A 55 year old obese black male with a history of Diabetes mellitus type 2 of more than 15 years, hyperlipidemia, hypertension, and recurrent CVA with residual right hemiparesis admitted for rehab. He complained of chest tightness which he first noted six months prior to current CVA and admission. He reported progressive skin tightness around the shoulders, neck, chest and upper back that affecting his mobility. He is non compliant with diabetic management, BMI was 43 and Hemoglobin A1C was 10.4 on oral hypoglemic agent. He denied any history of pharyngitis, skin tightness on fingers, feet or face, no joint pain. No dysphasia or odynophagia nor constipation. No chest pain, palpitation or dyspnea.
Examination revealed right hemiparesis non pitting, thickened, indurated, hard skin over the posterior neck, upper back and shoulder, chest and over the legs bilaterally with limited range of motion.
ANA, ANCA, Rheumatoid factor,antiitopoisomerase(anti‐scl‐70),anticentromeree(ACA),anti‐RNA polymerases II and III were negative . Serum and urine protein electrophoresis was normal. Cardiac enzymes and EKG were unremarkable.
MRI of the neck showed thickened posterior neck skin with mild degenerative joint disease of the cervical spine. Skin thickness was also noticeably increased on both the upper posterior thorax and anterior chest wall‐about 13mm on CT scan of the torso. No significant peripheral or systemic lymphadenopathy on multiple radiographs.
An upper back skin biopsy was positive for normal epidermis, thickened dermis with widened spaces with mucin deposition in between collagen bundles. There was minimal inflammatory infiltrates with no increased fibrobla
Scleredema,a very rare dermatopathy first by Abraham Buschke.it is characterized by non‐pitting induration of the skin. Any skin can be involved but it typically affects the neck, back, chest and extremities. Rarely, viscera can also be affected.
Etiology is unknown, it has been associated with chronic diabetes, antecedent febrile illness including streptococcal pharyngitis, febrile viral illness, paraproteinemias, monoclonal gammopathy and hematologic malignancies.
Clinical presentation depends on body site affected. It can results in restricted mobility; difficulty with eye/mouth opening, dysarthria and restrictive lung disease.
Clinical course ranges from complete spontaneous resolution particularly when precipitated by febrile illness to indolence or relentless progression especially when associated with diabetes mellitus. Scleredema differs from scleroderma in that esophagus, hands and feet are typically spared. Also there is no autoimmune involvement.
There is no definitive treatment for scleredema. Available treatment data is based on case reports. Trials of Immunosuppressive, steroid, electron beam radiation. PUVA phototherapy have been reported with variable success.
Patient may also benefit from treatment targeting of associated disease.
Physical therapy is essential since earlier initiation may limit long term range of motion restriction.
Awareness of this disease is very important as its treatment, prognosis differs significantly with scleroderma, a more common but similar medical condition.