Hypocomplementemic urticarial vasculitis: report of a 12-year-old girl with systemic lupus erythematosus

Johns Hopkins Medical Institutions and the Department of Dermatology, Johns Hopkins Hospital, Baltimore, MD, USA.

Journal of the American Academy of Dermatology (Impact Factor: 5). 12/2002; 47(5 Suppl):S273-4. DOI: 10.1067/mjd.2002.108586

Authors: Tracy DeAmicis, Mona Mofid, Bernard Cohen, Hossein C Nousari

ABSTRACT Urticarial vasculitis, a form of leukocytoclastic vasculitis involving the postcapillary venules, is classified as a type III hypersensitivity reaction and has been associated with connective tissue disease. The lesions resemble urticaria and typically persist for more than 24 hours. Urticarial vasculitis usually affects young women, and the diagnosis is confirmed at histologic examination. Patients with urticarial vasculitis can be divided into 2 types--those with normal complement levels and those with hypocomplementemic urticarial vasculitis (HUV). Patients with normocomplementemic urticarial vasculitis have a milder course than do patients with HUV, a condition that has a strong association with systemic lupus erythematosus. Angioedema, ocular inflammation, obstructive lung disease, and glomerulonephritis are commonly associated with HUV. We describe the case of a girl with systemic lupus erythematosus and HUV who also had pancreatitis, hypothyroidism, and elevated levels of antiphospholipid antibodies.  

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Primary hyperoxaluria: Report of a patient with livedo reticularis and digital infarcts

Department of Dermatology, Johns Hopkins Hospital, Baltimore, MD, USA.

Journal of the American Academy of Dermatology (Impact Factor: 5). 03/2002; 46(2 Suppl Case Reports):S16-8. DOI: 10.1016/S0190-9622(02)70223-5           

Authors: Vincent Marconi, BS, Mona Mofid, MD, Caroline McCall, MD, Israel Eckman, MD, Hossein C. Nousari, MD

ABSTRACT Primary hyperoxaluria encompasses 3 rare genetic disorders of glyoxylate metabolism characterized by excessive urinary excretion of oxalic acid, resulting in oxalosis. Patients typically have recurrent calcium oxalate nephrolithiasis and nephrocalcinosis, leading to chronic renal failure and death from uremia. Oxalate can deposit in extrarenal sites such as the heart, walls of arteries and veins, bone, and skin. We report a patient who presented with acute renal failure and later experienced livedo reticularis and peripheral gangrene before the diagnosis of primary hyperoxaluria was established. A skin biopsy specimen demonstrated numerous characteristic elongate to diamond-shaped, radially oriented, pale yellow translucent oxalate crystals within the vessels, and vessel walls of the subcutaneous fat that were strongly birefringent under polarized light. 

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Drug-induced linear immunoglobulin A bullous disease that clinically mimics toxic epidermal necrolysis

Department of Dermatology, The Johns Hopkins Medical Institutions, Baltimore, Maryland 21205, USA.

Journal of Burn Care & Rehabilitation (Impact Factor: 2.42). 01/2000; 21(3):246-7. DOI: 10.1067/mbc.2000.106394           

Authors: Mofid MZ, Costarangos C, Bernstein B, Wong L, Munster A,

ABSTRACT Drug-induced linear immunoglobulin A bullous disease is a subepidermal blistering disorder that most commonly occurs after exposure to vancomycin. It can clinically mimic toxic epidermolytic necrolysis. We describe an 87-year-old white woman in whom linear immunoglobulin A bullous disease developed while she was taking vancomycin and phenytoin. A few days after the linear immunoglobulin A bullous disease developed, both medications were discontinued. No new bullae developed, and the eruption completely resolved within 2 weeks. The patient was treated with only topical therapy. 

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Topical Immunotherapy: What's New

Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, MD 21205-0900, USA.

Dermatologic Clinics (Impact Factor: 1.43). 05/2005; 23(2):245-58. DOI: 10.1016/j.det.2004.08.002

Authors: Daniel N Sauder, Mona Mofid

ABSTRACT Further understanding of the pathogenesis of dermatologic conditions at a molecular level has led to targeted therapies. The topical immune response modifiers have contributed significantly to the treatment of cutaneous diseases. New topical remedies, particularly the Toll-like receptor agonists and calcineurin inhibitors, have added to the clinical armamentarium and have further advanced clinicians' ability to treat a wide variety of benign, premalignant, and malignant conditions. Furthermore, these agents have contributed to the understanding of the disease process. The next decade will witness even greater advances in targeted immunotherapies for dermatologic disease. 

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Hereditary epidermolytic palmoplantar keratoderma (Vörner type) in a family with Ehlers-Danlos syndrome

Department of Dermatology, The Johns Hopkins Hospital, Baltimore, Maryland, USA.

Journal of the American Academy of Dermatology (Impact Factor: 5). 06/1998; 38(5 Pt 2):825-30. DOI: 10.1016/S0190-9622(98)70467-0

Authors: Mona Zohdi Mofid, MD, Constantino Costarangos, MD, Stephen B. Gruber, MD, PhD, Susan E. Koch, MD

ABSTRACT We describe a kindred in whom epidermolytic palmoplantar keratoderma occurred in association with Ehlers-Danlos syndrome type III (benign hypermobility syndrome). This kindred consisted of 27 members of four generations, 14 of whom had palmoplantar keratoderma (PPK). Of those who had palmoplantar keratoderma, 6 had Ehlers-Danlos type III (EDS II). The proband presented with diffuse, symmetrical hyperkeratotic plaques that were yellow and sharply demarcated, covering the entire palms and soles, in addition to marked large and small joint flexibility and skin hyperextensibility. A biopsy specimen from the palm revealed features of epidermolytic hyperkeratosis with acanthosis. To our knowledge, this is the first report of PPK in a family with Ehlers-Danlos syndrome. Linkage analysis of these two clinical traits showed that the genes responsible for PPK and EDS III are not closely linked, and therefore are not immediately adjacent. However, linkage at greater genetic distances could not be excluded. 

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Bullous lesions in scleroderma

Department of Dermatology, of Johns Hopkins University, School of Medicine, Baltimore, MD, USA.

International Journal of Dermatology (Impact Factor: 1.23). 07/2002; 41(6):335-9. DOI: 10.1046/j.1365-4362.2002.01360.x

Authors: Adrienne Rencic MD, PhD, Supriya Goyal MD, Mona Mofid MD, Frederick Wigley MD and H. Carlos Nousari MD

ABSTRACT The occurrence of bullous lesions in localized or systemic scleroderma is rare. Three histologic patterns have been reported: lichen sclerosus et atrophicus-like, lymphangiectatic blisters and autoimmune blistering diseases.
To investigate the frequency, clinical, and immunopathologic features of patients with scleroderma and bullous eruptions and to review the literature regarding this rare condition.
A retrospective study of 53 cases of scleroderma (localized, generalized, and systemic) in the dermatology and rheumatology clinics at one institution over an 8-year span. Clinical, serologic, and immunopathologic findings were analyzed in four cases.
Four of 53 patients exhibited bullous lesions in association with scleroderma. The first case illustrates lymphangioma-like clinical and pathologic presentation. The second case demonstrates bullous lichen sclerosus et atrophicus-like pattern. The other two cases exemplify a superimposed autoimmune skin disease, epidermolysis bullosa acquisita and penicillamine induced pemphigus foliaceus after treatment for systemic scleroderma.
Of the 53 original patients, we have described four cases of bullous scleroderma (7.5%) Illustrating several pathogenetic mechanisms of bulla formation. inflammatory (lichen sclerosus et atrophicus), fibrotic/obstructive (lymphangiomatous), autoimmune (epidermolysis bullosa acquisita), and pemphigus foliaceus. The final case illustrates bullae as a complication of therapy for the underlying scleroderma. 

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