Fasciitis-panniculitis Syndrome Research Articles

O17 What lies beneath: A case of lymphohistiocytic myofasciitis in a 4-year-old girl

Case report - IntroductionA 4-year-old girl presented with a 1-year history of difficulty dressing, walking, and getting up from the ground. Examination revealed thickening of the skin, and the presumptive diagnosis was that of scleroderma. However, the dramatic loss of movement seemed out of proportion to her skin changes and therefore further investigations were completed. Her final diagnosis was that of a myofasciitis, a rare diagnosis in a child. This case highlights the importance of a thorough investigative process in order to make the correct diagnosis and explains our management plan for this young girl. Case report - Case descriptionA 4-year-old girl, previously well presented with a 1-year history of difficulty dressing, walking, and getting up from the ground. Her joints were never swollen. There were no other systemic symptoms. There was no past medical history. Developmentally all her milestones were achieved on time prior to symptom onset, though she always found it difficult to climb the stairs. Examination revealed thickening of the skin that felt like advanced scleroderma. The skin on her forearms and ankles was quite turgid. There was an area of morphea around her right shoulder. There were no areas of erythema. There was striking limitation of range of movement of her wrists and shoulders. There was marked stiffness around both ankle joints, although this appeared to be due to the overlying tissue structure, rather than the joint. Diagnosis was likely scleroderma. However, the skin changes seemed out of proportion with the dramatic loss of range of movement. An MRI of her shoulder girdle revealed dramatic fasciitis involved in the deep fascial planes, just superficial to the muscles. This was marked around the shoulder girdle. Her eosinophil counts gradually climbed. A plausible diagnosis was Eosinophilic Fasciitis/overlap with scleroderma features. This too is unusual in a young child. A muscle biopsy was performed. A full thickness biopsy of her right lateral bicep was taken. This revealed lymphohistiocytic granulomatous inflammation involving the skin, fascia and biceps muscle. The patient was started on high-dose methylprednisolone, for 3 consecutive days, followed by weaning oral prednisolone and subcutaneous methotrexate. Two months later her condition was completely transformed. She had gained full range of movement in all her joints. Her skin quality had greatly improved. She remained on methotrexate weekly. A repeat MRI was organised 8 months after commencing treatment. This revealed a dramatic improvement. She remains on methotrexate alone. Case report - DiscussionMyofasciitis is an unusual inflammatory myopathy almost exclusively described within the adult population. Myofasciitis is a distinctive entity, which is different from the usual idiopathic inflammatory myopathies, sarcoid myopathy and fasciitis-panniculitis syndrome. Clinical presentation can be non-specific particularly with the paediatric population. Hypotonia, developmental delay, fatigue, myalgia and muscle weakness have all been reported. Clinical presentation can be apparent in infancy, or slightly later in childhood. In macrophage myofasciitis the muscle biopsy typically reveals a monomorphous infiltration of the connective tissue by sheets of densely packed periodic acid-Schiff positive macrophages. There may be intermingled lymphocytes and muscle fibre damage.A few different associations have been postulated. Intramuscular injections containing aluminium adjunct may be associated with macrophagic myosfasciitis but the underlying process for this remains unclear. The association with autoimmune disease and macrophagic myofasciitis suggests that those from a particular genetic background may be more likely to develop this condition. Genetic predisposition, however, most likely accounts for the variability in the prevalence of macrophagic myofasciitis in different populations. Given the paucity of literature on macrophage myofasciitis in the paediatric population there is no clear consensus for a treatment strategy. On reviewing the literature many children were first treated with high dose IV pulsed methylprednisolone, followed by oral prednisolone, some received IV Immunoglobulin. Case report - Key learning pointsAs with many inflammatory muscle disorders patients are often seen by numerous clinicians prior to arriving at paediatric rheumatology. This may mean a prolonged time period before the patient receives appropriate treatment. Through delivering good education to our colleagues mainly in general paediatrics and general practice hopefully this will continue to improve.This case report highlights the importance of a muscle biopsy for diagnosis. Without tissue the correct diagnosis would not be possible and subsequently the treatment given may not be the most suitable for the patient.The histology for this myofasciitis is rare - in adult practice, but definitely within paediatric practice.Methotrexate is well recognised as a suitable and evidence-based treatment for many inflammatory myopathies. Although this condition is slightly different, it has worked incredibly well for the patient. This highlights that when faced with uncertainty as clinicians it is important to consider what we think theoretically will work for our patients.

Juvenile eosinophilic fasciitis: report of three cases with a review of the literature

Eosinophilic fasciitis (EF), also called Shulman's syndrome or fasciitis-panniculitis syndrome, is an uncommon scleroderma-like disorder of unknown etiology, characterized by induration and thickening of skin and soft tissue, usually associated with peripheral eosinophilia. In childhood, EF is poorly characterized.

The fasciitis-panniculitis syndrome in circulatory disorders of the limbs

Sporadic reports allude to the similarities between the subcutaneous and fascial sclerosis of venous disease and lymphedema with that of eosinophilic fasciitis and the related fasciitis-panniculitis syndrome. The objective of the present study was to compare the clinical and histological features of the subcutaneous induration in patients with impairment of the venous, arterial, and lymphatic circulation (6 patients) with those of patients with the fasciitispanniculitis syndrome of diverse etiologies (12 patients). The semiquantitative analysis of the microscopic changes evinces the equivalent intensity of subcutaneous septal fibrosis, inflammation and mucinosis, lobular panniculitis, fascial fibrosis and inflammation, as well as microscopic phlebitis and phlebosclerosis in both groups of patients. The similarities of the pathological alterations of the subcutaneous layers are indicative of a shared reaction pattern to a variety of insults. In the patients with circulatory disorders, analysis of the clinical circumstances establishes the convergence of synergistically acting factors in the causation of the fasciitis-panniculitis syndrome. The circulatory impediment is insufficient to induce the syndrome, but it apparently acts as a predisposing factor such that it amplifies the inflammatory-cicatrizing response to one ordinary damage or another. Awareness of the multifactorial etiopathogenesis of the fasciitis-panniculitis syndrome in patients with circulatory disturbances ushers in a novel perspective of therapeutic intervention at multiple levels.

Paraneoplastic fasciitis–panniculitis syndrome: a neurological point of view

A 54-year-old woman who had undergone gastrectomy to treat gastric adenocarcinoma 5 years previously and had since been in remission presented to a neuromuscular clinic complaining of stiffening and painful spasms of the legs and abdomen. Repeat pan-CT scans and gastric biopsy confirmed the recurrence of poorly differentiated signet ring gastric adenocarcinoma. Her symptoms improved remarkably on initiation of chemotherapy and worsened on discontinuation of chemotherapy. Neurological examination, MRI of the abdomen and lower extremities, whole-body fluorodeoxyglucose PET, pan-CT scan, electromyography, muscle biopsy, upper gastrointestinal tract radiography, esophagogastroduodenoscopy, and immunophenotyping (paraneoplastic, rheumatological and autoimmune diseases panels). Paraneoplastic fasciitis-panniculitis syndrome associated with the recurrence of poorly differentiated signet ring gastric adenocarcinoma. Chemotherapy of recurrent gastric adenocarcinoma and symptomatic management of painful spasms.

Therapeutic efficacy of intravenous cyclophosphamide concomitant with moderate- to high-dose prednisolone in two patients with fasciitis panniculitis syndrome

Fasciitis panniculitis syndrome (FPS) has been proposed as a new category of 'fasciitis' and includes the well-established eosinophilic fasciitis (EF). Unlike EF, FPS exhibits inconsistent eosinophilia and/or eosinophilic infiltration of the lesions. Principal histological FPS findings include dermal thickening, inflammation and thickening of the subcutaneous fat tissue, fibrous thickening of the fascia and inflammation of the adjacent muscle. FPS is commonly resistant to corticosteroids, and cimetidine is effective in approximately 80% of FPS patients. A new therapy for FPS is required for cases refractory to treatment or intolerant to cimetidine because of adverse drug reaction. In this report, two FPS patients were resistant to corticosteroids. Both received intravenous cyclophosphamide (IVCY) concomitant with moderate- to high-dose prednisolone (PSL), and this effectively treated the induration of the FPS lesions. Patient 1 was a 50-year-old woman who had been diagnosed with fasciitis following en bloc muscle biopsy of the thigh. She had been treated with high-dose PSL for 6 years, but the fasciitis was refractory. Induration of the neck, thorax and thighs resulted in impaired neck rotation, restrictive respiratory failure and impaired walking. A diagnosis of FPS was made by re-assessing the en bloc muscle biopsy. Although PSL (40 mg/day) for 18 days was ineffective, the addition of IVCY (400 mg) dramatically improved the disease manifestations. Patient 2 was a 68-year-old man who was diagnosed with fasciitis based on en bloc muscle biopsy of the left foot. He had been treated with PSL for 16 years, but the fasciitis was refractory. He exhibited lower limb induration and a refractory skin ulcer of the left foot. A diagnosis of FPS was made by re-assessing the en bloc muscle biopsy. Although PSL (40 mg/day) for 2 weeks was ineffective, the addition of IVCY (450 mg) improved both the lower limb induration and the skin ulcer. FPS may cause both entrapment vasculopathy of subcutis and perivasculitis of the subcutaneous fat tissue such that the skin ulcer might be closely related with the ischemic mechanism triggered by FPS. According to the clinical courses of our cases, IVCY combined with moderate- to high-dose PSL may be a new therapeutic choice for corticosteroid-resistant FPS patients.

Fasciitis-panniculitis syndrome simulating deep venous thrombosis

Fasciitis-panniculitis syndrome (FPS) is a condition characterized by inflammation of the subcutaneous cellular tissue, the aponeurosis and, eventually, the muscles. A 49-year-old female who was previously diagnosed as having retroperitoneal fibrosis presented recurrent episodes of intermittent inflammation of her limbs that simulated deep venous thrombosis (DVT). Biopsy revealed panniculitis with involvement of the fascia, characterizing FPS. Clinical improvement was achieved after corticosteroid therapy. Prompt recognition of FPS is important in order to avoid inadequate therapeutic measures since FPS can clinically simulate infectious or vascular diseases.

Fasciitis-panniculitis syndrome and advanced gastric adenocarcinoma in association with antibodies to single-stranded DNA

Fasciitis-panniculitis syndrome and advanced gastric adenocarcinoma in association with antibodies to single-stranded DNA

Unilateral Fasciitis of the Lower Leg

We report an elderly woman with a 3-month history of abdominal pain and painful swelling of her right lower leg. Magnetic resonance imaging revealed extensive fasciitis of the right superficial and deep crural fasciae. Endoscopic ultrasonography identified a tumor in the tail region of the pancreas with regional lymphatic nodal disease and suspicion of liver metastasis. The temporal relationship between the fasciitis and the pancreatic tumor suggests cancer-associated fasciitis-panniculitis syndrome. We report for the first time the incidence of the fasciitis-panniculitis syndrome in a patient with a previously undiagnosed solid pancreatic tumor.

The Fasciitis-Panniculitis Syndrome Presenting as Complex Regional Pain Syndrome Type 1

A 28-year-old man presented with a clinical picture suggestive of complex regional pain syndrome type I following a blow to the thenar eminence and thumb. Symptoms, including swelling of the hand and distal forearm, progressed until an amputation was carried out to rid the patient of an unendurable painful and nonfunctioning wrist and hand. The histologic evaluation of the amputation specimen showed: 1) dermal edema, perivascular dermatitis, and epidermal hyperkeratosis; 2) subcutaneous chronic inflammation with subtotal replacement of the adipose lobules by fibrous tissue associated with thickening of the muscular fascia, implying the fasciitis-panniculitis reaction pattern; 3) atrophy, degeneration, necrosis, and focal calcifications of the skeletal muscles; 4) phlebosclerosis, phlebectasias and lymphocytic arteritis; and 5) increased cortical porosity of the bones. It seems that the pathogenetic process underlying the fasciitis-panniculitis syndrome may rarely manifest as a complex regional pain syndrome-like disorder.

Les adjuvants vaccinaux et la myofaciite à macrophages

An unusual inflammatory myopathy characterised by an infiltration of non-epithelioid histiocytic cells has been recorded with increasing frequency in the past 5 years in France. We reassessed some of these cases.We did a retrospective analysis of 18 such cases seen in five myopathology centres between May, 1993, and December, 1997. The myopathological changes were reassessed at a clinopathology seminar.Detailed clinical information was available for 14 patients. The main presumptive diagnoses were polymyositis and polymyalgia rheumatica. Symptoms included myalgias in 12 patients, arthralgias in nine, muscle weakness in six, pronounced asthenia in five, and fever in four. Abnormal laboratory findings were occasionally observed, and included raised creatine kinase concentrations, increased erythrocyte sedimentation rate, and myopathic electromyography. Muscle biopsy showed infiltration of the subcutaneous tissue, epimysium, perimysium, and perifascicular endomysium by sheets of large macrophages, with a finely granular PAS-positive content. Also present were occasional CD8 T cells, and inconspicuous muscle-fibre damage. Epithelioid and giant cells, necrosis, and mitotic figures were not seen. The images were easily distinguishable from sarcoid myopathy and fasciitis-panniculitis syndromes. Whipple's disease, Mycobacterium avium intracellulare infection, and malakoplakia could not be confirmed. Ten patients were treated with various combinations of steroids and antibiotics; symptoms improved in eight patients, and stabilised in two.A new inflammatory muscle disorder of unknown cause, characterised by a distinctive pathological pattern of macrophagic myofasciitis, is emerging in France.

Macrophagic myofasciitis

A most unusual inflammatory myopathy, called macrophagic myofasciitis, first described by the Groupe d'Etudes et Recherche sur les Maladies Musculaires Acquises et Dysimmunitaires (GERMMAD), a specific branch of the Association Française contre les Myopathies was recorded with an increasing frequency from 1993 in the main French myopathologic centers. In October 1999, 65 macrophagic myofasciitis cases were recorded since our first description. We described the characteristics of the first 22 patients. The 22 patients (sex-ratio M/F, 1:3) were referred with the presumptive diagnosis of polymyositis (11), polymyalgia rheumatica (five), mitochondrial cytopathy (four), and congenital myopathy or muscle dystrophy (one each). Symptoms included myalgias (91%), arthralgias (68%), marked asthenia (55%), muscle weakness (45%), and fever (32%). Abnormal laboratory findings included elevated CK levels (50%), markedly increased ESR (37%), and myopathic EMG (35%). Muscle biopsy showed a unique myopathologic pattern characterized by 1) centripetal infiltration of epimysium, perimysium, and perifascicular endomysium by sheets of large cells of the monocyte/macrophage lineage (CD68+, CD1a-, S100-), with a PAS-positive content; 2) absence of necrosis, of both epithelioid and giant cells, and of mitotic figures; 3) presence of occasional CD8+ T-cells; 3) inconspicuous muscle fibre damage. The picture was easily distinguishable from sarcoid myopathy and fasciitis-panniculitis syndromes. The infectious diseases could not be documented in our patients. Patients improved under steroid therapy, associated or not with nonspecific antibiotic therapy. The authors discuss the main etiologic hypothesis of the macrophagic myofasciitis.

La myofasciite à macrophages: description, hypothèses étiopathogéniques

A new type of inflammatory myopathy of unknown etiology has recently been described in France. The myopathy, called macrophagic myofasciitis, had never been described in the literature. In December 1998, 35 cases of macrophagic myofasciitis were reported, showing an increase in its incidence since the description of the first case in 1993. The first 22 cases are described. The 22 patients were each referred with a presumptive diagnosis of either polymyositis (11 patients), polymyalgia rheumatica (5 patients), mitochondrial cytopathy (4 patients), or congenital myopathy or muscle dystrophy (1 patient for each). Clinical symptoms included myalgias (91%), arthralgias (68%), marked asthenia (55%), muscle weakness (45%), and fever (32%). Laboratory findings included elevated CK levels (50%) and a marked increased in the erythrocyte sedimentation rate (37%). Electromyographic recordings showed the existence of myopathy (35%). Muscle biopsy showed a unique pattern characterized by: (i) centripetal infiltration of the epimysium, perimysium and perifascicular endomysium by non epitheloid, cells of the monocyte/macrophage lineage (CD68+, CD1a-, S100-) with both large cytoplasm and PAS-positive content; (ii) absence of necrosis, of both epithelioid and giant cells, and of mitotic figures; (iii) occasional CD8+ T-cells; and, (iiii) minimal myocyte suffering. The disease symptoms were easily distinguishable from those of sarcoid myopathy and fasciitis-panniculitis syndromes. Infectious diseases known to be associated with reactive histiocytosis, including Whipple's disease, Mycobacterium avium intracellulare infection and malakoplakia, could not be documented. Patients' condition improved under corticosteroid therapy, associated or not with non-specific antibiotic therapy. A new inflammatory muscle disorder of unknown etiology, characterized by a distinctive pathological pattern of macrophagic myofasciitis, is emerging in France. Diagnosis is based on muscular biopsy. Numerous clinical, epidemiological and etiopathologic studies initiated by the GERMMAD (Groupe d'études et de recherche sur les maladies musculaires acquises) are in progress.

The fasciitis-panniculitis syndromes: histomorphometric assessment of the inflammatory and sclerosing processes affecting the subcutaneous and fascial tissues.

The fasciitis-panniculitis syndromes include several disorders characterized by skin induration due to fibrotic thickening of the septa of the subcutis and muscular fascia associated with chronic inflammation. Representative of the idiopathic form, eosinophilic fasciitis, is the prototype of the fasciitis-panniculitis syndromes. Secondary forms of the syndromes are causally related to a variety of diseases, such as infections, circulatory disorders, physical injuries and neoplasms. Histological sections of 10 idiopathic and 20 secondary cases of the fasciitis-panniculitis syndromes were assessed by means of computer-assisted image analysis. The percent of fibrous tissue, referred to as "extent of fibrosis", and the number of lymphocytes, macrophages, eosinophils, plasma cells, and mast cells within the subcutaneous-fascial complex were assessed. The "extent of fibrosis" varied from 1% to 2% in the control cases and from 4% to 98% in the index cases. The number of inflammatory cells per high power field varied from 0 to 33 in the control cases and from 9 to 323 in the index cases. There is a direct and positive correlation between the "extent of fibrosis" and the number of inflammatory cells. The "extent of fibrosis" and intensity of the inflammatory infiltration do not correlate with the nature of the underlying diseases, the patients'demographic data, the anatomic location of the subcutaneous induration or the duration of the lesion. It is suggested that the fasciitis-panniculitis syndromes are the morphological expression of a chronic, relapsing, inflammatory-sclerosing reaction pattern that is essentially identical in the idiopathic and the secondary forms of the disorder.

Macrophagic myofasciitis: an emerging entity

An unusual inflammatory myopathy characterised by an infiltration of non-epithelioid histiocytic cells has been recorded with increasing frequency in the past 5 years in France. We reassessed some of these cases. We did a retrospective analysis of 18 such cases seen in five myopathology centres between May, 1993, and December, 1997. The myopathological changes were reassessed at a clinopathology seminar. Detailed clinical information was available for 14 patients. The main presumptive diagnoses were polymyositis and polymyalgia rheumatica. Symptoms included myalgias in 12 patients, arthralgias in nine, muscle weakness in six, pronounced asthenia in five, and fever in four. Abnormal laboratory findings were occasionally observed, and included raised creatine kinase concentrations, increased erythrocyte sedimentation rate, and myopathic electromyography. Muscle biopsy showed infiltration of the subcutaneous tissue, epimysium, perimysium, and perifascicular endomysium by sheets of large macrophages, with a finely granular PAS-positive content. Also present were occasional CD8 T cells, and inconspicuous muscle-fibre damage. Epithelioid and giant cells, necrosis, and mitotic figures were not seen. The images were easily distinguishable from sarcoid myopathy and fasciitis-panniculitis syndromes. Whipple's disease, Mycobacterium avium intracellulare infection, and malakoplakia could not be confirmed. Ten patients were treated with various combinations of steroids and antibiotics; symptoms improved in eight patients, and stabilised in two. A new inflammatory muscle disorder of unknown cause, characterised by a distinctive pathological pattern of macrophagic myofasciitis, is emerging in France.

The fasciitis-panniculitis syndrome in circulatory disorders of the limbs

Sporadic reports allude to the similarities between the subcutaneous and fascial sclerosis of venous disease and lymphedema with that of eosinophilic fasciitis and the related fasciitis-panniculitis syndrome. The objective of the present study was to compare the clinical and histological features of the subcutaneous induration in patients with impairment of the venous, arterial, and lymphatic circulation (6 patients) with those of patients with the fasciitispanniculitis syndrome of diverse etiologies (12 patients). The semiquantitative analysis of the microscopic changes evinces the equivalent intensity of subcutaneous septal fibrosis, inflammation and mucinosis, lobular panniculitis, fascial fibrosis and inflammation, as well as microscopic phlebitis and phlebosclerosis in both groups of patients. The similarities of the pathological alterations of the subcutaneous layers are indicative of a shared reaction pattern to a variety of insults. In the patients with circulatory disorders, analysis of the clinical circumstances establishes the convergence of synergistically acting factors in the causation of the fasciitis-panniculitis syndrome. The circulatory impediment is insufficient to induce the syndrome, but it apparently acts as a predisposing factor such that it amplifies the inflammatory-cicatrizing response to one ordinary damage or another. Awareness of the multifactorial etiopathogenesis of the fasciitis-panniculitis syndrome in patients with circulatory disturbances ushers in a novel perspective of therapeutic intervention at multiple levels.

The Fasciitis-Panniculitis Syndromes Clinical and Pathologic Features

The authors propose to encompass under the designation of "fasciitis-panniculitis syndromes" (FPS) a group of disorders characterized by induration of the skin due to chronic inflammation and fibrosis of the subcutaneous septa and muscular fascia. The prototype of the FPS is eosinophilic fasciitis. Thirty-two consecutive patients with FPS were cared for at the author's hospital during a 10-year period. The association of the FPS with other diseases, clinical presentations, histologic features, and response to treatment were analyzed. Idiopathic FPS, that is, eosinophilic fasciitis, was diagnosed in 14 patients. In the remaining 18 cases, the FPS were ascribed to vascular disorders (n = 6), infections (n = 6), and neoplastic disorders (n = 3), while trauma, insect bites, and Sweet syndrome antedated the FPS in 1 patient each. The lesions had a sleeve-like distribution in 20 patients, plaque-like distribution in 7, and a combined pattern in 5. Skin biopsies revealed lesions in the deep subcutaneous layers with the pathologic triad of septal and fascial fibrosis, chronic inflammatory infiltration, and small-vessel vasculopathy. Spontaneous improvement occurred in 4 cases. Following cimetidine monotherapy, complete remission was achieved in an additional 3 of 5 patients. The concept of the FPS serves to advance our understanding on several fronts: emphasizing the clinical and etiologic diversity; recognizing a stereotypic tissue reaction pattern; highlighting the panniculitis in addition to the fasciitic component; and describing a similar response to drug therapy in different clinical settings. Based on the results of the present series, cimetidine may be recommended as first-line treatment.

The fasciitis-panniculitis syndrome: Clinical spectrum and response to cimetidine

The term fasciitis-panniculitis syndrome (FPS) is proposed as a novel compilation encompassing several disorders, common to which is subcutaneous induration caused by cicatrizing fasciitis as well as septal and lobular panniculitis and perimysial fibrosis. Included herein are Shulman's eosinophilic fasciitis, morphea profunda, lupus profundus, venous lipodermatosclerosis, toxic oil syndrome, altered tryptophane-related eosinophilic myositis, graft-versus-host reaction, and fasciitis reactive to subjacent basal cell carcinoma. FPS should be differentiated from scleroderma, which primarily affects the dermal structures and in which arterioles are injured. In contrast, vasculopathy of the subcutaneous medium-sized veins accompanies the hypodermal lesions of FPS. The importance of recognizing and grouping these disorders lies in their different histopathology, characterization as reactive phenomena, enhanced responsiveness to treatment, and better prognosis than scleroderma. In view of the excellent prognosis of FPS, steroid treatment is not warranted. Long-term therapy with cimetidine appears to benefit the majority of patients.

The phlebopathies of the fasciitis panniculitis syndrome.

The fasciitis-panniculitis syndrome is histologically characterized by fibrous thickening and inflammation of the subcutaneous septa, fascia and perimysium. These morphological features were the common denominator in 13 patients with Shulman's eosinophilic fasciitis, scleroderma profunda, venous lipodermatosclerosis, induration following recurrent erysipelas and erysipeloid erythema nodosum. Small to medium-sized vessel phlebitis was found in the subcutaneous and perimysial tissues of 11 of the patients and subcutaneous phlebosclerosis was encountered in all cases. These data contrast with the low incidence of phlebopathy reported by others in these disorders.

Treatment with cimetidine of atypical fasciitis panniculitis syndrome.

Three patients presented with septal fasciitis and panniculitis, associated with clinical and laboratory features which precluded straight-forward classification into eosinophilic fasciitis, localised scleroderma, or lupus erythematosus profundus. Treatment with cimetidine caused the remission of cutaneous manifestations and the extracutaneous abnormalities, such as nailfold capillary disturbances and the presence of antithyroid antibodies, improved. It is concluded that features of eosinophilic fasciitis or localised scleroderma and certain additional atypical elements should be categorised as atypical fasciitis-panniculitis syndrome.