderate to severe chronic plaque psoriasis: review of clinical data. part II.

BACKGROUND: The efficacy and safety of efalizumab have been evaluated in multiple clinical trials. OBJECTIVE: The purpose of this review is to provide an overview of the safety profile of efalizumab during the clinical trials. METHODS: Twelve-week data from four placebo-controlled trials were pooled and analyzed. Data from patients receiving 13-60 weeks of efalizumab therapy were pooled to evaluate longer-term safety. RESULTS: The most common adverse events were mild to moderate, self-limiting, flu-like symptoms that were most frequent following the first two efalizumab doses; by the third dose the incidence was comparable to placebo. Serious adverse events were observed in 2.2% and 1.7% of efalizumab- and placebo-treated patients, respectively. Nonserious adverse events leading to withdrawal were infrequent and similar to placebo (2.8% vs 1.8%). There does not appear to be increased risk of end-organ toxicity, infection, or malignancy in efalizumab-treated patients. CONCLUSION: Efalizumab was well tolerated, with a favorable safety profile.

Long-term implications of T-cell receptor gene rearrangement analysis by Southern blot in patients with cutaneous T-cell lymphoma.

BACKGROUND: T-cell clonality analysis by Southern blot (TSB) in skin biopsy specimens suggestive of mycosis fungoides may be helpful in confirming the diagnosis of a cutaneous lymphoma. However, there are no data available regarding the long-term prognostic implication of such results. OBJECTIVES: We sought to determine the long-term prognostic significance of TSB results from skin biopsy specimens of patients with mycosis fungoides. METHODS: We reviewed the records from the Cleveland Clinic Foundation and Northwestern University Medical Center for cases of biopsy-proven mycosis fungoides with results available for skin biopsy TSB from 1987 to 1990. RESULTS: The detection of clonality by TSB correlates with a higher TNM stage (median stage for positive TSB, IIb vs negative TSB, Ib; P <.05), but not with age at presentation (62 vs 59 years) or duration of disease before presentation (6.2 vs 5.9 years). Although the long-term survival was not significantly different between the 2 groups, there was a trend for patients with positive TSB to die earlier (5-year survival of 67% vs 87%). Disease progression did not correlate with TSB results. Higher clonality rates were noted among patients with biopsy specimens showing a denser lymphoid infiltrate and a higher grade of cytologic atypia. CONCLUSIONS: Detection of clonality with TSB requires a significant clonal burden. Although clonality can be detected in patients with patches and plaques (T1 and T2) most cases with positive results were obtained from patients with advanced disease (T3 and T4). In our experience, detection of clonality by TSB does not correlate with disease progression and does not carry long-term prognostic implications.

Implant retained overdentures for two patients with severe lichen planus: a clinical report.

Lichen planus is a common inflammatory mucocutaneous disease that often manifests itself intraorally. Oral lichen planus can appear in many forms; the most significant form for the edentulous patient is the erosive variety. For the patient, wearing a complete denture is quite dramatic because of the friability of the tissue. Implant-supported overdentures are a predictable treatment for edentulous patients, but this has been discouraged for the patient with erosive lichen planus. This article describes 2 patients with oral erosive lichen planus who were successfully treated with implant-retained mandibular overdentures.

Successful Breast Conservation in a Patient with Epidermolysis Bullosa Simplex.

Before breast conservation can be offered to a woman with breast cancer one must understand both the indications and contraindications to such an approach. Factors that play a role in this decision include tumor-related factors and factors related to the expected cosmetic outcome following breast conservation. Here we present a case of a woman with epidermolysis bullosa simplex (EBS), a rare skin disorder that is characterized by blister formation following minor trauma, who underwent successful breast conservation.