A case report of Parry Romberg Syndrome initially presenting as periodontitis

Parry Romberg Syndrome (PRS) is a rare disorder of progressive hemifacial atrophy, involving soft tissues, fat and occasionally bone. It can co-exist with presentations of Morphea. We describe an unusual case of persistent periodontal and alveolar destruction associated with PRS. A 56-year-old African female initially presented with persistent periodontal destruction, which showed minimal response to conventional periodontal treatment. After non-surgical treatment, surgical debridement followed by extraction of the two right maxillary incisor teeth was required to halt the periodontal destruction. Atrophy was not limited to the periodontal tissues. Multidisciplinary care and extensive investigations were required to diagnose PRS. Once the PRS has stabilised, adipose tissue transplants will be required to improve the facial appearance. We highlight the need for extensive investigations and a multidisciplinary approach to diagnose rare systemic causes for recalcitrant periodontal disease

A case of eosinophilic fasciitis and generalized morphea overlap

A 60-year old man developed skin hardening and edema on his extremities. Although he had been treated with oral prednisolone at another hospital, skin stiffness relapsed during tapering of prednisolone. At the initial visit to our department, physical examination showed skin hardening of the extremities and also symmetric erythematous macules on the back. Histological examination revealed fasciitis on the forearm and morphea on the back. Eosinophilic fasciitis is occasionally associated with morphea. However, cases of concurrent eosinophilic fasciitis and generalized morphea are rare. In the present case, CD34 was differentially expressed in both lesions, suggesting eosinophilic fasciitis and morphea are separate diseases with different origin of mesenchymal cells

Granulomatous fasciitis followed by morphea profunda: Is granulomatous fasciitis part of a spectrum of deep morphea? A case report and review of the literature.

Although eosinophilic fasciitis is known to be part of the deep morphea spectrum, this first report of the coexistence of granulomatous fasciitis and morphea profunda suggests that granulomatous fasciitis may also be a part of the spectrum of deep morphea

Laser therapy in superficial morphea lesions – indications, limitations and therapeutic alternatives

Morphea or localized scleroderma is an uncommon autoimmune and inflammatory disease which affects patients of any age. Even if morphea lesions present systemic symptoms as myalgias or arthritis, it is distinct from systemic sclerosis because it does not associate Raynaud’s phenomena or sclerodactyly, which are encountered in systemic scleroderma. The most common form of morphea in children is `en coup de sabre`, which can alter the local anatomy by deep tissue involvement. In contrast, the most frequent form that affects adults is represented by circumscribed morphea. The initial lesions present an inflammatory phase that manifests in the form of erythematous plaques, sometimes accompanied by edema. In later stages, the inflammation decreases and the lesions become sclerotic to atrophic. Therapy is most beneficial when initiated in the inflammatory stage. Topical application of high potency steroids along with phototherapy demonstrates the best results in the active phase of the disease. Localized superficial morphea can be treated with the excimer laser (using ultraviolet type B light, in range of 308nm) if topical steroid administration shows no significant clinical improvement. Phototherapy with ultraviolet light is capable of decreasing inflammation and may also have immunomodulatory effects

Eosinophilic fasciitis (Shulman's disease): review and comparative evaluation of seven patients.

OBJECTIVES: Eosinophilic fasciitis (EF) was described in 1974 by Shulman as a rare fibrosing connective tissue disease of unknown etiology. An undetermined trigger is thought to lead to the degranulation of eosinophils that interact with fibroblasts and express fibrogenic cytokines including the transforming factor of tumor growth a and b and interleukins 1 and 6. The purpose of this study was to summarize seven cases of EF in a central hospital. MATERIAL AND METHODS: This was a retrospective and descriptive study of a population with EF of a central hospital. All patients diagnosed with EF in a hospital unit were admitted to the study between January 1, 2005, and April 30, 2018. RESULTS: A total of seven patients diagnosed with EF were analyzed. The median age of the population at the time of diagnosis was 56 years, and 57% of the patients were women. All patients had elevated peripheral eosinophilia and sedimentation rate, and only one patient had hypergammaglobulinemia. All patients had edema and cutaneous thickening of the limbs, 57% had constitutional symptoms, and 57% had inflammatory arthritis with joint contracture. Prednisolone (PDN) therapy was initiated in all patients, and only in two was the association of PDN with methotrexate (MTX) initially performed. In one patient triple therapy of PDN, MTX, and cyclosporine was required. At the time of this publication, only one patient maintains active disease, and tocilizumab has been initiated. CONCLUSIONS: Recent studies show a more favorable response from the combination of PDN and MTX than from PDN alone. Considering the rarity of the disease, more long-term studies are needed regarding the etiopathogenetics, progression, recurrence of EF, and new effective therapies.info:eu-repo/semantics/publishedVersio

Bullous hemorrhagic lichen sclerosus of the breast: a report of two cases and review of the literature

Lichen sclerosus (LS) is a chronic dermatologic condition characterized by atrophic porcelain-appearing plaques that can cause intense discomfort and eventually lead to destruction of local architecture. Lichen sclerosus most commonly arises in the anogenital area, but up to 20% of cases can present on extragenital skin. Rarely, LS can also present with hemorrhage and bullae; the mechanism by which this occurs is unknown. We report two cases of bullous hemorrhagic LS of the breast arising in 66-year-old and 77-year-old women. Bullous hemorrhagic LS of the breast is exceedingly rare, with few reported cases, and presents a diagnostic challenge to clinicians

Abdominopelvic post-irradiation morphea in a prostate cancer patient: the first case of an under recognized condition

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How I explore ... the skin functional involvement in scleroderma

peer reviewedScleroderma refers to distinct clinical presentations sharing in common a sclerotic process most often clinically obvious on the skin. The involvement possibly affects the skin alone in morphea or in combination with internal lesions in systemic sclerosis. Some objective and non-invasive functional assessments are useful for better appreciating the severity and evolution of the disease, as well as to monitor the therapeutic efficacy. In this endeavour, in vivo measurements of the skin mechanical properties are unsurprisingly informative