Concluded Case

SYSTEMIC SCLEROSIS

Patient complains of stiffness in hands and feets along with pain in the lower left & right teeth back region since 1 year. H/O pain which is dull, intermittent & aggaravates while chewing food along with bluish discoloration of fingertips as a response to cold. H/O dysphagia, limitation of mouth opening , recent weight loss. Morning stiffness affecting both small and large joints was present. Patient was afebrile with Pulse rate: 76beats/min, Respiratory rate: 18 breaths/min & Blood pressure- 120/80mm of Hg. Tightening of the skin of extremities, claw-like the appearance of hands with hypopigmented areas on hands, knee, ankle, pre- and post-auricular region. Fingertips were pale, swollen, stiff and deformed. Claw like appearance of hands & feet. Extraoral examination revealed smooth, taut mask-like appearance of the facial skin and atrophied nasal alae giving rise to mouse facies appearance. Furrows are present in the peri-oral region. Maximum mouth opening: 28 mm inter-incisally. Right side two Submandibular lymph nodes were palpable which were non tender, mobile with size of 1.5cm & 2cms in maximum diameters without any localized rise in temperature. Overlying skin was pinchable. Intraorally, blanching was present on the buccal mucosa, soft palate, hypo pigmented areas on mandibular labial mucosa along with depappilisation and restricted movement of the tongue. Numerous blood vessels are seen on lower labial mucosa. Hand-Wrist radiography showed shortening of phalanges & soft tissue calcific deposits. w.r.t right and left side. Intra-oral periapical radiograph of 46, 47, 48 region revealed periodontal ligament space widening. OPG showed Proximal Caries(Deep): 16, Grossly decayed: 36 38 46 48, Root Stumps : 17 37. Based on history, clinical examination and investigations provisional diagnosis of systemic sclerosis (SS) was made. Kindly Suggest Differential Diagnosis and treatment plan for above mention condition of the patient. Thank you.

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Differential Diagnoses- CREST Syndrome Dermatologic Manifestations of Eosinophilia-Myalgia Syndrome Dermatologic Manifestations of Eosinophilic Fasciitis Dermatologic Manifestations of Graft Versus Host Disease Lichen Myxedematosus Lichen Sclerosus Scleredema SECTIONS Medical Care Different treatment regimens for systemic sclerosis exist. The therapeutic approach depends on the presentation of the disease and complexity of symptoms. In a recent review of the literature, calcium channel blockers, prostanoids, tadalafil, and bosentan received the strongest recommendations for their effectiveness for Raynaud phenomenon and digital ulcers. [47] In pruritus, the following agents are sometimes helpful: Camphor and menthol Topical emollients Psoralen UV-A (PUVA) treatment UVA-1 phototherapy
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Differential Diagnoses- CREST Syndrome Dermatologic Manifestations of Eosinophilia-Myalgia Syndrome Dermatologic Manifestations of Eosinophilic Fasciitis Dermatologic Manifestations of Graft Versus Host Disease Lichen Myxedematosus Lichen Sclerosus Scleredema SECTIONS Medical Care Different treatment regimens for systemic sclerosis exist. The therapeutic approach depends on the presentation of the disease and complexity of symptoms. In a recent review of the literature, calcium channel blockers, prostanoids, tadalafil, and bosentan received the strongest recommendations for their effectiveness for Raynaud phenomenon and digital ulcers. [47] In pruritus, the following agents are sometimes helpful: Camphor and menthol Topical emollients Psoralen UV-A (PUVA) treatment UVA-1 phototherapy
Thank you @Dr. Anil Sharma Sir for sharing detailed and informative views on this case.
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@Dr. Saloni Arora As it involves Multiple organs, it has multidisciplinary approach: Anti-inflammatory medicines: corticosteroids Immune-suppressants: methotrexate Blood pressure medications (particularly ACE inhibitors) for high blood pressure or kidney problems. Medications to treat Raynaud's phenomenon- calcium channel blockers Physical Therapy Management • Manage pain • Improve strength • Improve/maintain mobility Dd :Apart from all the above mentioned Sjogren syndrome, Raynaud's phenomenon , As these can occur isolated as well .
Thanks @Dr. Haritma Nigam mam, for your valuable opinion.
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Detailed and concise case description @Dr. Saloni Arora . The hallmark of scleroderma is clinical heterogeneity with subsets that vary in the degree of disease expression, organ involvement, and ultimate prognosis.Refined clinical phenotyping and careful early evaluation for active occult organ disease are the keys to deciding appropriate treatment options.
D/d- Eosinophilic fasciitis, lichen sclerosis atrophicus , systemic amyloidosis, nephrogenic systemic fibrosis, lipodermatosclerosis, Graft Vs host reaction,occupational exposure to silica,epoxy resins,organic solvents PVC .
Valuable opinion
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Excellent case@Dr. Saloni Arora . Never came across such case. Thanks for sharing with detailed case presentation :)
Thanks @Dr. Ashish Tiwari :)
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Very informative and detailed description of the case. Good going doc
Thanks alot @Dr. Ashish Bichpuriya Sir for your appreciation. Means alot. Regards
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NICE AND. INFORMATIVE UPDATE....
Thank you doctor
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Informative and helpful post
Thank you doctor
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