Studies in Cutaneous Immunofluorescence

Twenty patients with rheumatoid arthritis (RA) had a biopsy taken from clinically normal skin. These were examined for histological and immunological abnormalities and were compared to those of 8 patients with osteoarthritis (OA). No 'lupus band' linear deposition of immunoglobulin or complement at the dermo-epidermal interface was seen in any patient in either group. Perivascular deposits were seen in 5 out of 20 (25%) patients with RA. These were of IgM in all 5 cases with additional C, in 2 cases (10%) and additional fibrin in one case (5%). No immunoprotein deposits were seen in specimens from any OA patient. 4 of the 5 patients with perivascular immunoprotein deposits had circulating ANAs present and dilutions of 1/256 or higher but normal DNA binding. A sparse perivascular, predominantly lymphoeytie infiltrate was seen in 13 out of 20 (65%) patients with RA and 3 of 8 (35%) patients with OA.     

Studies in Cutaneous Immunofluorescence

Forty-eight biopsy samples submitted to a diagnostic immunofluorescence service have been stored at - 20°C and sequentially re-tested at monthly intervals to ascertain the duration for which immunoglobulins and complement can be identified in these samples in typical and diagnostic patterns of distribution. Four biopsies from pemphigus vulgaris, 14 biopsies from bullous pemphigoid, nine biopsies from dermatitis herpetiformis and 21 from chronic discoid lupus erythematosus have been studied in this manner. In all cases diagnostic immunofluorescence patterns were present up to 4 months after biopsy and in several biopsies a diagnostic pattern was observed at 9 months. It is concluded that adequate storage facilities for a diagnostic immunfluorescence service can be achieved with a - 20°C storage system and that known positive material can be retrieved from this system for use as controls or in research studies for a minimum of 4 months.     

Management of Cutaneous Dermatomyositis

Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations and proximal muscle weakness. Patients with this condition frequently require treatment for muscular, systemic, and cutaneous involvement; however, the cutaneous symptoms often cause significant morbidity and frustration to patients. The skin lesions of dermatomyositis often persist or recur after treatment of myositis, and there are subsets of patients who have significant cutaneous disease with little or no muscle involvement. Only anecdotal evidence is available for cutaneous treatment, and the approach to therapy is guided by disease severity and refractoriness. First-line therapy for localized disease begins with topical agents such as antipruritics and/or corticosteroids, while widespread disease often requires initial systemic therapy. Antimalarials are generally used for significant skin disease, followed by other anti-inflammatory agents, systemic corticosteroids, corticosteroid-sparing immunosuppressants and, recently, biologics and intravenous immunoglobulin. While there is a recognized absence of randomized prospective clinical trials examining the optimal monotherapy and combination treatment for the cutaneous manifestations of dermatomyositis, we have tried to provide a review of the literature and a systematic approach to dealing with these often refractory and debilitating symptoms.     

Palmoplantar Hyperkeratosis: A Rare Cutaneous Manifestation of Juvenile Dermatomyositis

We report the case of a 12‐year‐old girl with juvenile dermatomyositis (JDM). She had a 12‐month history of palmoplantar hyperkeratosis that was initially treated as eczema. We wish to alert clinicians to this rare cutaneous manifestation of JDM.     

Successful Treatment of Cutaneous Lesions of Dermatomyositis with Topical Pimecrolimus

Dermatomyositis (DM) is an idiopathic inflammatory process characterized by proximal muscle weakness and cutaneous lesions, such as the Gottron''s sign, heliotrope rash, and erythematous photosensitive rash. Administration of systemic agents for the treatment of underlying systemic diseases leads to remission of the cutaneous lesions in many cases. However, cutaneous lesions may remain refractory to treatment. Pimecrolimus is a calcineurin inhibitor with combined anti-inflammatory and immunomodulatory activity. It has high affinity to the skin and low permeation potential, even in patients with acute skin inflammation and in those undergoing post-topical corticosteroid therapy. We herein report two DM patients whose cutaneous lesions were refractory to conventional treatment but showed dramatic response to topical pimecrolimus. The clinical outcomes suggest that topical pimecrolimus may be a good therapeutic alternative for the management of the cutaneous lesions of DM.     

Inverse Gottron's Papules: An Unusual Cutaneous Manifestation of Juvenile Dermatomyositis

Dermatomyositis is an autoimmune inflammatory myopathy characterized by unique cutaneous features. Gottron's papules are pathognomonic, lichenoid papules that can be found overlying the joints of the dorsal hand. Papules on the palms of the hand are less commonly seen, especially in the pediatric age group. Recognition of these inverse Gottron's papules as a sign of dermatomyositis is important as they may be the only cutaneous feature and may be a clue of underlying interstitial lung disease.     

Cutaneous Immunofluorescence Studies to Adult Rheumatoid Arthritis in Sun-exposed and Non-sun-exposed Areas

The incidence and significance of positive cutaneous immunofluorescence findings were assessed in biopsy specimens of both sun-exposed and non-sun-exposed skin of 34 adult patients with rheumatoid arthritis (RA) who were not receiving systemic corticosteroids. The incidence of lupus erythematosus (LE)-band was low (8.6%) in both groups. Twenty-eight percent of the patients had perivascular IgM and/or C3 deposits, and 74% had cytoid bodies in the papillary dermis. These studies indicate that the incidence of LE band is low in RA and that the detection of such a band in normal skin warrants close follow-up of RA patients for possible development of LE.     

43例皮肌炎患者皮损及肌肉直接免疫荧光检查分析

目的探讨皮肌炎的发病机理.方法采用抗人Ig(IgG、IgM、IgA和补体C3)DIF技术,检查了43例皮肌炎患者肌肉组织和其中21例皮肌炎患者皮肤损害的荧光分布.结果皮肌炎患者肌肉组织IgG、IgM、IgA和补体C3的阳性率分别为72.09%、23.26%、11.63%和51.16%,分布于肌膜;皮损区IgG、IgM、IgA和补体C3的阳性率分别为66.67%、19.05%、4.76%和61.90%,分布于基底膜.患者外周血IgG水平明显升高(70.83%).结论体液免疫亢进在皮肌炎的发病机理中起重要作用.     

Modification of the Cutaneous Dermatomyositis Disease Area and Severity Index,an outcome instrument

Background Validated outcome measures in dermatology help standardize and improve patient care. A scoring system of skin disease severity in dermatomyositis known as the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) has been developed. Objectives To simplify and improve the tool for clinical research and care, we modified the CDASI and validated the new version, v2. Methods The original CDASI has four activity and two damage measures. The modified CDASI has three activity and two damage measures. The skin disease of 20 patients with dermatomyositis was evaluated by the same dermatologist using both the original and the modified CDASI. Global validation measures were implemented to assess overall skin disease state, skin disease activity and skin damage. Spearman’s rho (r_sp), adjusted for multiple observations on subjects, was used to determine the relationship between the two versions of the CDASI and their correlation with the physician global measures (PGMs). Results The total score and activity and damage subscores of the original and the modified CDASI correlated perfectly with each other (r_sp = 0·99, 1·00, 1·00). The PGM‐overall skin scale correlated with the total scores (r_sp = 0·72, r_sp = 0·76) and activity subscores (r_sp = 0·68, r_sp = 0·63) but not with the damage subscores (r_sp = 0·14, r_sp = 0·15) of the original and the modified CDASI, respectively. However, the PGM‐activity and PGM‐damage scales correlated with the activity (r_sp = 0·76, r_sp = 0·75) and damage subscores (r_sp = 0·90, r_sp = 0·90), respectively, of the original and the modified CDASI. Conclusions The modified CDASI is perfectly correlated with the original CDASI. It has equally good concurrent validity with the PGM‐overall skin and PGM‐activity scales. The CDASI subscores have equally good concurrent validity with the PGM‐activity and PGM‐damage scales. We suggest that PGMs of skin disease activity and damage should be assessed separately for greater specificity. The modified CDASI is a refined and equally as useful outcome measure.     

Dermatomyositis

Ohne Zusammenfassung     

Dermatomyositis

A 51-year-old woman presented with weakness in her arms and legs and an eruption on the dorsal aspects of the hands, upper back, and face. Histopathologic features showed vacuolar alteration of the basal layer, a thick basement membrane, and deposits of connective-tissue mucin in the papillary dermis and the upper reticular dermis. Dermatomyositis is an idiopathic disease that is characterized by specific cutaneous manifestations and myopathy, which may be associated with occult malignancy. Treatment options include corticosteroids and other immunosuppressive agents such as methotrexate, cyclosporine, mycophenolate mofetil, and azathioprine.     

Dermatomyositis

Dermatomyositis is a disease that has a characteristic skin eruption that may occur with or without a proximal myopathy. The disease with cutaneous features only is classified as amyopathic dermatomyositis. The origin is unknown, but autoimmune factors are believed to play an important role. Autoantibodies are found in most patients and some have myositis-specific antibodies. Systemic changes may occur and there appears to be a relationship to internal malignancy, particularly in older patients. Juvenile disease has an associated vasculopathy. Treatment includes systemic corticosteroids and other immunosuppressive agents. The cutaneous changes may be difficult to treat.     

Dermatomyositis

ABSTRACT: Dermatomyositis (DM) is an acquired, inflammatory muscle disorder of unknown etiology characterized by symmetrical proximal muscle weakness, inflammatory myositis on biopsy, elevation of serum muscle enzymes, abnormal electromyogram, and a typical rash, with or without internal organ involvement. Untreated muscle disease can produce severe and long-term disabilities, and the cutaneous lesions can cause considerable discomfort from intractable pruritus. A prompt and usually aggressive approach to therapy is indicated, with each patient's management depending on the severity of the skin and muscle disease and the presence or absence of multisystem involvement. The mainstay of therapy for DM is the use of systemic corticosteroids. In those patients that do not respond to steroids or develop significant steroid-related side effects, immunosuppressive agents (methotrexate, azathioprine, cyclophosphamide, and more recently cyclosporine), or intravenous immunoglobulin (IVIG) are used. General measures include bed rest, passive range-of-motion exercises, and active physical therapy. Overall the use of corticosteroids with or without immunosuppressive agents has significantly reduced mortality and increased functional recovery. Corticosteroids may not control the rash. In these cases aminoquinolone antimalarials have been advocated, though partial improvement is more common than complete control. Emollients and photoprotection are essential. In recalcitrant cases of cutaneous DM, low-dose methotrexate and IVIG have been used with success. Finally, with the recognition of distinct subsets limited to the skin (e.g., amyopathic DM), the dermatologist is confronted with the decision for appropriate therapeutic modalities tailored to the individual patient's needs.     

Dermatomyositis

Dermatomyositis is one of the idiopathic inflammatory myopathies. It is characterized clinically by progressive symmetrical proximal muscle weakness and a characteristic rash. There are patients with rash who have little or no muscle disease. Although the process primarily attacks the skin and the muscles, it is a systemic disease with frequent manifestations in the gastrointestinal tract and pulmonary system. Dermatomyositis has been linked to internal malignancy in somewhere between 15% and 25%. Therapy for the muscle disease includes systemic corticosteroids with or without an immunosuppressive agent. Therapy of the skin disease begins with photoprotection and topical corticosteroids, but also includes the use of antimalarial agents and immunomodulatory therapies.     

Dermatomyositis

Ohne Zusammenfassung