The rash that presents as a red swollen face

A red swollen face can be a skin sign of a potentially life-threatening condition. We present in detail the main clinical presentations, diagnostic tests, and management of some of the most severe conditions that can frequently present as a red and swollen face: acute or recurrent angioedema, mast cell-driven or bradykinin-mediated angioedema, nonhereditary and hereditary angioedema, allergic or photoallergic facial contact dermatitis, contact urticaria, severe adverse drug reactions (particularly drug reaction with eosinophilia and systemic symptoms [DRESS]), skin infections (erysipelas, cellulitis, necrotizing fasciitis), and autoimmune diseases (dermatomyositis). There are many other conditions that also have to be considered in the differential diagnosis of a red swollen face.     

New aspects of the pathogenesis and treatment of Schulmann's eosinophilic fasciitis

A female patient, aged 64, was admitted to hospital with a tentative diagnosis of dermatomyositis or systemic scleroderma. Basing on the clinical symptoms and histologic findings in examination of a musculocutaneofascial biopsy specimen from the focus of involvement on the left thigh, Schulmann's eosinophilic fasciitis was diagnosed. The mechanism of the disease is polypathogenic, it includes newly detected disorders of the blood rheology, of the enzymic and electrolyte metabolism, of the immunologic homeostasis at the cellular (E0-RFC) and humoral (IgE) levels, of the peripheral skin analyzer function, of the orocaudal relationships, and of the microcirculation. Treatment with enzymic, vasoactive, and immunocorrective drugs combined with D-penicillamine and He-Ne laser therapy has yielded a good clinical effect without resorting to glucocorticoids; the time of the treatment was reduced to 28 days. A stable clinical remission for 1.5 years was achieved.     

Eosinophilic Fasciitis Occurring Four Weeks after the Onset of Dialysis in a Renal Failure Patient

BACKGROUND: Eosinophilic fasciitis is a rare, scleroderma-like disease that usually affects the extremities of young to middle-aged males. The disease may cause flexion contractures and limit joint mobility and is associated with peripheral eosinophilia. The fascia, by definition, is infiltrated with mononuclear cells and typically with eosinophils. Eosinophilic fasciitis may be separated from another sclerodermatous disorder, linear scleroderma, by its response to systemic corticosteroids. The etiology is unclear but eosinophilic fasciitis has numerous disease associations. However, it has not previously been associated with renal failure and hemodialysis. OBJECTIVE: This article reports a case of eosinophilic fasciitis occurring four weeks following the onset of hemodialysis. METHODS: The clinical and histologic features confirmed the diagnosis of eosinophilic fasciitis. He was treated with systemic corticosteroids with good response. CONCLUSION: This is the first reported patient who developed eosinophilic fasciitis in close temporal relationship with the start of hemodialysis. While eosinophilic fasciitis may be coincidental with a common disorder, namely, renal failure, it is interesting to note that hemodialysis patients often have immune-regulation abnormalities and peripheral eosinophilia.     

Dermatomyositis in a human immunodeficiency virus infected person

It is interesting to study an autoimmune condition like dermatomyositis (DM) in the setting of immunosuppression due to human immunodeficiency virus (HIV) infection. An HIV seropositive female aged 30 years, presented with a nonitchy rash over the face, breathlessness, diarrhoea and difficulty in raising her hands above her head. A heliotrope rash around the eyes, Gottron's papules and proximal muscle weakness were found to be present. C reactive protein, erythrocyte sedimentation rate and lactate dehydrogenase levels were raised, but creatinine phosphokinase and anti-nuclear antibody profile were normal. Her HIV serostatus was confirmed by Western blotting, keeping in mind the potential for false positive HIV serology in an autoimmune disorder. Her CD4 count was 379 cells/mm3. An X-ray of the chest showed bilateral pleural effusion with raised pleural fluid adenosine deaminase levels. Clinical findings and laboratory investigations favored the diagnosis of DM and HIV infection with tuberculous effusion in an HIV seropositive patient. She was treated with antibiotics, four-drug anti-tubercular treatment, systemic steroids and later, antiretroviral treatment. Chances of a false positive antibody test for HIV should be considered in a patient having an autoimmune disease such as DM.     

Polymyositis and dermatomyositis associated with malignancy: a 30-year retrospective study

BACKGROUND: Polymyositis and dermatomyositis in association with malignancy are paraneoplastic syndromes, but the incidence, treatment and factors that predict associated cancer and its prognosis all remain unclear. PATIENTS AND METHOD: During the 30-year period 1969-99, we treated 64 patients who had polymyositis (including two with cancer) and 28 patients who had dermatomyositis (including 10 with cancer). We compared the clinical findings of the patients who had cancer with the findings of those who did not have cancer. RESULTS: The risk of cancer is significantly higher in dermatomyositis and somewhat higher in polymyositis. An increased cancer risk was found in male patients with dermatomyositis who were older than 50 years. Cancer was diagnosed within 4 years before or after the diagnosis of polymyositis or dermatomyositis, and usually within 1 year. An operation was not possible in many of the patients with cancer because of the advanced stage of the disease. CONCLUSION: Our findings suggest that early discovery of malignancy is critical in cases of polymyositis and dermatomyositis.     

Dermatomyositis with a pityriasis rubra pilaris-like eruption: an uncommon cutaneous manifestation in dermatomyositis

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. We describe an 11-year-old girl with dermatomyositis who had additional clinical findings of pityriasis rubra pilaris. Over a year, she developed muscle weakness, increasing fatigue, and a markedly elevated creatinine kinase level in addition to her cutaneous eruption and was seen in our clinic for these complaints. A year earlier, when a generalized, scaly erythematous eruption had appeared, she had been diagnosed as pityriasis rubra pilaris clinically and histopathologically. Dermatologic examination found scaling erythematous plaques involving the trunk and upper and lower extremities. Islands of unaffected skin were intermingled with erythematous plaques that were characteristic of pityriasis rubra pilaris. A skin biopsy specimen showed the findings of dermatomyositis and that diagnosis was made. The laboratory findings, electromyographic pattern, and muscle biopsy were also consistent with dermatomyositis. Her presentation is interesting, as she had been diagnosed as pityriasis rubra pilaris both clinically and histopathologically 1 year earlier and, although the cutaneous lesions had not changed, a diagnosis of dermatomyositis was made a year later.     

Clinical manifestations of skin,lung and muscle diseases in dermatomyositis positive for anti‐aminoacyl tRNA synthetase antibodies

Patients with dermatomyositis positive for anti‐aminoacyl tRNA synthetase (ARS) antibodies, also known as antisynthetase syndrome (ASS), frequently present with mechanic's hand and interstitial lung disease (ILD). We first screened the antibody profiles of 59 patients with dermatomyositis, and then examined the cutaneous, muscular and pulmonary manifestations characteristic for patients with ASS. The anti‐ARS antibodies Jo‐1, PL‐7, PL‐12, EJ and KS, along with antibodies to TIF1‐γ, MDA5 and Mi‐2, were examined. Among the 59 patients, 20, 21, 15 and three patients were classified into the ASS, non‐ASS, myositis‐specific antibody‐negative and unknown groups, respectively. Five of 16 patients (31%) with ASS had six relatives with a history of collagen diseases, within the second degree of relationship, including two cases of dermatomyositis (vs the non‐ASS group, P = 0.018). Patients with ASS more frequently presented with fever and arthralgia, and had elevated levels of C‐reactive protein. Nine of the 11 finger lesions (82%) clinically diagnosed as mechanic's hands showed a psoriasiform tissue reaction. ILD was observed in 19 of 20 patients (95%) with ASS, and eight of 21 patients (38%) in the non‐ASS group, in which six patients possessed anti‐MDA5 antibody. Patients with ASS showed higher serum levels of muscle enzymes, and four of 12 patients (33%) had fasciitis‐dominant myopathy, while only one of 11 patients (9%) in the non‐ASS group had fasciitis‐dominant myopathy. Patients with ASS often present with a psoriasiform tissue reaction in the hand lesions and fasciitis‐dominant myopathy, and the relatives of those with ASS are at high risk for collagen diseases.     

Cranial fasciitis of childhood: a case report and review of literature

Cranial fasciitis is a rare reactive, non-tumoral lesion (pseudosarcoma) that develops in childhood. It is a subset of nodular fasciitis that preferentially involves the scalps of children. This report presents a case of 24-month-old boy with a previous history of head trauma. A firm nodule, showing rapid growth over 1-month period, was seen at the occipital region. Computerized tomography showed the absence of erosion of the skull bones. The mass was completely excised with ample safety margin. Histologically, the growth was composed of mitotically active spindle-shaped cells embedded in a myxoid matrix. The lesional cells were immunoreactive for smooth muscle actin and the diagnosis of cranial fasciitis was established. No evidence of recurrence was seen 18 months after the diagnosis. This report describes the salient features of the case and discusses the relevant literature. It stresses the fact that cranial fasciitis is a rare lesion that must be included in the differential diagnosis of pediatric skull tumors. It also emphasizes that the correct diagnosis of these lesions is needed to avoid unnecessary surgery and radiotherapy.     

Dermatomyositis and mucinosis

BACKGROUND: Mucin deposition is a common feature in autoimmune collagen diseases including dermatomyositis. Nevertheless, clinical manifestations of mucinosis are uncommon in patients with dermatomyositis. Two cases of mucinosis associated with dermatomyositis are reported. PATIENTS: A 53-year-old woman presented with symmetrical plaques on the upper limbs formed by the coalescence of small, violaceous papules. In addition, she showed the typical cutaneous and muscle features of dermatomyositis. A 44-year-old woman with dermatomyositis of 5 years' evolution developed linear, flesh-colored papules across the flexural creases of her palms and fingers. RESULTS: Skin biopsy of the upper limb lesions in the first patient showed epidermal changes compatible with dermatomyositis and dermal mucin deposition. Histopathologic examination of the palmar lesions of the second patient showed less intense epidermal changes of dermatomyositis and dermal mucin deposition. CONCLUSIONS: Mucin deposition in patients with dermatomyositis may have an unusual clinical presentation, and it should be considered in the differential diagnosis of atypical cutaneous lesions in these patients.     

Fulminant group A streptococcal necrotizing fasciitis: clinical and pathologic findings in 7 patients

BACKGROUND: Necrotizing fasciitis is a rapidly progressive soft tissue infection with high morbidity and mortality rates. Examination of deep incisional biopsy specimens can provide prompt diagnosis and improve survival. We describe 7 patients with necrotizing fasciitis caused by group A Streptococcus species. OBJECTIVE: Our purpose was to describe the unique dermatopathology and clinical features in 7 patients with necrotizing fasciitis caused by group A Streptococcus. METHODS: We conducted a retrospective review. RESULTS: The average age of the patients was 47 years. Fasciitis occurred on an extremity in all cases. All 5 patients with streptococcal toxic shock syndrome died of their disease. The histopathologic findings from early fascial disease revealed superficial epidermal necrosis, edema, and hemorrhage with few inflammatory cells, whereas clinically advanced, necrotic skin lesions revealed diffuse necrosis, thrombosis, neutrophilia, and numerous gram-positive diplococci. CONCLUSIONS: Patients with clinical features of necrotizing fasciitis should have a deep incisional biopsy specimen obtained from the central area of ecchymotic, necrotic plaques to confirm the diagnosis. Immediate surgical intervention is necessary to reduce the morbidity and mortality rates associated with necrotizing fasciitis.     

The value of frozen section biopsy in diagnosing necrotizing fasciitis: proposal of a new grading system

Goals and objectivesNecrotizing fasciitis is a serious disease entity, with only limited pathognomic features.PurposeIn necrotizing fasciitis a rapid diagnosis is necessary in order to reduce morbidity and mortality. In this study the investigation focused on specific features in the frozen section biopsy for diagnosing necrotizing fasciitis.MethodsA total of ten patients are described with final pathological examination of resected tissue.ResultsA new grading system is suggested for frozen section biopsy in patients with necrotizing fasciitis. In the herein reported study it was found that granulocytes were present in both the frozen section biopsy and in the definitive paraffin coupes, in the subcutis and fascia layer.ConclusionFrozen section biopsy could be useful in diagnosing necrotizing fasciitis.     

小儿寻常型银屑病伴发皮肌炎1例

患儿男，9岁，头皮、凹肢、躯干部鳞屑性斑块4年。病理报告符合寻常型银屑病，面部水肿性红斑伴肌痛、肌无力2年。肌酶指标升高，肌电图示广泛肌源性损害神经电图示运动神经传导速度在正常范围内。诊断为寻常型银屑病伴发皮肌炎。     

Cutaneous tuberculous gummas in a patient with polymyositis

Metastatic tuberculous abscesses or cutaneous tuberculous gummas occur mostly by reactivation of ancient cutaneous foci and is triggered mainly by pharmacological immunosuppression, diabetes mellitus, alcoholism or acquired immunodeficiency syndrome. The present case report refers to a 33-year-old male patient with polymyositis and a previous diagnosis of treated pulmonary tuberculosis. He presented cutaneous abscesses and fever months after the tuberculosis regimen. The patient was diagnosed as metastatic tuberculous abscesses associated with immunosuppression as result of polymyositis treatment.     

Relapse of dermatomyositis after 10 years in remission following curative surgical treatment of lung cancer

A 55-year-old woman with dermatomyositis and small cell lung cancer was successfully treated with surgery followed by combination chemotherapy in 1987. She had been in remission without further immunosuppressive therapy for 10 years. However, myositis with cutaneous manifestations specific for dermatomyositis relapsed when the patient was 69 years old. Intensive examinations revealed no neoplasm, and she responded to a moderate dose of systemic corticosteroids. This case suggests a long-lasting autoimmune abnormality in dermatomyositis and that a neoplasm is an important factor in eliciting an occult dermatomyositis.     

Eosinophilic fasciitis treated with psoralen-ultraviolet A bath photochemotherapy

Eosinophilic fasciitis is a rare disorder which can markedly affect the quality of life in individual patients. So far, no generally accepted and effective treatment modality has been available. Although the precise nature of eosinophilic fasciitis is still unknown, it is often regarded as a variant of localized scleroderma (morphoea). Phototherapy and photochemotherapy have been shown to be effective in the treatment of sclerodermatous skin lesions. We report a patient with eosinophilic fasciitis which was successfully treated with psoralen plus ultraviolet A bath photochemotherapy within 6 months.     

Eosinophilic fasciitis as a paraneoplastic phenomenon associated with metastatic colorectal carcinoma

A 72-year-old man presented with erythema and induration of his calves and forearms. He had a past history of stage 1 colorectal carcinoma, treated with resection and primary anastamosis 4 years earlier. A diagnosis of eosinophilic fasciitis was made based on the characteristic clinical appearance, peripheral blood eosinophilia and a skin biopsy. There was no improvement in the condition following treatment with prednisolone or methotrexate. One year later, abnormal liver function studies were noted, and an abdominal computed tomography scan and subsequent needle biopsy of the liver confirmed a neoplastic lesion in the liver consistent with a metastatic colorectal carcinoma. Systemic chemotherapy with oxaliplatin, 5-fluorouracil and capecitabine was commenced, and resulted in partial remission of the colorectal carcinoma. Simultaneously, the indurations of the forearms and calves also improved, suggesting that the eosinophilic fasciitis was a paraneoplastic phenomenon.     

Dermatomyositis. Disease associations and an evaluation of screening investigations for malignancy

Fifty-three adult patients (19 men, 34 women) with dermatomyositis were studied. Two had dermatomyositis associated with benign disorders. Twenty-three (43%) had a malignancy; the risk of malignancy increased with age, but there was no sex difference. Seven malignancies were recurrences and 9 were diagnosed during investigation of dermatomyositis; these 16 were suspected clinically or from abnormal results of simple investigations. Extensive screening tests did not increase the number of malignancies diagnosed. In 7 patients, a diagnosis of malignancy was made more than 9 months after onset of dermatomyositis, although a relationship between malignancy and dermatomyositis was uncertain in two cases; the diagnosis of gynecological malignancy was missed in 2 patients despite appropriate investigations, 1 patient had poorly controlled dermatomyositis, and in 2 patients late diagnosis of malignancy was due to failure to reinvestigate relapse of previously stable dermatomyositis.     

Myxoid intravascular fasciitis

Nodular fasciitis is a common reactive proliferation of soft tissue. It is composed of high density of fibroblasts and myofibroblasts, and sometimes shows a sarcomatoid appearance histologically. A rare variant of nodular fasciitis, intravascular fasciitis can be found in the veins or arteries. Histologically, intravascular fasciitis has been described that resembles nodular fasciitis with a less prominent mucoid matrix. Here, we report a case of intravascular fasciitis with prominent mucin deposition. Clinically, it presented as multiple nodules on the leg of the patient. Histologically, it showed proliferation of spindle cells in a vein of the dermis and Alcian blue staining confirmed prominent mucin deposition between the spindle cells. Immunohistochemistry studies showed the spindle cells were positive for vimentin, actin and smooth muscle actin, but negative for keratin, S100 protein, desmin, CD31 and CD34. Based on the clinical and histopathological properties, a diagnosis of myxoid intravascular fasciitis was made. Wang L, Wang G, Wang L, Gao T. Myxoid intravascular fasciitis.     

Cutaneous changes of dermatomyositis precede muscle weakness

A retrospective review of 50 patients with dermatomyositis was performed to determine the temporal relationship between onset of muscle weakness and skin involvement. We found that cutaneous changes sometimes preceded muscle weakness more than a year before the onset of muscle weakness. These findings suggest that the characteristic dermatomyositis eruption without muscle weakness should not preclude a diagnosis of dermatomyositis, and these cases should be carefully followed.