Leukocytoclastic vasculitis in association with linear epidermal basement membrane zone immunoglobulin deposition: Linear vasculitis.

Cutaneous leukocytoclastic vasculitis (LCV) has a distinctive clinical and light microscopic presentation; however, the etiologic basis of LCV is varied. Most cases are attributable to immune complex deposition within a vessel wall and represent an Arthus type III immune complex reaction. The prototypic immunoreactant profile is characterized by granular deposits of components of complement activation in concert with immunoglobulin within the cutaneous vasculature. We encountered nine patients with vasculitic and/or vesiculobullous clinical presentations exhibiting an LCV in association with an immunoreactant profile characterized by homogeneous linear deposits of immunoglobulin along the dermal epidermal junction in a fashion resembling an autoimmune vesiculobullous disease. Among the clinical presentations were palpable purpura, urticarial vasculitis, and vesiculobullous eruptions with supervening purpura. Two patients with Crohn disease presented with classic palpable purpura with biopsy-proven LCV, and direct immunofluorescence (DIF) studies demonstrated linear immunoglobulin G (IgG) with floor localization on the salt-split skin assay. Four patients with systemic lupus erythematosus (SLE) showed purpuric vesiculobullous lesions, with evidence of a neutrophilic interface dermatitis and LCV in three of the four. The remaining patient had urticarial nonbullous lesions showing small-vessel vasculitiswith a neutrophilic interface dermatitis. In all of the patients with SLE, DIF studies showed linear immunoglobulin deposits within the basement membrane zone (BMZ). These constellation of findings clinically, light microscopically, and by immunofluorescence were those of a vasculitic presentation of bullous systemic lupus erythematosus. Two patients had linear IgA disease, which was drug induced in one and paraneoplastic in the other, and the dominant morphology on biopsy in both cases was an LCV. One patient microscopically demonstrated drug-associated and eosinophilic enriched LCV with DIF studies showing striking linear deposits of IgG suggestive of bullous pemphigoid, which was consistent with a vasculitic presentation of drug-induced bullous pemphigoid. In all cases, typical granular vascular immunoglobulin and complement deposition compatible with immune complex mediated vasculitis was observed. It is likely that local immune complexes derived from BMZ antigen bound to antibody are pathogenically relevant. We propose the designation of linear vasculitis for this unique scenario of LCV and linear immunoglobulin epidermal BMZ staining, which in some cases represents a vasculitic presentation of conventional autoimmune vesiculobullous disease.

Annular lipoatrophy of the ankles: case report and review of the literature.

Annular lipoatrophy of the ankles also termed "annular atrophic connective tissue panniculitis of the ankles,""annular atrophy of the ankles," and "atrophic annular panniculitis of the ankles," is an inflammatory panniculitis that results in lipoatrophy with a predilection for the ankles. The disorder is seen predominantly in female children and demonstrates a mixed lobular panniculitis. It is likely a disorder of autoimmune etiology, supported by the findings of concurrent autoimmune disorders in several patients. We report a case of a 6-year-old female with annular lipoatrophy of the ankles associated with Hashimoto thyroiditis and review the epidemiology and proposed pathogenesis of annular lipoatrophy of the ankles.

Amelanotic lentigo maligna: a report of three cases and review of the literature.

BACKGROUND: Amelanotic lentigo maligna is not clinically suspected and is often mistaken for a basal cell carcinoma, squamous cell carcinoma, or dermatitis. OBJECTIVE: Our objective was to review previously reported cases of amelanotic lentigo maligna and compare them with our 3 cases. METHODS: The clinical presentation and histologic findings of 3 new cases are described and compared with those in the literature. RESULTS: The index of suspicion for amelanotic lentigo maligna is extremely low. No reported cases have been diagnosed clinically. None of our 3 cases was suspected. LIMITATIONS: Only three cases were reviewed. CONCLUSION: A high degree of clinical and histologic suspicion is required to make the diagnosis of this clinically nondescript neoplasm.

Early-onset lichenoid graft-vs.-host disease: a unique variant of acute graft-vs.-host disease occurring in peripheral blood stem cell transplant recipients.

BACKGROUND: A complication of stem cell transplantation is chronic graft-vs.-host disease (GvHD), developing months to years after transplant; the two commonest manifestations are lichenoid GvHD and scleroderma. The purpose of this study was to characterize early-onset lichenoid GvHD. METHODS: A retrospective study identified patients diagnosed with early-onset lichenoid GvHD. This diagnosis was correlated with type of transplant and concurrent or prior episodes of acute GvHD. RESULTS: Patients in whom a sex mismatch was present between donor and recipient were included, representing a study population of 17. All received an allogeneic peripheral blood stem cell transplant (PBSCT). All patients had biopsy proven lichenoid GvHD within 60 days or less following transplantation. All had concurrent gastrointestinal symptoms which was biopsy proven GvHD in thirteen of the cases. FISH XY studies revealed that the infiltrating lymphocytes were of donor origin in 12 of the cases, mixed in three and of host origin in two cases. CONCLUSIONS: Early-onset lichenoid GvHD is exclusive to the PBSCT setting and appears to be mediated by donor lymphocytes, reflecting the higher numbers of donor T cells encountered in PBSCT. We consider this reaction pattern a distinctive subtype of acute GvHD.

Granulomatous pigmented purpura: an unusual histological variant.

Pigmented purpuric dermatoses (PPDs) tend to vary clinically, yet share a similar histology. Granulomas are only rarely seen, with 4 prior reported cases, designated granulomatous pigmented purpura. Historically, granulomatous pigmented purpura has been seen in Asians. In this article, we report the case of a 42-year-old, white female with granulomatous pigmented purpura, the fifth such reported case in the literature. Histopathological examination revealed a granulomatous dermatitis with eosinophils, extravasated erythrocytes, melanophages, and vascular proliferation. These findings were consistently reported on subsequent biopsy 6 months later. This case illustrates a rare histological finding in PPDs. Medications associated with PPD are reviewed and the patient's use of mesalamine and balsalazide for ulcerative colitis are deemed potential triggers, given their relative similarity to aspirin, a known trigger of PPD. Although other granulomatous processes must be excluded in such patients, one must consider the possibility of granulomatous pigmented purpura in the appropriate setting.

Cutaneous lesions of dermatomyositis with supervening fibrosis.

BACKGROUND: Dermatomyositis (DM) is a distinctive systemic connective tissue disease whereby the skin defines a cardinal site of involvement. There exists a body of literature, which suggests that a significant component of its clinical manifestations may be related to endothelial cell injury. We have postulated in the past that anti-endothelial cell antibodies may be the defining trigger leading to endothelial cell dysfunction. The primary organs affected by DM are the skin and muscle. A significant albeit rare complication is pulmonary fibrosis, which our recent study postulated to be attributable to an autoimmune endothelialitis. DESIGN: We describe six patients, four women and two men who ranged in age from 3 to 60 years, and had classic clinical presentations and cutaneous lesions of DM without any supervening clinical changes indicative of cutaneous sclerosis. RESULTS: Skin biopsies showed cell-poor interface dermatitis with variable dermal mucin and C5b-9 within the cutaneous vasculature. However, at variance with classic DM was the presence of a sclerodermoid tissue reaction, which was of variable depth. All of these patients had severe muscle involvement. One pediatric patient had concomitant significant cutaneous, central nervous system and oral mucosal ischemic infarcts. Significant pulmonary disease ensued in the four adult patients, manifesting as pulmonary fibrosis in two, diffuse alveolar damage in one and diaphragmatic failure in one. In three patients, direct immunofluorescent studies were corroborative of immune-based microvascular injury while Western blot and/or indirect immunofluorescent studies showed anti-endothelial cell antibody activity within the serum of three patients. CONCLUSIONS: The identification of sclerosis in biopsies of skin lesions typical clinically for DM may be a harbinger for more severe autoimmune-based endothelial cell injury phenomenon. One could speculate that its basis may be attributable to elevated serum levels of the natural fibrogenic factor, transforming growth factor beta, which in turn is released from damaged endothelium.

Shrinkage of cutaneous specimens: formalin or other factors involved?

BACKGROUND: Shrinkage of cutaneous tissue during processing is a source of controversy. This study was designed to prospectively determine tissue shrinkage at two intervals: 1 min after excision and after 24 to 48 h of formalin fixation. Secondarily, gender, age, site, prior biopsy scar and solar elastosis were evaluated with respect to shrinkage. METHODS: Ninety-seven cutaneous specimens were measured prior to excision, 1 min after removal and after 24 to 48 h of formalin fixation. Width of prior biopsy scar, damage to elastic fibers and solar elastosis were subjectively quantified. RESULTS: Significant tissue shrinkage occurred immediately after excision, prior to formalin fixation. Mean shrinkage (95% confidence interval): length 20.66% +/- 2.15% and width 11.79% +/- 2.35%. Range of shrinkage: length 0 to 41.18% and width -18.75% (indicating expansion) to 37.50%. Patient age was significant; shrinkage decreased 0.3% per year of increasing age. Site was less significant; trunk excisions measured 5% greater shrinkage than head/neck excisions. As solar elastosis increased, shrinkage decreased. CONCLUSIONS: Cutaneous tissue shrinkage following excision is primarily because of intrinsic tissue contractility. Increasing patient age and solar elastosis correlate with less shrinkage. The clinicians and dermatopathologists must be cognizant of the expected shrinkage of submitted specimens for settling discrepancies within the medical record.

Off-label uses of biologic agents in dermatology: a 2006 update.

The introduction of a number of biologic therapies into the market has revolutionized the practice of dermatology. These therapies include adalimumab, alefacept, efalizumab, etanercept, infliximab, IVIg, omalizumab, and rituximab. Most dermatologists are familiar with the indications of these medications that have been approved by the Food and Drug Administration; however, numerous off-label uses have evolved. To update the reader on more recent uses of the biologics for off-label dermatologic use, this article will emphasize more recent published data from 2005 through the date of submission in May 2006.