Initially, the diagnosis of gottrons papules was considered and an analytical evaluation was performed, revealing normal whole blood count, erythrocyte sedimentation rate 9 mmh and creactive protein 5. Following the patient s inadequate response to corticosteroid. Histopathology of gottrons papules utility in diagnosing. Erythematous and hyperpigmented macules on the chest.
Biopsy reveals perimysial inflammation with perifascicular atrophy. Gottron s punch biopsies from clinically characteristic papules, which are violaceous papules or gottron s papules were obtained from 11 paplaques occurring over the joint spaces of the tients with known dermatomyositis table 1. Histologically, it is characterized by the presence of lymphocytic vascular inflammation and endothelial swelling. Differential diagnosis in surgical pathology second edition, 2010. Dyspnea associated with dermatomyositis respiratory care. Histopathology of gottron s papules j cutan pathol.
Systemic sclerosis is a multisystem form of scleroderma with hallmark skin signs. The changes illustrated in the following images are indicative of damage at the site of gottron s papules. Adultonset dermatomyositis, dermatopolymyositis in adults, wagnerunverricht syndrome in adults, polymyositis with skin involvement in adults. Of them, gottron papules have been the most pathognomonic, while the other skin findings are less specific and can be seen in other disease entities. Gottron sign redness over the backs of the hands, elbows, and knees. Eruptions between the mcp and ip s joints may also be seen and be associated with scale and ulcerations. As in typical poikilodermatous dermatomyositis, basal layer vaculopathy, paspositive basement membrane thicken. Cutaneous stigmata of dm include gottron s papules, similarly colored papules and plaques overlying the extensor surfaces of finger joints. How are gottron papules in dermatomyositis characterized. While biopsy of the typical poikilodermatous skin eruption found in patients with suspected dm is a standard algorithmic component in the workup and diagnosis of the disease, gottron s papules are rarely sampled for histopathologic assessment. Skin biopsy demonstrates a reduced capillary density, deposition of mac on small bloodvessels along the dermalepidermal junction, and variable kerotinocyte decoration 1 mac 3. We studied gottron s papules in 11 cases of known dermatomyositis to determine whether the findings were similar to those in the.
Gottrons papules, but not non gottrons dm lesions, exhibit abundant accumulation of cd44v7 in the papillary dermis to study the effect of location versus disease in our patient samples, we acquired biopsies from the skin overlying the fourth proximal ip pip joint of five healthy volunteers with no history of autoimmune disease. Violaceous macules and patches on the upper back and shoulders. Rashes typical of dermatomyositis, which include heliotrope rash, gottron sign, and gottron papules the fifth criterion is what differentiates dermatomyositis from polymyositis. Case 7 78 year old man with first degree heart block. Based on symptoms, blood tests, electromyography, muscle biopsies. Morphological descriptions of these lesions are mainly focused on changes of the epidermis and interface, and on the nature of the inflammatory infiltrate. The most frequent clinical manifestations of jdm are gottron papules, heliotrope rash, malar rash, and proximal muscle weakness. Histopathology of gottrons papules, journal of cutaneous. Histopathology of gottrons papules hanno 1985 journal. Antimda5 dermatomyositis mimicking psoriatic arthritis. Of the 26 patients with ulcerative gottron papules gottron. We examined non gottron s dm lesions and gottron s papule skin biopsies for c4s, cd44 variant 7 cd44v7, a chondroitin sulfatebinding isoform causally implicated in autoimmunity, and osteopontin opn, a cd44v7 ligand implicated in chronic inflammation. B atrophic palmar papules suggestive of inverse gottron papules and annular, scaly, erythematous plaques with resolving postinflammatory hyperpigmentation suggestive of subacute cutaneous lupus on arm and forearm.
Discrete, lichenoid papules, typical of gottron papules, were seen over the interphalangeal joints fig. In this paper, i will present the case of a 48yearold female patient whose dermatomyositis was initially diagnosed as vasculitis. Inverse gottron papules in a patient with sle a confluent psoriasiform plaques on the upper and lower back with scattered erythematous, annularpolycyclic lesions on upper limbs. Muscle biopsies in dermatomyositis and polymyositis. Dermatomyositis dm is an uncommon connective tissue disease that presents with a characteristic violaceous skin eruption as well as proximal muscle weakness, primarily of the upper extremities. Gottron papules show histopathologic features of localized. She cannot comb her hair or climb the stairs due to muscle weakness. Hallmark cutaneous skin manifestations of dermatomyositis including a heliotrope rash and b. View the article pdf and any associated supplements and figures for a period of 48 hours. We intend to study the main histopathologic features of gottron papules in a series of 12 biopsies.
Lab results show an increased creatine kinase, positive antinuclear antibody ana and antijo1 antibody. The onset is slow, over months in the majority of patients, but some people have. As in typical poikilodermatous dermatomyositis, basal layer vaculopathy, pas. Request pdf histopathology of gottrons papules utility in diagnosing dermatomyositis dermatomyositis dm is an uncommon connective tissue disease. All the patients presented with proximal myopathy, which was. Dermatomyositis as the first manifestation of gallbladder. The histopathology of gottron s papules has not been often reported. A case of erythrodermic dermatomyositis associated with gastric. Histopathology of gottron s papules utility in diagnosing dermatomyositis. Gottrons papules exhibit dermal accumulation of cd44 variant. Histopathology of gottrons papulesutility in diagnosing. Juvenile dermatomyositis jdm is a systemic inflammatory disease involving children, which primarily affects the skin and the musculoskeletal system.
Gottrons papules are erythematous to violaceous slightly scaly. Gottron s papules violaceous papules over the knuckles, four 50% had periorbital edema, and one 12. It is associated with a high mortality and morbidity rate. We studied gottron s papules in 11 cases of known dermatomyositis to determine whether the findings were similar to those in the poikilodermatous eruption usually biopsied in this disease. Gottrons papules exhibit dermal accumulation of cd44 variant 7 cd44v7 and its binding partner osteopontin. Gottron s papules are a particularly resistant manifestation of dm that often persist after other lesions have resolved with therapy. Dermatomyositis symptoms, diagnosis and treatment bmj. However, with the patients developing clinical picture of palmar annular lesions and her pathology results showing cornoid lamellae a hallmark of porokeratosis. Violaceous, scaling papules on the skin overlying the joints and proximal nailfolds. Interestingly, confluent macular violaceous erythema. Unpruned model of prediction without histology criteria. Gottrons papules are described as erythematous to violaceous papules found symmetrically over the extensor aspects of the metacarpophalangeal mcp and interphalangeal ip joint. From peter and bohan in 1975 to the eularacr classification criteria of 2016, the heliotrope rash, gottrons papules, and gottrons sign have endured as the indisputable cutaneous hallmarks of dm 1,2.
The case of childhood dermatomyositis developed subcutaneous calcinosis around her knees. While biopsy of the typical poikilodermatous skin eruption found in patients with suspected dm is a standard algorithmic component in the workup and diagnosis of the disease, gottron s papules are rarely. Although gottron papules are a relevant feature of dermatomyositis, they are reported in very few papers in the literature, and the largest series on the subject includes 11 cases. Histopathology is helpful in differentiating it from other skin diseases, but.
The characteristic findings include gottron papules, heliotrope rash, calcinosis cutis, and symmetric proximal muscle weakness. Werth2,3 the accumulated mucin in non gottrons dermatomyositis dm lesions is primarily chondroitin4. Pdf dermatomyositis with a pityriasis rubra pilarislike. Request pdf histopathology of gottron s papules utility in diagnosing dermatomyositis dermatomyositis dm is an uncommon connective tissue disease that presents with a characteristic. The histology of gottrons papules is similar, and often shows a lymphocytic infiltrate, epidermal hyperkeratosis, papillomatosis, acanthosis and less epidermal atrophy. Photograph showing gottron papules over the extensor surfaces of. Gottrons papules exhibit dermal accumulation of cd44. Case report a yearold caucasian boy presented with various erythematous hyperkeratotic plaques on the extensor surfaces of. Although the diagnosis is clinical gottron papules are rarely sampled for histopathologic assessment. By contrast, gottrons biopsies demonstrated a mean 4. Their recognition is essential for an adequate diagnosis and posterior orientation. Cutaneous manifestations of dermatomyositis and their. A patient presents with a heliotrope rash, a malar rash and gottron s papules on the elbows and knees.
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