Scleredema

Airway exam - Check neck extension, mouth opening, and the prayer sign. Patients complain of limited shoulder and temporomandibular joint motion with difficulty chewing, and scleredema is the skin form of diabetic stiff joint syndrome, so a positive prayer sign points at both a long diabetic history and a hard intubation.

Plan for a difficult airway - Woody induration over the neck limits extension, and a stiff TMJ limits mouth opening. The 2022 ASA difficult airway guidelines say to consider awake intubation when you suspect a difficult intubation and any one of the following also applies: suspected difficult ventilation, increased aspiration risk, the patient may not tolerate a brief apnea, or emergency invasive airway rescue would itself be difficult. Diabetic gastroparesis puts the aspiration box in play, so awake technique deserves real consideration here. Awake options per the guideline are flexible bronchoscope, video laryngoscopy, direct laryngoscopy, combined techniques, and retrograde wire-aided intubation. In predicted difficult airways, video laryngoscopy gives improved laryngeal views, more successful and more first-attempt intubations, and fewer maneuvers than direct laryngoscopy.

Restricted chest wall - The induration spreads across the shoulders and upper trunk, so chest wall compliance falls and peak inspiratory pressures run higher under positive pressure. Skin thickness can be measured on ultrasound, which is also how disease activity and severity are tracked.

Diabetes comes with it - Type 3 is the diabetic form. Check the A1C and a fasting plasma glucose preoperatively, then monitor blood glucose every 30 to 60 minutes intraoperatively and correct as needed. Long-standing diabetes here means assuming ischemic heart disease until proven otherwise — these patients have a much higher risk of intraoperative MI, and the pain of an MI may be mild or absent.

Treat as a full stomach - Diabetic gastroparesis and delayed GI motility make aspiration a real risk. RSI, and premedicate with drugs that inhibit secretion and neutralize gastric acid.

Autonomic dysfunction - The corrected QT interval on the ECG tracks the severity of diabetic autonomic dysfunction. Expect blunted responses to atropine and beta blockers, delayed hemodynamic compensation, and a higher likelihood of needing a vasopressor under general anesthesia. Hydrate ahead of induction and have a pressor drawn up.

Dysphagia - Extra-cutaneous scleredema can involve the tongue and esophagus, and dysphagia is a recognized complication. Ask about swallowing before you plan an oral airway or blind esophageal instrumentation.

Cardiac and effusions - Extra-cutaneous disease can reach the heart, lungs, and pleurae, and pleural or pericardial effusions are described. Get an echocardiogram for unexplained dyspnea or a muffled precordium.

Positioning - Shoulder motion is restricted, and diabetic patients are much more susceptible to compression and stretch nerve injuries. Position the arms only within the range the patient can reach awake, and pad generously.

Regional anesthesia - Thickened, indurated skin over the neck, shoulders, and upper trunk makes surface landmarks unreliable, so use ultrasound. The induration spares the hands and feet, so distal blocks are unaffected.

Keep them warm - Diabetics with autonomic dysfunction compensate slowly for temperature swings. Forced air and warmed fluids from the start.

Immunocompromise - Diabetes itself is a cause of immunocompromise, with a higher risk of infection and delayed recovery. Keep sterility tight for lines and blocks.

Screen for paraprotein - Type 2 scleredema is associated with a lymphoproliferative disorder, and paraprotein screening is recommended in types 2 and 3. A monoclonal gammopathy changes what you look for on the CBC and the renal panel.

Scleredema (scleredema of Buschke) is a rare scleroderma-like connective tissue disorder in which fibroblasts of the reticular dermis lay down excess type 1 collagen and glycosaminoglycans, with mucin filling the spaces between thickened collagen bundles. The skin becomes woody and non-pitting, starting at the neck and spreading to the shoulders, upper trunk, and arms, and characteristically sparing the hands and feet.

Three types. Type 1 follows a febrile infection and is self-limited over 6 to 24 months. Type 2 is tied to paraproteinemia. Type 3 is the diabetic form, runs a chronic progressive course, and is the one you will meet most often in an adult. It matters perioperatively because the induration sits on exactly the real estate you need — neck extension, temporomandibular joint motion, and chest wall expansion — and because it travels with long-standing diabetes.