Case for diagnosis. Noninfectious suppurative panniculitis induced by mesotherapy with deoxycholate.

A 28-year-old white female patient presented with multiple erythematous-to-violaceous, painful, suppurative nodules on the buttocks and thighs that appeared after two weeks of mesotherapy with deoxycholate, caffeine, sunflower liposomes, and sinetrol for localized fat. She was treated for atypical mycobacteriosis, but with no satisfactory response after antibiotic therapy. Bacterial, mycobacterial, and fungal culture were all negative. Histopathologic examination of the biopsy showed noninfectious suppurative panniculitis. It resolved after treatment with methotrexate, prednisone, and hydroxychloroquine. This report highlights the rarity of this complication, the importance of its early recognition, and differentiation with atypical fast growing mycobacterioses.

Case for diagnosis. Noninfectious suppurative panniculitis induced by mesotherapy with deoxycholate

Abstract A 28-year-old white female patient presented with multiple erythematous-to-violaceous, painful, suppurative nodules on the buttocks and thighs that appeared after two weeks of mesotherapy with deoxycholate, caffeine, sunflower liposomes, and sinetrol for localized fat. She was treated for atypical mycobacteriosis, but with no satisfactory response after antibiotic therapy. Bacterial, mycobacterial, and fungal culture were all negative. Histopathologic examination of the biopsy showed noninfectious suppurative panniculitis. It resolved after treatment with methotrexate, prednisone, and hydroxychloroquine. This report highlights the rarity of this complication, the importance of its early recognition, and differentiation with atypical fast growing mycobacterioses.

[Weber-Christian's disease: a preleukemic disorder?] / La maladie de Weber-Christian: s'agit-il d'un état pré-leucémique?

Weber christian's disease or idiopathic panniculitis is a rare condition characterized by an inflammation of the subcutaneous adipose tissue; it is a non-specific pathological condition that remains a central issue of debate and whose outcome is unpredictable. We here report the case of a 9-month-old female infant admitted in the hospital with sepsis preceded by respiratory symptoms and followed by the occurrence of small, hard, susceptible, asymmetrical erythematous subcutaneous nodes located in the arms and lower limbs. Cutaneous biopsy showed lobular hypodermitis with polymorphonuclear neutrophil infiltrations. The diagnosis of Weber Christian's disease was retained after eliminating other differential diagnoses. The patient was treated with prednisone with good outcome; during the follow-up period and during disease regression the diagnosis of B-cell acute lymphoblastic leukemia was made; this uncommon outcome has never been described in the literature. The aim of this study is to discuss any similar situation where another diagnosis was made in patients initially treated for idiopathic lobular panniculitis. This highlights the need for exhaustive etiological investigations and prolonged monitoring in order to search for a possible associated disorder.

Mycobacterium chelonae infection presenting as recurrent cutaneous and subcutaneous nodules--a presentation previously diagnosed as Weber Christian disease.

Although the dermatologic community rarely uses "Weber-Christian Disease" as a diagnosis, it still appears in the internal medicine literature. Herein we present a patient with recurrent cutaneous and subcutaneous nodules who was initially treated with aggressive immunosupression for a presumptive diagnosis of Weber-Christian Disease. After more than a decade the patient was diagnosed with cutaneous Mycobacterium chelonea. This case is an excellent example of the difficulty in diagnosing mycobacterial infections and underscores the importance of having a high suspicion for infectious etiologies for unresponsive cutaneous eruptions in patients on immunosuppressive medications.

An unreported case of pancreatic panniculitis in a liver transplant patient.

Pancreatic panniculitis is an uncommon condition that can occur in association with pancreatic disease. Most of the cases reported to date were associated with acute or chronic pancreatitis and pancreas cancer. Recently, development has been described in kidney transplant patients and secondarily to allograft pancreatitis in a pancreas-kidney transplant recipient. Both findings suggest that immunological processes may be involved in the pathogenesis of this entity. We report for the first time a case of acute pancreatitis associated with pancreatic panniculitis in a patient who underwent a liver transplant 10 months before. A 69-year-old man with a history of epigastric pain of a few days of evolution was presented with painful subcutaneous nodules on both legs. Blood chemistry showed raised serum amylase and lipase levels. Ultrasonography and multislice CT scan were suggestive of an acute pancreatitis. A skin biopsy showed typical features of pancreatic panniculitis which included lobular panniculitis with lipocyte degeneration with ghost cells. The administration of octreotide resulted in both a rapid improvement of symptoms and a disappearance of skin lesions. Liver transplant specialists should be aware that the pancreatic panniculitis could be a manifestation ofpancreas disease in patients who have undergone l ver transplantation.

Panniculitis in children.

This paper will give a comprehensive view of the most frequent panniculitides seen in childhood, with emphasis on the types exclusively found in infancy, and for all other types of panniculitides also found in adults. Aim of this paper is also to analyze the clinical differences between panniculitis in childhood and in adulthood, and to give reliable histopathologic criteria for a specific diagnosis. A review of the literature is here integrated by authors' personal contribution. Panniculitides in children is a heterogeneous group of diseases, as well as in adult life, characterized by inflammation of the subcutaneous fat. Only very few types of panniculitis are exclusively found in childhood, such as Sclerema neonatorum and subcutaneous fat necrosis of the newborn, while the vast majority of the other types may be found both in paediatric age and in adults. Furthermore, this paper will consider in detail panniculitis according to their frequency, such as Erythema nodosum, Lupus panniculitis, Cold panniculitis, panniculitis in Behçet disease, and poststeroid panniculitis. It will also describe rare forms of panniculitis, such as Eosinophilic panniculitis (a pathological entity debated by many authors), Subcutaneous panniculitis T-cell lymphoma, and the different forms of the so call "Lipophagic panniculitis", encompassing respectively the febrile relapsing panniculitis of Weber-Christian disease and the non-relapsing form of Rothmann-Makai disease. For each type of panniculitis considered concise information will be given about epidemiology, etiology, clinical findings, laboratory data, prognosis and therapy, while histopathologic findings will be described in detail.

Panniculitis with vasculitis.

Panniculitides encompass a great number of different entities; however, once a vasculitis has been detected histopathologically within the subcutaneous tissue, the differential diagnosis is mainly restricted to polyarteritis (panarteritis) nodosa (PAN), nodular vasculitis (NV), and Bazin's erythema induratum (EI). Patients with PAN may have the disease confined to the skin, but must be followed over a long period because many of them develop late systemic disease. The NV/EI group represents by far the most common type of lobular panniculitis with vasculitis; we prefer keeping the distinction between the two entities by underlining the equation NV positive tuberculin skin test = EI. Other lobular panniculitides with vasculitis are exceedingly rare and set in a systemic background which can be infectious (lepromatous leprosy panniculitides) or autoimmune/dysreactive (neutrophilic lobular panniculitis in rheumatoid arthritis, lobular panniculitis in inflammatory bowel disease).

Weber-Christian disease associated with the tip variant of focal segmental glomerulosclerosis: a case report.

Weber-Christian disease (W-CD) is associated with relapsing nodular panniculitis and a variety of systemic findings. Renal parenchymal involvement has been rarely reported. The authors describe a case of nephrotic syndrome in an African-American man with a W-CD flare. The patient had chills and low-grade fever with painful lower extremity skin lesions. A renal biopsy demonstrated the tip variant of focal segmental glomerulosclerosis (FSGS). The kidney biopsy also suggested parenchymal involvement by W-CD disease, with supportive ultrastructural findings. The synchronous W-CD flare and biopsy-proven FSGS and the rapid and sustained response of both to limited treatment suggest a causative association.

[Lipoatrophic panniculitis in adults: treatment with hydroxychloroquine]. / Panniculite lipoatrophiante de l'adulte : traitement par hydroxychloroquine.

BACKGROUND: Lipoatrophic panniculitis is generally considered to be a rare disease affecting children. We report a case involving this condition in an adult patient presenting with striking clinical features and responding to hydroxychloroquine therapy. We discuss the nosological relationship between lipoatrophic panniculitis and connective tissue panniculitis. PATIENTS AND METHODS: A 62-year-old woman was referred to our institution with a six-month history of painful erythematous nodules and plaques on the calves, thighs, buttocks, breasts, abdomen and arms. With each outbreak of new lesions, she felt unwell and experienced fever, chills and sweating. After a few weeks, the lesions progressed circumferentially and led to large areas of subcutaneous atrophy showing a central depression covered with a yellowish, supple skin and surrounded by an annular, infiltrated, erythematous and tender inflammatory margin. Deep subcutaneous biopsy specimens showed typical features of lobular and septal lipophagic panniculitis, with a dense inflammatory infiltrate composed of large histiocytes, multinucleated giant cells and few neutrophils, without vasculitis. The patient was started on hydroxychloroquine 400 mg daily. Three weeks later, her pain and tenderness had completely resolved and the inflammatory margin of the lesions had clearly regressed. DISCUSSION: This unique condition, with distinctive clinical and histological features, is similar to cases described under the term "lipophagic panniculitis", seen mostly in children, but also "connective tissue panniculitis". Their clinical resemblance and response to hydroxychloroquine therapy leads us to think that these two entities, previously subsumed under the eponym of Weber-Christian disease or Rothman-Makai syndrome, are closely related. CONCLUSION: Dermatologists and dermatopathologists should be made aware of this unusual entity, and of the fact that it can arise in adult patients, so that they may make an early diagnosis and thus prevent the unsightly consequences of lipoatrophy.

A case of Weber-Christian disease with later development of rheumatoid arthritis.

Weber-Christian disease (WCD) is a syndrome characterized by recurrent subcutaneous nodules, fever, occasional lipoatrophy, fatigue, arthralgia, and myalgia. We report a case of WCD associated with rheumatoid arthritis. A 65-year-old woman consulted our outpatient clinic because of bilateral hand swelling. The patient had presented with fever and subcutaneous nodules in her trunk and upper and lower extremities in 1983. At that time, the dermatology department diagnosed this patient as having WCD after biopsy of the nodules demonstrated lobular panniculitis. She has been treated with corticosteroid (5-15 mg/day) since then. The patient continued to have recurrent episodes of transient inflammatory arthritis in the small joints of the fingers and fever, and was initially assessed at our institution in October 2007. Finally, in November 2007, she was diagnosed as having both WCD and rheumatoid arthritis (RA) and treated with corticosteroid (5 mg/day) and methotrexate (MTX) (7.5 mg/week). Thereafter, her clinical symptoms gradually improved. This is the second case of WCD showing the subsequent development of RA, successfully treated with MTX, in the English literature. This case may provide clinical insight into WCD and RA.

Lipoatrophic connective tissue panniculitis.

Many causes of what was formerly called Weber-Christian and Rothmann-Makai diseases are being clarified as specific forms of panniculitis. Among them, an autoimmune process involving the subcutaneous fat without criteria for another defined disorder coined "connective tissue panniculitis" by Winckelman et al in 1980 has been described. We describe this disease in a 4-year-old boy who presented with multiple subcutaneous inflammatory nodules that extended in an annular fashion, resolved leaving lipoatrophy, with recurrence 8 years later. The histologic findings were consistent with a granulomatous lipophagic panniculitis. We review previous reports and emphasize the limited therapeutic options, chronic evolution, severe esthetic sequelae and possible association with other autoimmune disorders of this uncommon condition.

Acute infectious id panniculitis/panniculitic bacterid: a distinctive form of neutrophilic lobular panniculitis.

BACKGROUND: Lobular panniculitis encompasses lupus profundus, atypical lymphocytic lobular panniculitis, erythema induratum and subcutaneous Sweet's syndrome, while septal panniculitis includes erythema nodosum and fibrosing dermal processes of burn out necrobiosis lipoidica and morphea profundus. Panniculitis may represent a sign of systemic disease and/or a modified immune response to hematogenously disseminated antigen. METHODS: We describe 10 cases of sterile neutrophilic dominant lobular panniculitis that represented an id reaction to non-tuberculous stimuli. RESULTS: Four males and six females had sudden tender non-ulcerated lower extremity nodules with preceding non-tuberculous infectious triggers. Three also had upper extremity lesions. Biopsies showed dominant neutrophilic infiltrate with subcutaneous microabscesses (7), extravascular granulomatous infiltrates (5), thrombotic microangiopathy (5) and necrotizing vasculitis (5). Stains evaluating microbial pathogens were negative. Lesions resolved with antibiotic treatment and/or abscess drainage; no case recurred. Medical histories included atopic diathesis (4), primary antiphospholipid antibody syndrome (1), ulcerative colitis (1) and acute lymphocytic leukemia (1). Serologies showed polyclonal hypergammaglobulinemia, cold agglutinins and cryofibrinogens. CONCLUSIONS: We propose the term acute infectious id panniculitis for cases of neutrophilic lobular panniculitis triggered by non-tuberculous infectious stimuli. This course may be self-limited. Microvascular cofactors and/or procoagulant states may be pathogenetically important. Recognizing this entity may circumvent the need for an exhaustive evaluation for other causes of neutrophilic lobular panniculitis.