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Biomedical subjects

J P Callen

Publications and source records attributed to J P Callen.

At least 37 records · Page 2Linked to original sources

Drug-induced linear IgA bullous dermatosis after vancomycin discontinuance in a patient with renal insufficiency.

Linear IgA bullous dermatosis (LABD) is an autoimmune, subepidermal, vesiculobullous disease that has been commonly associated with the use of vancomycin hydrochloride. Lesions typically appear during vancomycin therapy, 24 hours to 15 days after the first dose. A 65-year-old white man with renal insufficiency developed pruritic, tense bullae on the right chest, right medial arm, right flank, abdomen, and right upper thigh 14 days after his last dose of vancomycin. Histopathologic examination and immunofluorescence studies were diagnostic of LABD. Vancomycin-related LABD may appear as long as 2 weeks after the drug is discontinued.

Aged↗

New and emerging therapies for collagen-vascular diseases.

Cutaneous disease is frequent in many of the rheumatic disorders. Some of these disorders such as lupus erythematosus, dermatomyositic, scleroderma, and vasculitis are defined by their cutaneous manifestations. As investigation increases our understanding of these disorders, available therapy becomes more logical and less empiric. This article deals with some of the newer therapies for these disorders.

Dermatomyositis↗

Cavitating pulmonary infiltrate in an adolescent with pyoderma gangrenosum: a rarely recognized extracutaneous manifestation of a neutrophilic dermatosis.

Neutrophilic dermatoses such as pyoderma gangrenosum are characterized by sterile, neutrophilic cutaneous infiltrates. Extracutaneous neutrophilic infiltrates can occur, primarily in the joints, lungs, heart, central nervous system, gastrointestinal tract, and eyes. Pulmonary disease is the most frequently reported extracutaneous manifestation of pyoderma gangrenosum and is characterized by patchy infiltrates or interstitial pneumonitis. We describe an adolescent with typical pyoderma gangrenosum who presented with cavitary pneumonia and responded completely to oral corticosteroids. In patients with inflammatory ulcers, extracutaneous neutrophilic disease should be considered, once an infectious process has been excluded.

Adolescent↗

Adult-onset recalcitrant eczema: a marker of noncutaneous lymphoma or leukemia.

BACKGROUND: Generalized eczema or erythroderma may be the presenting sign of cutaneous T-cell lymphoma. Additionally, intractable pruritus has been associated with Hodgkin's lymphoma. However, reports of adult-onset eczematous dermatitis has rarely been linked to noncutaneous lymphoproliferative disorders. OBSERVATIONS: We observed one patient in 1993 who had the onset of intractable dermatitis characterized by prurigo nodularis-like lesions and widespread erythematous plaques. After 18 months of cutaneous symptoms he experienced dyspnea. At this time Hodgkin's disease was diagnosed. This observation prompted us to evaluate subsequent patients with adult-onset eczema who were poorly responsive to therapy and in whom an obvious cause could not be determined. Over the next 24 months we identified an additional 2 patients with lymphoma who met this criteria. CONCLUSION: Unexplained eczema of adult onset may be associated with an underlying lymphoproliferative malignancy. When a readily identifiable cause (eg, contactants, drugs, or atopy) is not found, a systematic evaluation should be pursued. Patients should be evaluated with a careful physical examination, complete blood cell counts, peripheral blood smears, chest roentgenography, computed tomography of the chest and abdomen, and serum protein electrophoresis.

Adult↗

Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions.

Pyoderma gangrenosum (PG) is an idiopathic, inflammatory, ulcerative disease of undetermined cause. The diagnosis is based on clinical and pathologic features and requires exclusion of conditions that produce ulcerations. An atypical bullous variant (atypical pyoderma gangrenosum, APG) exists with clinical features similar to those of Sweet syndrome. Because PG is a rare disease, few large case series have been reported. Pyoderma gangrenosum was first recognized as a unique disease entity in the first half of the 20th century. Cumulative knowledge of PG is based on a handful of case series and multiple individual case reports. To augment that knowledge, we present our experience with a large number of patients over a significant time. We performed a retrospective analysis of the medical records of 86 patients with PG who were evaluated and treated over 12 years at 2 university-based dermatology departments. The mean (+/- standard deviation) age of onset of PG and APG, respectively, was 44.6 +/- 19.7 years and 52.2 +/- 15.3 years. Lower extremity involvement was most common in PG, whereas upper extremity involvement was most common in APG. Associated relevant systemic diseases were seen in 50% of patients. Inflammatory bowel disease was the most common association in patients with PG, whereas hematologic disease or malignancy was most common in those with APG. Although a few patients were managed with local measures or nonimmunosuppressive treatment, the majority required oral corticosteroid therapy, often with systemic immunosuppressive treatment. PG patients required a mean 11.5 +/- 11.1 months of treatment to achieve remission compared with 9.0 +/- 13.7 months for patients with APG. Five patients (5.8%) had disease that was extremely refractory to multiple intensive therapies. The prognosis and disease associations for PG and APG appear to be different. Compared with PG, APG is more often associated with hematologic disease or malignancy, and remits more quickly.

Adolescent↗

Peripheral ulcerative keratitis--an extracutaneous neutrophilic disorder: report of a patient with rheumatoid arthritis, pustular vasculitis, pyoderma gangrenosum, and Sweet's syndrome with an excellent response to cyclosporine therapy.

The term peripheral ulcerative keratitis represents a spectrum of inflammatory diseases, characterized by cellular infiltration, corneal thinning, and ulceration. Neutrophilic dermatoses are rarely associated with peripheral ulcerative keratitis. To date, peripheral ulcerative keratitis has only been reported in patients with pyoderma gangrenosum. Separate episodes of pyoderma gangrenosum, Sweet's syndrome, and pustular vasculitis developed in a 60-year-old patient with rheumatoid arthritis over an 8-year period. Over the past 2 years, 3 episodes of peripheral ulcerative keratitis occurred. Cyclosporine (4 mg/kg/d) treatment was started on confirmation of pyoderma gangrenosum. Over the ensuing 2 years, it became evident that the activity of her ocular and skin diseases, as well as her arthritis, paralleled the administration or cessation of cyclosporine therapy. Dermatologists should be aware of the association of Sweet's syndrome, pyoderma gangrenosum, and pustular vasculitis with peripheral ulcerative keratitis. This rare ocular manifestation and the serious sequelae when left untreated make recognition crucial. Cyclosporine proved to be a very effective treatment for all of our patient's diseases.

Arthritis, Rheumatoid↗

Photosensitivity in collagen vascular diseases.

Clinically, photosensitivity is clearly part of both lupus erythematosus and dermatomyositis, but the prevalence of this reaction varies. Photosensitivity does not appear to be part of the disease spectrum of scleroderma or part of the clinical picture of vasculitis. Implications of photosensitivity in both lupus erythematosus and dermatomyositis are systemic. The pathogenesis of photosensitivity in lupus erythematosus and dermatomyositis is not fully understood. The presence of photosensitivity has therapeutic implications.

Dermatomyositis↗

Autoantibodies in lichen planus pemphigoides react with a novel epitope within the C-terminal NC16A domain of BP180.

Lichen planus pemphigoides is an autoimmune subepidermal blistering disease. The finding of immunoglobulin G antibodies directed against the basement membrane zone differentiates it from bullous lichen planus. The aim of this study was to identify the target antigen of lichen planus pemphigoides autoantibodies. Sera from lichen planus pemphigoides patients (n = 4) stained the epidermal side of NaCl-split human skin in a pattern indistinguishable from that produced by bullous pemphigoid sera. In bullous pemphigoid, the autoimmune response is directed against BP180, a hemidesmosomal transmembrane collagenous glycoprotein. We previously demonstrated that bullous pemphigoid sera predominantly react with a set of four epitopes (MCW-0 through MCW-3) clustered within a 45 amino acid stretch of the major noncollagenous extracellular domain (NC16A) of BP180. By immunoblotting and enzyme-linked immunosorbent assay, lichen planus pemphigoides sera were also strongly reactive with recombinant bullous pemphigoid 180 NC16A. The lichen planus pemphigoides epitopes were further mapped using a series of overlapping recombinant segments of the NC16A domain. All lichen planus pemphigoides sera reacted with amino acids 46-59 of domain NC16A, a protein segment that was previously shown to be unreactive with bullous pemphigoid sera. Two lichen planus pemphigoides sera, in addition, reacted with the immunodominant antigenic region associated with bullous pemphigoid. In conclusion, there are now five bullous diseases that are associated with an autoimmune response to BP180: bullous pemphigoid; pemphigoid/herpes gestationis; cicatricial pemphigoid; linear immunoglobulin A disease; and lichen planus pemphigoides. In addition, we have identified a novel epitope within the BP180 NC16A domain, designated MCW-4, that appears to be uniquely recognized by sera from patients with lichen planus pemphigoides.

Adult↗

Necrotizing vasculitis of the skin and uterine cervix associated with minocycline therapy for acne vulgaris.

In recent years, minocycline has become a commonly used agent for the treatment of acne vulgaris and rosacea. With this increased use have come reports of severe and in some cases life-threatening toxicity, often occurring in otherwise healthy young women after prolonged courses of minocycline. These adverse reactions include hepatotoxicity, drug-induced lupus erythematosus, eosinophilic pneumonitis, and hypersensitivity syndrome. We describe a 35-year-old woman who had necrotizing vasculitis of the skin and uterine cervix after 2 years of minocycline therapy for acne vulgaris. Skin and cervical biopsies revealed acute inflammation involving through-and-through necrosis of vessel walls with thrombosis, focal fibrinoid change, and a perivascular lymphohistiocytic infiltrate. The disease fully resolved within 3 months of discontinuance of the minocycline therapy. Patients should be informed of these rare but potentially serious adverse effects before the initiation of minocycline therapy. Early recognition of these complications can result in complete resolution.

Acne Vulgaris↗

A case of congenital multiple myofibromatosis developing in an infant.

BACKGROUND: Infantile myofibromatosis is marked by the development of firm, discrete, flesh-colored to purple nodules in skin, muscle, bone, and/or subcutaneous tissues. In cases without visceral involvement, the prognosis is excellent with expected spontaneous regression of nodules in 1 to 2 years. Visceral lesions are associated with significant morbidity and mortality generally within the first few months of life secondary to obstruction of a vital organ, failure to thrive, or infection. OBSERVATION: We present a case of congenital myofibromatosis initially presenting as a single, asymptomatic nodule on the midback of an otherwise healthy 1-month-old white boy. Over the subsequent 6 months, the child developed a left-sided head tilt with the formation of additional myofibromas of the skin and musculature, but without visceral involvement. Physical examinations have continued to show age-appropriate growth and development. CONCLUSION: Clinicians should be aware of this rare but potentially life-threatening entity and consider infantile myofibromatosis in the differential diagnosis of pediatric dermal and subcutaneous nodules, particularly when associated with a new onset of head tilt. Close clinical follow-up is recommended in all cases of infantile myofibromatosis.

Humans↗

Atypical presentation of herpes simplex virus in a patient with chronic lymphocytic leukemia.

Perianal infections caused by herpes simplex virus are common in immunocompromised patients. The cutaneous presentation in these patients is often atypical, overlaps with the clinical features of other diseases, poses a difficulty in diagnosis, and responds poorly to treatment. An immunocompromised patient with chronic lymphocytic leukemia, treated with oral corticosteroids, presented with chronic perianal ulcerations. This patient was referred for evaluation and treatment of "recalcitrant" pyoderma gangrenosum. Prompt diagnosis was possible when the clinical features were recognized and appropriate biopsy and cultures were obtained. We describe an atypical presentation of herpes simplex virus associated with both an endogenous and exogenous induced immunodeficiency, and stress the importance of routinely performing cultures on all perianal ulcerations and anal fissures to avoid the misdiagnosis, inappropriate treatment, and prolonged discomfort of these afflicted patients.

Aged↗

Effects of acitretin on the liver.

BACKGROUND: Therapy with aromatic retinoids for psoriasis is associated with abnormal liver function test findings and toxic hepatitis (in 1.5% of patients). OBJECTIVE: Our purpose was to determine the safety of acitretin with respect to liver function, on the basis of biopsy. METHODS: We treated 128 adults (with chronic, stable psoriasis) with oral acitretin (25-75 mg/day) for four 6-month intervals in a prospective, open-label, 2-year multicenter study. Liver biopsies were performed before and after study completion (2 years). RESULTS: Eighty-three available pairs of pretreatment and posttreatment liver biopsies demonstrated no change in 49 patients (59%), improvement in 20 (24%), and worsening in 14 (17%). Of these 14 patients with decrements in biopsy status, most changes were mild. There was no correlation between liver function test abnormalities or cumulative acitretin dose and changes in liver biopsy status. CONCLUSION: Acitretin therapy elicited no biopsy-proven hepatotoxicity in this prospective 2-year study. These findings suggest that periodic liver biopsy may not be necessary with acitretin treatment.

Acitretin↗