Medication Summary
No specific uniformly effective therapy for Weber-Christian disease is recognized. Therapeutic responses have been reported using fibrinolytic agents, hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, mycophenolate, and clofazimine. Systemic steroids (eg, prednisone) may be effective in suppressing acute exacerbations. [10, 20, 21, 22, 23] Nonsteroidal anti-inflammatory agents (eg, indomethacin) may reduce fever, arthralgias, and other signs of malaise. Involvement of specific organs may require specific supportive drugs.
Corticosteroids
Class Summary
These agents are used for suppression of acute inflammatory exacerbations. These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.
Prednisone (Deltasone, Sterapred)
May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
Immunomodulators
Class Summary
These agents inhibit key factors that mediate immune reactions, which, in turn, decrease inflammatory responses. They may have potential long-term therapeutic response.
Azathioprine (Imuran)
Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.
Cyclosporine (Neoral, Sandimmune)
Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions (eg, delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, graft versus host disease) for a variety of organs. For children and adults, base dosing on ideal body weight. Demonstrated to be helpful in variety of skin disorders.
Cyclophosphamide (Neosar, Cytoxan)
Chemically related to nitrogen mustards. As an alkylating agent, mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells. Demonstrated to be helpful in various skin disorders.
Mycophenolate (CellCept)
Inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. Inhibits antibody production.
Hydroxychloroquine (Plaquenil)
Inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.
Thalidomide (Thalomid)
Immunomodulatory agent that may suppress excessive production of TNF-alpha and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration.
Antibiotics
Class Summary
Several antibiotics (eg, tetracycline, clofazimine) are used for their anti-inflammatory activity.
Tetracycline (Sumycin)
Treats gram-positive and gram-negative organisms, as well as mycoplasmal, chlamydial, and rickettsial infections. Inhibits bacterial protein synthesis by binding with 30S and possibly 50S ribosomal subunit(s).
Clofazimine (Lamprene)
Clofazimine is a lipophilic rhimophenazine dye that inhibits template function of DNA by binding to it. It is weakly bactericidal and has anti-inflammatory effects. Although the mechanism of action is unclear, it seems to exert its main effect on neutrophils and monocytes in various ways (eg, stimulating phagocytosis and release of lysosomal enzymes). Clofazimine is indicated for panniculitis of mycobacterial infections. It has been used in other inflammatory dermatoses but with unknown efficacy.
Use of clofazimine for the treatment of any disease other than leprosy is discouraged by the WHO and the manufacturer since indiscriminate use may promote emergence of resistant strains of M leprae.
Nonsteroidal anti-inflammatory drugs
Class Summary
These agents may reduce fever, arthralgia, and pain.
Indomethacin (Indocin)
Rapidly absorbed; metabolism occurs in liver by demethylation, deacetylation, and glucuronide conjugation. Inhibits prostaglandin synthesis.
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Lesion of erythema nodosum: tender, erythematous, nodular lesions located over the extensor surfaces of the legs.
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Standard posteroanterior chest radiograph reveals extensive bilateral hilar and mediastinal lymph node enlargement not associated with a pulmonary abnormality in a patient with sarcoidosis.
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Young male patient with fever and cough has a focal opacity in the left lower lobe that looks like a pneumonia. This is a case of primary tuberculosis.
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Lesion of Weber-Christian disease: tender, erythematous, nodular lesions located over the limbs with cutaneous atrophy.
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A portion of skin is examined in multiple sections and at various magnifications. The epidermis is intact; however, it is infiltrated by small numbers of lymphocytes. A mild infiltrate of lymphocytes and histiocytes are present in the upper dermis. The most prominent change is in the subcutaneous tissue, where a prominent infiltrate of histiocytes, smaller numbers of lymphocytes, and a few plasma cells in the subcutaneous adipose tissue are noted. Occasional foam cells are also evident, and, in places, histocytes surround lipid cysts. Small clusters of necrotic cells and scattered nuclear dust are noted. Minimal extension of this infiltrate into adjacent dense collagenous tissue is observed. (Courtesy of Milton J. Finegold, MD, Professor of Pathology and Pediatrics, Baylor College of Medicine, Houston, TX).
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Magnification of previous specimen X 100.
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Magnification of previous specimen X 200.
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Histopathologic features of alpha-1-antitrypsin deficiency panniculitis. (A) Scanning power shows a mostly lobular panniculitis. (B) Aggregations of neutrophils within the fat lobule are seen. (C) Neutrophils are interstitially arranged between collagen bundles of the deep reticular dermis. (A-C, hematoxylin-eosin stain; original magnifications: A, X 20; B, X 400; C, X 200).
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Histopathologic features of late stage lesions of traumatic panniculitis. This lesion corresponds to the so-called nodular cystic fat necrosis or mobile encapsulated lipoma. A, Scanning power shows encapsulated and well-circumscribed lesion with no inflammatory infiltrate (arrow indicates area enlarged in B). B, At periphery of the lesion necrotic adipocytes appear as anucleated fat cells. (A and B, Hematoxylin-eosin stain; original magnifications: A, ×20; B, ×200.)
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Histopathologic features of paraffinoma. A, Scanning power shows a mostly lobular panniculitis (arrow indicates area enlarged in B). B, Higher magnification demonstrates cystic spaces within the fat lobule surrounded by foamy histiocytes. (A and B, hematoxylin-eosin stain; original magnifications: A, ×20; B, ×200.)
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Histopathologic features of subcutaneous fat necrosis of the newborn. (A) Scanning power shows a mostly lobular panniculitis (arrow indicates area enlarged in B). (B) Higher magnification demonstrated narrow needle-shaped clefts radially arranged and surrounded by histiocytes. (A and B, hematoxylin-eosin stain; original magnifications: A, X 20; B, X 200).
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Histopathologic features of lipoatrophy secondary to subcutaneous injections of corticosteroids. (A) Low-power view showed small fat lobules (arrow indicates area enlarged in B). (B) Higher magnification demonstrates small adipocytes and prominent capillary proliferation, resembling embryonic fat. (A and B, hematoxylin-eosin stain; original magnifications: A, X 20; B, X 200).
