Three cases of localized cutaneous nodular amyloidosis in patients with limited systemic sclerosis and a brief literature review.

Localized cutaneous nodular amyloidosis (LCNA) is the rarest form of localized cutaneous amyloidosis. In patients with LCNA, local plasma cells secrete immunoglobulin light chains called amyloid L via an unknown mechanism. LCNA has been associated with autoimmune connective tissue diseases such as most commonly Sjögren syndrome. A few reported cases of LCNA are associated with limited systemic sclerosis (LSSc). We report three cases of LCNA in patients with LSSc to add to the existing literature, discuss the disease association and proposed pathophysiology, and briefly review the existing information in current literature. It is important to closely follow patients with LCNA to monitor progression to systemic amyloidosis.

Antimalarial lichenoid tissue reactions in patients with pre-existing lupus erythematosus.

Recently a number of cases of drug induction or exacerbation of lupus erythematosus (LE) specific skin disease have been described in the literature. Many of the responsible medications are also known for their ability to induce a lichenoid tissue reaction. Aminoquinoline antimalarials are currently the first line of therapy in cutaneous LE specific skin disease. Lichenoid tissue reactions are among the most common cutaneous side effects of aminoquinolone antimalarials. We report three cases of aminoquinolone antimalarial induced or exacerbated LE specific skin disease. We also review the pathophysiology of LE specific skin disease and propose a mechanism by which induction of the lichenoid tissue reaction may result in Koebnerization of LE specific skin lesions.

Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus and the spectrum of the acute syndrome of apoptotic pan-epidermolysis (ASAP): a case report, concept review and proposal for new classification of lupus erythematosus vesiculobullous skin lesions.

The acute clinical syndrome of toxic epidermal necrolysis (TEN) is currently thought to be a distinct clinical-pathological entity typically resulting from drug hypersensitivity. We describe an adult woman who experienced a fulminate pattern of apoptotic epidermal cell injury following tanning bed exposure while taking naproxen that resulted in a clinical presentation having combined features of drug-induced TEN and an infrequently recognized form of bullous cutaneous lupus erythematosus (LE). This case calls attention to the fact that TEN-like injury can occasionally be seen in settings other than drug hypersensitivity (e.g., LE, acute graft versus host disease) and illustrates the need for a unifying concept in this area. We therefore propose the term 'Acute Syndrome of Apoptotic Pan-Epidermolysis (ASAP)' to designate a clinical syndrome that is characterized by life-threatening acute and massive cleavage of the epidermis resulting from hyperacute apoptotic injury of the epidermis. We also review vesiculobullous skin disorders that can be encountered in LE patients and suggest a new classification scheme for such lesions.

Trauma-induced simulator of targetoid hemosiderotic hemangioma.

Reported here is a 15-year-old with lesions demonstrating histologic features of targetoid hemosiderotic hemangioma (THH) developing after trauma to inflammatory lesions. These lesions pose as simulators of THH. Targetoid hemosiderotic hemangioma is a benign vascular lesion first described by Santa Cruz and Aaronburg. It classically presents as a single, small, red/brown, targetoid lesion on the trunk or extremities of a young or middle-aged individual. Histologically, it is characterized by ectatic vascular lumina in the papillary dermis lined by a single layer of endothelial cells with an epithelioid or "hobnail" appearance. In the deeper dermis, vascular spaces become slit-like and angulated, appearing to dissect through collagen bundles. A commonly proposed etiology of THH is trauma to a preexisting hemangioma. This case is remarkable for its unusual clinical presentation, histologic simulation of THH, and for its support for the theory that trauma can induce the histologic changes seen in THH.

Treatment of atrophoderma of Pasini and Pierini-associated hyperpigmentation with the Q-switched alexandrite laser: a clinical, histologic, and ultrastructural appraisal.

BACKGROUND AND OBJECTIVE: Atrophoderma of Pasini and Pierini (APP) is an uncommon cutaneous disorder, with no known effective treatment, manifested by hyperpigmented patches that appear to be depressed compared with surrounding skin. This study investigated the effectiveness of the Q-switched alexandrite laser on a patient with extensive APP, and evaluated histopathologic and ultrastructural changes. STUDY DESIGN/MATERIALS AND METHODS: A man with stable APP underwent Q-switched alexandrite laser treatment to a patch on the trunk. Biopsies were obtained from treated and untreated sites of involvement. Light and transmission electron microscopic evaluation was performed to investigate melanosome number, size, and volume, as well as melanin granule number and size. RESULTS: After three treatment sessions, the treated area showed marked clinical improvement. Electron microscopy showed a 19% reduction in melanin granule number and size and a 65% reduction in melanosome number, size, and volume in larger melanosomes in treated compared with untreated sites. CONCLUSION: Treatment of APP with the Q-switched alexandrite laser results in clinical improvement. Electron microscopic evaluation suggests that the mechanism may be a reduction in the number, size, and volume of larger melanosomes as well as a decrement in melanin granule number and size.

Update on selected viral exanthems.

Viral exanthems are common in childhood and account for a large number of patient visits to pediatric or family medicine clinics. Most exanthems are virtually harmless to the healthy child, but others can be signs of more significant systemic disease. Some exanthems that are benign or self-limited in the healthy child may propose significant risk to pregnant or immunocompromised individuals. Therefore, recognition of exanthems, which may be associated with certain viral illnesses, is important for the primary care provider. For example, prompt recognition of a viral exanthem caused by parvovirus may allow a pregnant female from exposing her fetus to a potentially fatal infection, or, if the exposure has already occurred, may indicate the need for appropriate fetal monitoring. In this manuscript, we review the recent literature pertaining to four characteristic exanthems that are thought to be viral in nature: papular purpuric gloves and socks syndrome; pityriasis rosea; unilateral lateral thoracic exanthem; and Gianotti-Crosti syndrome.

Erythematous plaques due to platelet plugging: a clue to underlying myeloproliferative disorder.

We report a syndrome in a middle-aged woman characterized by tender erythematous plaques with histologic evidence of dramatic dermal vessel occlusion. These cutaneous findings occurred in association with progressive inferior vena cava and portal vein thrombosis while on coumarin anticoagulation, following hepatic transplantation for Budd-Chiari syndrome. The material occluding dermal vessels was proven by immunohistochemical staining to be platelet plugs. These findings led to the diagnosis of an underlying myeloproliferative disorder explaining both her cutaneous and liver abnormalities and institution of appropriate platelet directed anticoagulation with aspirin.

An unusual hematopoietic proliferation seen in a nevus sebaceous.

Nevus sebaceous is a tumor of primary epithelial germ cells that is generally first noticed at birth. It is relatively quiescent until puberty when a variety of benign and potentially malignant tumors may arise within it. We describe a nevus sebaceous with immature fat cells, vascular ectasia, and extramedullary hematopoiesis that was present at birth. At 4 months, the immature adipose and hematopoietic elements, as well as the vascular changes, had resolved.

High perimeter impedance defibrillation electrodes reduce skin burns in transthoracic cardioversion.

During cardioversion, skin burns result from current preferentially flowing at the electrode edge. We tested new electrodes with high perimeter impedance to yield more uniform current distribution; these electrodes reduced histopathologic skin injury.

Cutaneous parachordoma. A light microscopic and immunohistochemical report of two cases and review of the literature.

Parachordomas are rare cutaneous tumors that show virtually identical histologic findings to chordomas. Therefore, the major differential diagnosis in a case of parchordoma is metastatic chordoma. Parachordomas are benign neoplasms and most often develop on the extremities adjacent to tendons, synovium or osseous structures, as opposed to chordomas, which are malignant tumors located along the craniospinal axis. While recurrences may occur in cases of parachordoma, metastases have not been reported. In this report, two cases of parachordomas are reported and the literature reviewed. By light microscopy, parachordomas show eosinophilic bands of fibrous tissue separating lobules of cells with variably vacuolated cytoplasm (physaliphorous cells) admixed with more epithelioid cells in a myxoid stroma. Parachordomas and chordomas share immunohistochemical and ultrastructural features. Both stain with S-100 protein and vimentin, and ultrastructurally both demonstrate cytoplasmic vacuoles, intermediate filaments, pinocytotic vesicles, celljunctions, and cytoplasmic membranes with microvillous processes. Chordomas more frequently express cytokeratin (98% vs. 66% in parachordomas) and epithelial membrane antigen (90% vs. 20% in parachordomas) and chordomas have a larger number of rough endoplasmic reticulum-mitochondrial complexes. Thus, positive staining with epithelial membrane antigen and the identification of a large number of rough endoplasmic reticulum-mitochondrial complexes are suggestive of metastatic chordoma. However, the definitive distinction remains a clinical one after appropriate radiologic studies of the skull and spinal chord.

Subacute radiation dermatitis from fluoroscopy during coronary artery stenting: evidence for cytotoxic lymphocyte mediated apoptosis.

A patient with fluoroscopy-induced subacute radiation dermatitis is described. Her biopsy specimen showed vacuolar change and necrotic keratinocytes with lymphocytes in direct apposition, or "satellitosis". Lymphocytes in the dermis and infiltrating the epidermis were predominantly CD8+ and a number of the lymphocytes stained for TIA-1, a cytotoxic granule protein in T-cells and natural killer cells, that appears to be involved in the induction of apoptosis. Our findings suggest cytotoxic lymphocyte mediated apoptosis is involved in the pathogenesis of subacute radiation dermatitis.

Nature and determinants of skin "burns" after transthoracic cardioversion.

Skin biopsies obtained 24 hours after elective cardioversion of 30 patients showed variable epidermal necrosis and upper dermal perivascular inflammation, most noticeably in patients receiving high individual peak (> or = 300 J) and cumulative (> or = 350 J) shock energies. Thus, damped sine wave shocks cause skin injury--first degree burns--the severity of which is a function of peak and cumulative shock energy.