Published on 19/03/2015 by admin
Filed under Dermatology
Last modified 22/04/2025
This article have been viewed 2551 times
Carrie Ann R. Cusack and Matthew Fanelli
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Panniculitis is a term used to describe the different inflammatory diseases of the subcutaneous fat. The subcutaneous fat is derived embryologically from mesenchymal cells. It is this mesenchyme which gives rise to adipocytes, or fat cells. A cohesive collection of fat cells, termed a microlobule, makes up the basic unit of the subcutaneous tissue. When these microlobules are grouped together, they make up the individual lobules of the subcutaneous layer of skin. Lobules are separated from each other by fibrous septae. It is within these septae that the main arteries and veins that supply the fat tissue are located. Classically, the panniculitides have been defined by knowledge of this basic architecture of the subcutaneous tissue. Most classifications define them based on whether the inflammation is located in the lobules or in the fibrous septae. In reality, none of the panniculitides have their inflammation located solely in the septae or solely in the lobules. It is more correct to describe them as predominantly septal or predominantly lobular panniculitides. Based on this classification, we can better separate the individual disease processes.
The characteristic septal panniculitis is erythema nodosum, which is also the most common panniculitis. The majority of other panniculitides are predominantly lobular. However, some other rarer panniculitides have predominantly septal inflammation, such as subacute nodular migratory panniculitis, panniculitis of morphea/scleroderma, and α1-antitrypsin deficiency panniculitis. Within the lobular panniculitides, a further division can be made based on the presence of vasculitis. The main lobular panniculitis with vasculitis is erythema induratum, or more aptly termed, nodular vasculitis. Rarer vasculitic panniculitides occur in the setting of leprosy with erythema nodosum leprosum and Lucio phenomenon. The remaining lobular panniculitides are not associated with vasculitis, but can be associated with systemic disease. Subtypes include lupus panniculitis, pancreatic panniculitis, cytophagic histiocytic panniculitis, panniculitis of dermatomyositis, infectious panniculitis, sclerosing panniculitis/lipodermatosclerosis, oxalosis, and those with needle-shaped clefts (sclerema neonatorum and subcutaneous fat necrosis of the newborn).
In previous editions of this book, as well as in much of the older literature, Weber–Christian disease (WCD) has been described. This was defined loosely as a lobular panniculitis without vasculitis which also displayed non-specific systemic features. This was a diagnosis of exclusion which essentially lumped together all panniculitides that did not fit into one of the more specific diagnoses. This term has recently fallen out of favor. Many of the cases originally defined as WCD have been reclassified since newer panniculitides, such as nodular vasculitis, α1-antitrypsin panniculitis, and pancreatic panniculitis, were separated as specific diseases. In a report by White and Winkelmann, they reviewed 30 previously diagnosed cases of WCD and found that, in all cases, a more specific diagnosis could be found. Therefore, we will not include WCD in our classification for panniculitides in this chapter.
Many studies can be performed in evaluating panniculitides; however, none is more important than the skin biopsy. Therefore, it is extremely important that the biopsy includes a substantial portion of the subcutaneous fat. A punch biopsy alone is not enough. And while a double punch biopsy technique is better, the best way to get a sample of the subcutaneous tissue is through an excisional biopsy that extends through the subcutis, or a narrow incisional biopsy that includes a significant amount of fatty tissue. If an infectious cause of the panniculitis is suspected, a small piece of this biopsy should be sent for Gram staining as well as culture for bacteria, mycobacteria, and fungi. If a mycobacterial infection is in consideration, the specimen should be grown at 24, 30, 37, and 42°C.
Lupus panniculitis, also known as lupus profundus, makes up only a small portion of cutaneous lupus erythematosus, comprising only 2–3%. In many instances, it is the first cutaneous sign of lupus erythematosus, preceding many other features. It is associated mainly with discoid lupus erythematosus (DLE), occurring in more than one-third of cases of DLE. Lesions of DLE often are present in the skin overlying the lesions of lupus profundus. Lupus panniculitis occurs in approximately 10–15% of patients with systemic lupus erythematosus (SLE), which tends to have a chronic course with few systemic manifestations, usually arthralgias. The distribution of these lesions can be helpful in making the diagnosis as they are typically located on the face, trunk, and proximal extremities, in contrast to erythema nodosum. Laboratory studies should include antinuclear antibody (ANA), dsDNA, ssDNA, SSA, SSB, complement levels, complete blood count (CBC), and a chemistry panel. On biopsy, it shows a predominantly lobular inflammation of T lymphocytes and macrophages. A biopsy for direct immunofluorescence (DIF) should be performed to confirm the diagnosis in cases where the histopathology is not specific. In a high majority of cases, a linear deposition of C3 and IgM is present along the dermoepidermal junction in the overlying skin. First-line therapy for lupus profundus consists of systemic antimalarial agents as well as sunscreen use. The addition of a second antimalarial agent has proven helpful in patients who are resistant to just one agent. In addition, systemic corticosteroids can also be used first line; however, they are usually effective only in the early phases of disease.
Nodular vasculitis includes both tuberculous and non-tuberculous causes. The tuberculous form is referred to as erythema induratum (of Bazin). Non-tuberculous etiologies include other infectious agents such as Nocardia or hepatitis C virus and medications such as propylthiouracil. The non-tuberculous form has sometimes been referred to as erythema induratum of Whitfield. Clinically, lesions appear on the posterior legs of middle-aged women, and present as painful, erythematous nodules. In making the diagnosis, mycobacterial culture as well as polymerase chain reaction (PCR) of mycobacterial DNA should be ordered. Purified protein derivative as well as Quantiferon-TB Gold test can be helpful in making the diagnosis caused by tuberculosis. On biopsy, it is typically a lobular panniculitis with a mixed inflammation, comprising lymphocytes and neutrophils. The key is identifying vasculitis as well, as the majority of cases show inflammation of either arteries or veins. Treatment includes multi-drug anti-tuberculous agents in cases caused by tuberculosis, and other antibiotics in cases of other infectious etiologies. Other effective therapies include potassium iodide, colchicine, and corticosteroids.
Pancreatic panniculitis can occur in many different pancreatic disorders that cause tissue necrosis. This includes acute or chronic pancreatitis, pancreatic carcinoma, with acinar cell being the main type, pancreas divisum, and pancreatic pseuodocysts. It occurs in about 2–3% of all cases of pancreatic diseases. The presence of panniculitis may precede the detection of pancreatic disease, and warrants an investigation for potential etiologies. It may be a harbinger of metastasis in cases of pancreatic carcinoma. Lesions consist of subcutaneous nodules that usually occur on the legs (around the ankles and knees predominantly) but can occur in any location. They may or may not be painful, but the majority of lesions ulcerate, discharging an oily brown substance. Histopathology shows fat necrosis and the formation of ‘ghost cells’ is classic. Amylase and, more specifically, lipase are elevated in pancreatic panniculitis and should be ordered. A CBC should be ordered as well, as eosinophilia may be present in 60% of cases. Imaging with MRI can identify a pancreatic malignancy. Treatment involves treating the underlying pancreatic inflammation. Octreotide has also been helpful.
Cytophagic histiocytic panniculitis is defined based on the presence of hemophagocytosis on histopathology. There are characteristic ‘bean-bag cells’ which are macrophages that engulf lymphocytes, neutrophils, and erythrocytes. The vast majority of cases are caused by a lymphoma. The main types are Epstein-Barr virus associated extranodal NK/T-cell lymphoma, and primary cutaneous gamma/delta T-cell lymphoma. Clinically, painful subcutaneous nodules are present. Patients may have a prolonged clinical course that may involve fever, hepatosplenomegaly, and pancytopenia secondary to hemophagocytosis of the bone marrow. Treatment consists of treating any underlying malignancy, possibly with a bone marrow transplant. In cases where lymphoma has not been identified, cyclosporin has been effective.
α1-Antitrypsin deficiency panniculitis occurs in patients with a severe deficiency of this enzyme, a protease inhibitor. There are many different alleles of the gene that encodes this protein, with the most common being the M allele. The most common phenotype for the protease inhibitor (Pi) is PiMM. Patients with the S or Z allele may exhibit a mild deficiency in the enzyme. Patients with the phenotype PiZZ have the most severe enzyme deficiency, and it is typically these patients who manifest the panniculitis. Inflammation in the subcutaneous fat occurs because lipase, elastase, and other enzymes are not neutralized. These patients have painful, often purpuric nodules that ulcerate and drain. On pathology, there is usually focal necrosis of the fat lobules with a predominantly neutrophilic inflammation. An elastic stain of the biopsy may be helpful to show the reduced elastic tissue. Gene phenotyping helps determine an enzyme mutation. The most effective treatment is enzyme replacement through intravenous injections. Other treatments include dapsone, colchicine, and liver transplantation to permanently replace the missing enzyme.
Panniculitis can also occur in infants. The two main types are sclerema neonatorum and subcutaneous fat necrosis of the newborn. They both are characterized histologically by needle-shaped clefts within lipocytes. In sclerema neonatorum, an extremely rare condition, the skin becomes hardened on the buttocks or thighs, and then rapidly spreads in the first few days of life, causing immobility. Death typically occurs in a few days. Treatment for this condition is disappointing. It involves supportive care, treating any underlying condition, and exchange transfusions. The prognosis for subcutaneous fat necrosis of the newborn, on the other hand, is good. It involves full-term infants in contrast to the premature newborns in sclerema neonatorum. Clinically, this condition consists of localized, indurated plaques involving the trunk that form during the first few weeks of life. Most lesions resolve spontaneously, so treatment involves supportive care. It may be complicated by hypercalcemia, in which case, treatment for this may be needed.
Lipodermatosclerosis, or sclerosing panniculitis, is a condition that usually develops in patients with chronic venous insufficiency. It is classically located on the medial lower legs in middle-age women. It has been described as looking like an “inverted champagne bottle.” Histologically there is ischemia and necrosis of the central fat lobule, and there are characteristic lipomembranous changes. Treatment involves correcting the venous insufficiency with compression stockings and leg elevation. Stanozolol has also been helpful.
Panniculitis can also occur in the setting of other connective tissue diseases such as dermatomyositis and morphea/scleroderma. In morphea, usually there is a septal panniculitis with a plasma lymphocytic cell infiltrate. Indurated plaques appear on the trunk and extremities. In dermatomyositis, the panniculitis is more lobular and the inflammation is primarily lymphocytic. Clinically, there are tender, indurated plaques that may ulcerate and heal with atrophy. Treatment of both conditions involves treating the underlying connective tissue disease.
The physical forms of panniculitis, such as those resulting from cold exposure, foreign body, or factitious causes, usually resolve by removal of the offending trigger or surgical removal of the foreign body. This is similar to panniculitis caused by silicone or paraffin that has been used for cosmetic purposes. There are also forms of panniculitis associated with chronic renal failure, with the main ones being calciphylaxis and oxalosis. Calciphylaxis can be very severe and cause large areas of necrosis. Treatment involves parathyroidectomy, sodium thiosulfate, binding agents, or renal transplantation. Oxalosis is a crystalline deposit panniculitis. Calcium oxalate crystals typically are deposited on the palmar fingers, among other locations. Treatment for this is renal transplantation as well.
Skin biopsy for routine microscopy (most important)
Skin biopsy for culture and sensitivity (routine, mycobacterial, fungal)
Skin biopsy for PCR
Skin biopsy for immunoperoxidase and gene rearrangement studies
ANA and other rheumatologic serologic tests
Serum α1-antitrypsin levels
Serum lipase and amylase
Abdominal MRI
Complete blood count with differential
Chemistry panel
Connective tissue panniculitis: lupus panniculitis, dermatomyositis, morphea/scleroderma.
Hansen CB, Callen JP. Dermatol Ther 2010; 23: 341–9.
A review article which touts oral antimalarials and sunscreen as first-line treatment for lupus profundus. Hydroxychloroquine is recommended at a dose of 200 mg twice a day with a maximum dose of 6.5 mg/kg/day. The onset of action is said to be around 4 to 8 weeks, but can take up to 6 months for optimal benefit. Chloroquine is also recommended at a dose of 250–500 mg/day, but it seems to have a higher risk of ophthalmological toxicity.
Systemic lupus erythematosus presenting as panniculitis (lupus profundus).
Diaz-Jouanen E, DeHoratius RJ, Alarcón-Segovia D, Messner RP. Ann Intern Med 1975; 82: 376–9.
A case study in which five or six patients improved after adding hydroxychloroquine to their systemic corticosteroid regimen.
Connective tissue panniculitis.
Winkelmann RK, Padilha-Goncalves A. Arch Dermatol 1980; 116: 291–4.
Case report of a patient obtaining remission with hydroxychloroquine 200 mg twice daily and systemic corticosteroids. However, she developed SLE. A second patient on hydroxychloroquine was cleared of the condition after failing to respond to non-steroidal anti-inflammatory drugs (NSAIDs), potassium iodide, and corticosteroids.
Lupus erythematosus panniculitis (profundus).
Maciejewski W, Bandmann HJ. Acta Derm Venereol 1979; 59: 109–12.
Case report of a patient diagnosed with lupus profundus by direct immunofluorescence and treated with chloroquine.
Lupus erythematosus presenting as panniculitis.
Verbov JL, Borrie PR. Proc R Soc Med 1971; 64: 28–9.
A case report of the condition clearing in one patient with hydroxychloroquine 200 mg twice daily.
Treatment of chronic discoid lupus erythematosus with intralesional triamcinolone.
Rowell NR. Br J Dermatol 1962; 74: 354–7.
One patient with lupus profundus and 27 patients with DLE were treated with intralesional triamcinolone 10 mg/mL, and in 27 of the 28 the condition cleared or was clearing. All patients had failed to respond to antimalarials. The condition cleared in the lupus profundus patient.
Lupus erythematosus profundus treated with clobetasol propionate under a hydrocolloid dressing.
Yell JA, Burge SM. Br J Dermatol 1993; 128: 103.
A single case of a cure with clobetasol under hydrocolloid dressing occlusion, changed weekly. The patient responded after 1 month.
Lupus panniculitis treated by a combination therapy of hydroxychloroquine and quinacrine.
Chung HS, Hann SK. J Dermatol 1997; 24: 569–72.
In this case report, a 24-year-old male presented with non-tender, indurated plaques on the left facial and submandibular regions. Histopathologic and direct immunofluorescence studies confirmed the diagnosis of lupus profundus. The patient had marginal improvement with either hydroxychloroquine or systemic steroids alone. However, the combination of hydroxychloroquine and quinacrine together produced essential clearing of the lesions.
Intravenous immunoglobulin in lupus panniculitis.
Santo JE, Gomes MF, Gomes MJ, Peixoto LC, Pereira S, Acabado A, et al. Clin Rev Allergy Immunol 2010; 38: 307–18.
The patient was a 51-year-old Caucasian female who initially developed nodular lesions on the abdomen and the limbs. Pathology and DIF were consistent with lupus panniculitis. The patient was initially treated with hydroxychloroquine but developed transaminitis and liver biopsy was consistent with nonspecific hepatic inflammation so the antimalarial was stopped. She was started on systemic steroids and azathioprine, but had minimal response. Over the next several years, she was tried on thalidomide 300 mg, but developed peripheral neuropathy and diarrhea. She was also tried on dapsone, but again had a rise in liver enzymes so these medications were stopped. She then was tried on increasing doses of systemic steroids and azathioprine, but there was no improvement. After 15 years of failing different therapies, she was started on IVIG once monthly for 6 months and saw complete regression of the subcutaneous nodules. The doses were then spread out to every 3 months and the patient remained in remission.
A case of ‘refractory’ lupus erythematosus profundus responsive to rituximab [case report].
McArdle A, Baker JF. Clin Rheumatol 2009; 28: 745–6.
The patient was a 22-year-old African-American female who presented with painful nodules on the buttocks, consistent with a diagnosis of lupus panniculitis. The patient was started on prednisolone 1 mg/kg and hydroxychloroquine. Despite this treatment, the patient worsened. The patient had a history of hepatitis so azathioprine was not considered. Mycophenolate mofetil was attempted, but it resulted in significant nausea and vomiting. Rituximab, an anti-CD20 monoclonal antibody, was then considered. The dosage was 1000 mg administered 2 weeks apart. Over a 1-month period, the patient was noted to have complete resolution of all signs of lupus profundus.
Lupus erythematosus profundus.
Arnold HL Jr. Arch Dermatol 1956; 73: 14–26.
One patient had a partial response to intravenous gold followed by bismuth sodium thioglycolate.
Facets of lupus erythematosus: panniculitis responding to thalidomide.
Wienart S, Gadola S, Hunziker T. J Deutschen Dermatologischen Gesellschaft 2008; 6: 214–16.
Lupus erythematosus profundus with partial C4 deficiency responding to thalidomide.
Burrows NP, Walport MJ, Hammond AH, Davey N, Jones RR. Br J Dermatol 1991; 125: 62–7.
A female patient with disfiguring lupus erythematosus profundus from the age of 13 years was found to have an isolated partial C4 deficiency. Marked improvement in her cutaneous lesions occurred with thalidomide.
The dynamism of cutaneous lupus erythematosus: mild discoid lupus erythematosus evolving into SLE and SCLE and treatment-resistant lupus panniculitis.
Wozniacka A, Salamon M, Lesiak A, McCauliffe DP, Sysa-Jedrzejowska A. Clin Rheumatol 2007; 26: 1176–9.
A 47-year-old woman with biopsy proven lupus panniculitis responded to cyclosporine at a dose of 4 mg/kg/day in addition to methylprednisolone. After 10 days, there was improvement. The steroids were tapered and stopped after 3 months and the cyclosporine was gradually tapered to 2 mg/kg/day. The patient had previously failed antimalarials, systemic steroids, azathioprine, cyclophosphamide, methotrexate, and pulse doses of methylprednisolone.
Lupus erythematosus profundus successfully treated with dapsone: review of the literature.
Ujiie H, Shimizu T, Ito M, Arita K, Shimizu H. Arch Dermatol 2006; 142: 399–401.
A 56-year-old woman with ulcerated, biopsy proven lupus profundus responded to dapsone at a dose of 75 mg/day after 6 weeks.
Potassium iodide in the treatment of erythema nodosum and nodular vasculitis.
Horio T, Imamura S, Danno K, Ofuji S. Arch Dermatol 1981; 117: 29–31.
A case study in which 11 of 51 patients had nodular vasculitis. Seven of the 11 patients responded within 2 weeks to potassium iodide 300 mg three times daily.
Treatment of erythema nodosum and nodular vasculitis with potassium iodide.
Schulz EJ, Whiting DA. Br J Dermatol 1976; 94: 75–8.
Sixteen of 17 patients responded to potassium iodide, usually with relief of symptoms within 2 days. The average duration of therapy was 3 weeks. The daily dose ranged from 360 mg to 900 mg.
Successful treatment of erythema induratum of Bazin following rapid detection of mycobacterial DNA by polymerase chain reaction.
Degitz K, Messer G, Schirren H, Classen V, Meurer M. Arch Dermatol 1993; 129: 1619–20.
A single case of erythema induratum successfully treated with isoniazid, rifampin, and ethambutol.
Erythema induratum of Bazin.
Cho KH, Lee DY, Kim CW. Int J Dermatol 1996; 35: 802–8.
A retrospective study of 32 patients with proven erythema induratum of Bazin. All improved with triple antituberculous therapy, but four relapsed and subsequently cleared.
Diagnosis and treatment of erythema induratum (of Bazin).
Feiwel M, Munro DD. Br Med J 1965; 1: 1109–11.
Twelve patients were diagnosed with erythema induratum secondary to tuberculosis and all responded well to two to three drug therapy: streptomycin, p-aminosalicylic acid 12.5 mg daily, and isoniazid 200–260 mg daily for 9 months.
Neutrophilic vascular reactions.
Jorizzo JL, Solomon AR, Zanolli MD, Leshin B. J Am Acad Dermatol 1988; 19: 983–1005.
A review article in which nodular vasculitis is one of the causes of necrotizing vasculitis. NSAIDs may benefit some symptoms, such as serum sickness-like features, but do not help cutaneous lesions.
Chloroquine-induced remission of nodular panniculitis present for 15 years.
Shelley WB. J Am Acad Dermatol 1981; 5: 168–70.
One patient responded to chloroquine 250 mg/day within 1 month after failing to respond to various therapies for nodular panniculitis. These included corticosteroids, NSAIDs, and tetracycline.
Cutaneous necrotizing vasculitis.
Lotti T, Comacchi C, Ghersetich I. Int J Dermatol 1996; 35: 457–74.
By inhibiting neutrophil chemotaxis, oral colchicine in doses of 0.6 mg twice daily may be helpful in chronic forms of the disease.
Nodular vasculitis (erythema induratum): treatment with auranofin.
Shaffer N, Kerdel FA. J Am Acad Dermatol 1991; 25: 426–9.
One patient with nodular vasculitis responded to oral gold 3 mg twice daily and improved after 3 weeks. She had previously failed to respond to prednisone, colchicine, D-penicillamine, sulindac, and bemetanide for suspected erythema nodosum.
Case reports: nodular vasculitis responsive to mycophenolate mofetil.
Taverna JA, Radfar A, Pentland A, Poggioli G, Demierre MF. J Drugs Dermatol 2006; 5: 992–3.
A 70-year-old woman was treated with 1 g of mycophenolate mofetil twice daily. She slowly responded over a 1-year period.
Resolution of panniculitis after placement of pancreatic duct stent in chronic pancreatitis.
Lambiase P. Am J Gastroenterol 1996; 91: 1835–7.
One patient with pancreatitis secondary to alcohol presented with chest pain and tender skin nodules on the shins. After a skin biopsy showed panniculitis he was diagnosed with pancreatitis without abdominal pain, but a high amylase. A stent was placed to correct a stricture in the pancreatic duct, leading to resolution of the symptoms and the skin lesions within 1 month.
Panniculitis caused by acinous pancreatic carcinoma.
Heykarts B, Anseeuw M, Degreef H. Dermatology 1999; 198: 182–3.
One patient with skin nodules was found to have acinar pancreatic carcinoma upon surgical resection. She was initially unresponsive to high-dose corticosteroids and methotrexate, but the skin lesions resolved slowly after the resection. Subsequently, metastases were found in the right liver and the patient failed to respond to fluorouracil.
Resolution of pancreatic panniculitis following metastasectomy.
Banfill KE, Oliphant TJ, Prasad KR. Clin Exp Dermatol 2012; 37: 440–1.
A 69-year-old man presented with a 6-month history of painful skin lesions. Three months after developing the skin lesions, a CT scan revealed a large necrotic mass in the liver consistent with a metastasis. The patient underwent resection of a solitary liver metastasis, which resulted in complete resolution of his panniculitis.
Liquefying panniculitis associated with acinous carcinoma of the pancreas responding to octreotide.
Hudson-Peacock MJ, Regnard CF, Farr PM. J R Soc Med 1994; 87: 361–2.
One patient presented with increasing numbers of painful leg nodules secondary to poorly differentiated adenocarcinoma. She failed to respond to prednisolone, but octreotide 50 µg twice daily subcutaneously halted progression of the skin nodules. However, despite the therapy, the patient died 3 weeks later.
Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases.
Marzano AV, Berti E, Paulli M, Caputo R. Arch Dermatol 2000; 136: 889–96.
A report of seven cases of CHP. Five patients had subcutaneous T-cell lymphoma and died after failing to respond to various chemotherapeutic agents. One patient has done well with prednisone for 13 months and the other living patient has done well with systemic corticosteroids, cyclophosphamide, and dapsone for 36 years.
Successful treatment of cytophagic histiocytic panniculitis with modified CHOP-E: cyclophosphamide, adriamycin, vincristine, prednisone, and etoposide.
Matsue K, Itoh M, Tsukuda K, Miyazaki K, Kokubo T. Am J Clin Oncol 1994; 17: 470–4.
One patient with CHP received eight courses of modified CHOP-E every 3 weeks and obtained a remission that has lasted 2 years.
Cytophagic histiocytic panniculitis. Case report with resolution after treatment.
Alegre VA, Fortea JM, Camps C, Aliaga A. J Am Acad Dermatol 1989; 20: 875–8.
One patient with CHP for 2 months was treated with cyclophosphamide, vincristine, doxorubicin, and prednisone for nine cycles and achieved a cure. The authors recommend early, aggressive treatment.
Cytophagic histiocytic panniculitis – a syndrome associated with benign and malignant panniculitis: case comparison and review of the literature.
Craig AJ, Cualing H, Thomas G, Lamerson C, Smith R. J Am Acad Dermatol 1998; 39: 721–36.
Case report of two patients with CHP. One patient responded to prednisone plus cyclosporine 15 mg/kg/day. The other patient died during treatment with chemotherapy for T-cell lymphoma.
Subcutaneous panniculitic T-cell lymphoma in children: response to combination therapy with cyclosporine and chemotherapy.
Shani-Adir A, Lucky AW, Prendiville J, Murphy S, Passo M, Huang FS, et al. J Am Acad Dermatol 2004; 50: S18–22.
A case report of two adolescents with CHP. Both responded symptomatically to cyclosporine and complete remission was obtained in one with chemotherapy.
Successful treatment of severe cytophagic histiocytic panniculitis with cyclosporin A.
Ostrov BE, Athreys BH, Eichenfield AH, Goldsmith DP. Semin Arthritis Rheum 1996; 25: 404–13.
A 16-year-old patient with CHP who was on prednisone flared and cyclosporine was added. At a dose of 4 mg/kg/day, remission occurred.
Successful treatment of cyclosporin-A-resistant cytophagic histiocytic panniculitis with tacrolimus.
Miyabe Y, Murata Y, Baba Y, Ito E, Nagasaka K. Mod Rheumatol 2011; 21: 553–6.
A 34-year-old female with CHP initially failed treatment with high-dose prednisolone with cyclosporine. After changing cyclosporine to tacrolimus, the CHP responded.
Effective high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation in a patient with the aggressive form of cytophagic histiocytic panniculitis.
Koizumi K, Sawada K, Nishio M, Katagiri E, Fukae J, Fukada Y, et al. Bone Marrow Transplant 1997; 20: 171–3.
One patient with CHP was treated with CHOP-E and GM-CSF, and achieved remission. He was subsequently treated with bone marrow transplant and remained disease free for 1 year.
Cytophagic histiocytic panniculitis is not always fatal.
White JW Jr, Winkelmann RK. J Cutan Pathol 1989; 16: 137–44.
One patient with CHP was treated with potassium iodide and achieved remission for 15 years.
Successful treatment of a patient with subcutaneous panniculitis-like T-cell lymphoma with high-dose chemotherapy and total body irradiation.
Mukai HY, Okoshi Y, Shimizu S, Katsura Y, Takei N, Hasegawa Y, et al. Eur J Haematol 2003; 70: 413–16.
One patient with CHP and T-cell lymphoma achieved remission after three courses of CHOP followed by total body irradiation and autologous stem cell transplant.
Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis.
Behrens EM, Kreiger PA, Cherian S, Cron RQ. J Rheumatol 2006; 33: 2081–4.
A 14-year-old girl with CHP who failed cyclosporine and etoposide responded to methylprednisolone and anakinra.
Use of anti-collagenase properties of doxycycline in treatment of alpha 1-antitrypsin deficiency panniculitis.
Humbert P, Faivre B, Gibey R, Agache P. Acta Derm Venereol 1991; 71: 189–94.
Three patients with recurrent α1-antitrypsin (A1AT) panniculitis were treated with doxycycline 200 mg daily for 3 months. All cleared within 8 weeks.
Clinical and pathologic correlations in 96 patients with panniculitis including 15 patients with deficient levels of alpha 1-antitrypsin.
Smith KC, Su Pittelkow MR, Winkelmann RK. J Am Acad Dermatol 1989; 21: 1192–6.
Fifteen patients had A1AT panniculitis. Out of six treated with dapsone, five responded.
Alpha 1-antitrypsin deficiency-associated panniculitis: resolution with intravenous alpha 1-antitrypsin administration and liver transplantation.
O’Riordan K, Blei A, Rao MS, Abecassis M. Transplantation 1997; 63: 480–2.
Two patients with homozygous deficiency had panniculitis. One received a liver transplant and was cured. The other was treated with intravenous A1AT and was clear while on this medicine.
Treatment of alpha-1-antitrypsin deficiency, massive edema, and panniculitis with alpha-1 protease inhibitor.
Furey NL, Golden RS, Potts SR. Ann Intern Med 1996; 125: 699.
A patient with red thigh nodules was found to have A1AT deficiency panniculitis. The patient failed to respond to doxycycline and received α1-protease concentrate and improved within 24 hours.
Atlantic Provinces Dermatology Association Society Meeting, May 3, 1986.
Miller RAW, cited by Ross JB. J Can Dermatol Assoc 1986; 13–17.
One patient with A1AT deficiency panniculitis failed to respond to prednisone and azathioprine but responded after dapsone, potassium iodide, and plasmapheresis two to three times per week.
Cyclophosphamide therapy for Weber–Christian disease associated with alpha 1-antitrypsin deficiency.
Strunk RW, Scheld WM. South Med J 1986; 79: 1425–7.
One patient with A1AT deficiency panniculitis failed to respond to prednisone and heparin. Cyclophosphamide was added with a good response.
Necrotic panniculitis with alpha-1 antitrypsin deficiency.
Viraben R, Massip P, Dicostanzo B, Mathieu C. J Am Acad Dermatol 1986; 14: 684–7.
A patient with A1AT deficiency panniculitis failed to respond to prednisolone, lincomycin, colchicine, and cyclophosphamide. Rapid improvement occurred following plasma exchange transfusions once daily for 8 weeks.
Familial occurrence of alpha 1-antitrypsin deficiency and Weber-Christian disease.
Breit SN, Clark P, Robinson JP, Luckhurst E, Dawkins RL, Penny R. Arch Dermatol 1983; 119: 198–202.
A report of two cases of A1AT deficiency panniculitis. One patient responded once cyclophosphamide was added to the initial treatment with dexamethasone. The other patient received colchicine and dicloxacillin and the panniculitis resolved.
Severe panniculitis caused by homozygous ZZ alpha 1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin).
Chowdhury MM, Williams EJ, Morris JS, Ferguson BJ, McGregor AD, Hedges AR, et al. Br J Dermatol 2002; 147: 1258–61.
Life-threatening panniculitis and skin necrosis was cleared with Prolastin and prednisolone. She was put on a dose of 100 mg/kg every 6 days.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
WhatsApp us