A rare case report leelavathy budamakuntla, dipali malvankar department of dermatology std and leprosy, bowring and lady curzon hospitals and bangalore medical college and research institute, bangalore, karnataka, india. Morphea morfeeuh is a rare skin condition that causes painless, discolored patches on your skin. This causes confusion with systemic sclerosis scleroderma often resulting in unnecessary evaluation and anxiety. This girl is a walking encyclopidia whose dad was a veterinarian so she learned a lot of medicine from him so go to.
Morphea is a skin condition that causes patches of reddish skin that thicken into firm, ovalshaped areas. Juvenile case of multiple morphea profunda resulting in. Indian dermatology online journal extensive morphea. Pdf extensive morphea profunda with autoantibodies and benign. Morphea profunda may mimic localized lipodystrophy which in some cases may be iatrogenic following steroid injections. Morphea is a chronic autoimmune disease characterized by sclerosis of the skin. Solitary morphea profunda smp is a variant of localized scleroderma ls. Facial solitary morphea profunda presenting with painful. The study, entitled, prospective evaluation of frequency of signs of systemic sclerosis in 76 patients with morphea was conducted by researchers from france. A 43yearold woman developed an oval lesion on the right cheek. Morphea genetic and rare diseases information center. Histopathological features in morphea localized scleroderma and their clinical correlates are poorly described.
It is a form of scleroderma patches most often occur on the abdomen, stomach, and back, and sometimes on the face, arms and legs. It is characterized by an insidious onset of one or more oval or round circumscribed areas of induration, which are larger than 1 cm in diameter, and varying degrees of pigment changes. The cochrane library, medline and embase from inception until may of 2011 were searched using the key words morphea and morphea treatment. Systematically describe histologic changes of morphea in a large, well annotated cohort and determine the association between histopathology. Morphea morphoea is an immunemediated disease in which excess synthesis and deposition of collagen in the skin and underlying connective tissues results in hardened cutaneous areas. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. Morphoea american spelling, morphea is characterised by an area of inflammation and fibrosis thickening and hardening of the skin due to increased collagen deposition. Morphea en plaque morphea en plaque, the most prevalent of the plaque group, involves only one or two anatomic sites that is, the back, upper extremity, lower extremity, buttock, face and neck, or scalp. Morphea is classified according to the localization of the lesions and the depth of tissue involvement into localized or circumscribed limited to one or. Morphea profunda, international journal of dermatology. Localized scleroderma localized morphea morphea lichen sclerosus et atrophicus overlap generalized morphea atrophoderma of pasini and pierini pansclerotic morphea morphea profunda linear scleroderma.
Extensive morphea profunda with autoantibodies and benign tumors. Have you read the information available on the roadback site. Concurrent development of dermatomyositis and morphea profunda. Morphea coexisting with other systemic autoimmune diseases such as primary. Morphea profunda involves deep subcutaneous tissue, including fascia, and there is a clinical overlap with eosinophilic fasciitis, eosinophiliamyalgia syndrome, and the spanish toxic oil syndrome 171 morphea profunda shows little response to corticosteroids and tends to. Our treatment plan involved oral methotrexate and topical. The term scleroderma covers various types of morphoea and systemic sclerosis. Typically, the skin changes appear on the abdomen, chest or back. Some authorities feel that linear morphea and progressive facial hemiatrophy parry romberg syndrome are related conditions.
The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea. Hashimoto, et tence of generalizad morphea with histological changes in. It is differentiated from systemic sclerosis scleroderma by the absence of sclerodactyly, raynaud phenomenon, nail fold capillary changes, and organ involvement. Morphea localized scleroderma is a rare chronic inflammatory disease, which involves skin and subcutaneous tissues and is characterized by skin induration and sclerosis. Early and latestage morphea subtypes with deep tissue. Linear morphea is the most common subtype of morphea in children. Update on management of morphea localized scleroderma in. Linear morphea with inflammatory myositis pdf free download. Treatment for linear morphea in child scleroderma inspire.
Study results indicate that morphea and systemic sclerosis. Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. The predominant histopathologic features were 1 thickening and hyalinization of collagen bundles in the deep dermis. Atrophodermalike guttate morphea mdedge dermatology. Morphea profunda refers to inflammatory and sclerotic lesions that start primarily from the deep dermis, subcutaneous fat and fascia. This is a temporary file and hence do not link it from a website, instead link the url of this page if you wish to link the pdf file. Subtypes of morphoea vary according to the location. Morphea co existing with other systemic autoimmune diseases such as primary biliary cirrhosis, vitiligo, and systemic lupus erythematosus lend support to morphea. Thickening and hyalinization of connective tissue of deep dermis, subcutaneous fat and muscular fascia, with perivascular and focal interstitial lymphocytic and plasma cell infiltrate in subcutaneous fat.
Morphea, also known as localized scleroderma, is a disorder characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissues, or both. Morphea has different clinical features according to the subtype and stage of evolution of the disease. Morphea profunda, eosinophilic fasciitis, and disabling pansclerotic morphea of children are included in this group, but overlapping of the extent and depth of. Solitary morphea profunda following trauma sustained in an. Abstract morphea profunda is a rare skin condition of unknown etiology, involves deep subcutaneous tissue, including fascia. After 1 year, she noticed constant pulling pain and episodes of lancinating pain, both spontaneous and triggered by chewing and cold drinks.
View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Twenty to thirty percent of morphea begins in childhood, but it can occur at any age. Pdf morphea profunda is a rare skin condition of unknown etiology, involves deep. Morphea subtypes with deep tissue involvement is a rare type of morphea mainly affecting women which is often progressive and debilitating and may be generalized. Morphea, also known as localized scleroderma, is a rare, fibrosing skin disorder caused by the dysregulation of collagen production. We report the case of a 50yearold white woman with a history of trauma sustained in an automobile accident who presented with smp on the right upper arm. The term deep morphea describes a variant of morphea localised scleroderma in which inflammation and sclerosis are found in the deep. Histopathological changes in morphea and their clinical. Evidencebased treatment strategies in morphea are lacking.
The term localized scleroderma is also used in an attempt to highlight the systemic features of morphea. It frequently involves the deep dermis and subcutaneous tissue and may occasionally extend and involve the fascia and superficial muscle. Morphea and lichen sclerosus fitzpatricks dermatology. Morphea is a skin condition that involves a patch or patches of discolored or hardened skin on the face, neck, hands, torso, or feet.
I think you should check this out with one of the volunteers there. Hence, the diagnosis tilts more toward the diagnosis of morphea profunda. We report a 10yearold girl with a history of morphea since age 4, with plaques involving multiple body sites and some lesions overlying joints. Morfea genetic and rare diseases information center. The case is reported in view of extensive morphea profunda without systemic involvement, occurring in a male, with raised rheumatoid factor levels, with associated benign tumours, i. Patients with morphea do not have raynaud phenomenon fingers turning red, white, and blue with cold exposure, redness and swelling of both hands red puffy hands, changes in the blood vessels along the nail edge nailfold capillary changes, or tightening of the skin of the fingers sclerodactyly. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. The case is reported in view of extensive morphea profunda without systemic involvement, occurring in a male, with raised rheumatoid factor levels, with associated benign tumours. Herein, we report the case of a patient with severe disabling generalized deep morphea who showed a.
A study recently published in the online journal clinical and experimental rheumatology is challenging the clinical assumptions that morphea and systemic sclerosis ssc should be considered the part of the same disease spectrum. Pdf the term deep morphea describes a variant of morphea localised. Morphea profunda is a rare skin condition of unknown etiology, involves deep subcutaneous tissue, including fascia. Frequent atrophy of adnexal structures, increased fibroblasts, thickening and luminal narrowing of small vessels, blunting of dermalsubcutis. Morphea is classified into circumscribed, generalized, linear, and pansclerotic subtypes according to the clinical presentation and depth of tissue involvement. The condition is rare and thought to affect less than 3 out of. Extracorporeal photochemotherapy for generalized deep morphea. Morphea, or localized scleroderma, can at times constitute a severely disabling and aggressive disease with no single therapy proven to be curative or the standard of care. Extensive morphea profunda with autoantibodies and benign. This is likely due to use of different classification systems. Morphea selected local treatment methods and their effectiveness. The linear form of atrophoderma linear atrophoderma of moulin lacks the inflammation and induration associated with linear morphea. She was diagnosed with solitary morphea profunda and ct scan, ultrasonography, cranial mri and biopsy were completed.
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