Lipodystrophy and Calcinosis; Two Rare Manifestations in Adult Dermatomyositis
Main Author: Suad Hannawi
Dubai, United Arab Emirates
Al Kuwait Hospital, Dubai, Emirates Health Services (EHS)
Background (s): Dermatomyositis (DM) is an autoimmune connective tissue disease (CTD) characterized by special skin rashes and muscle inflammation. Calcinosis occurs as a complication of DM due to abnormal deposition of insoluble calcium salts in tissues, including skin, subcutaneous tissue, fascia, muscle, and tendons. Although calcinosis is more common in juvenile DM (JDM), it can occur in adult DM. Muscle calcification is generally asymptomatic, and may be seen only on radiological assessment. calcinosis often correlates with the level of long-term disability. It is usually a sign of “burned-out” myositis; it contributes to muscle atrophy and joint contractures. On the other hand, Lipodystrophy (Loss of subcutaneous fat ) is another rare manifestation characterized by loss of adipose tissue that can be seen in 10% of primarily JDM, and very rarely in adult-onset dermatomyositis (DM).
Method(s): We are presenting a case of adult-onset DM who presented with lipodystrophy with coexisting underlying muscle calcification.
Result(s): Mrs. M.A.T. is a 39-year-old Sudanese case of DM for 10 years and mild bronchial asthma for 8 years (not on regular treatment). Mrs. M.A.T presented to Al Kuwait-Dubai hospital (Al-Baraha) for the first time with fever, lower back pain, body rash, increase in hair loss, mouth dryness, and blurring of vision. But there was no muscle weakness. The symptoms were of a week duration. The medications she was on were esomeprazole 40 mg OD, Prednisolone 5 mg OD, Azathioprine 200 mg BID, Diltiazem hydrochloride 180 mg OD
On examination: vital signs were normal. Eyes were surrounded with heliotrope rash (Figure. 1), mechanic’s hands (Figure 2. And Figure 3.). There were regional localized areas in the back and over the frontal aspect of the right thigh with loss of subcutaneous fat, and dark discoloration of the skin (Figure 4, and Figure 5).
Laboratory work revealed: Negative Rheumatoid Factor (RF), Anti-dsDNA, Anti SS-A/RO, Anti SS-B/La, Anti-RNP, Anti-centromere antibody, Anti-JO1, Anti smith antibody (AntiSM), Anti-scleroderma 70, Anti -Histone, Anti-cardiolipin antibody. Complete blood count showed normal total and differential white cell and platelet counts, but slightly low hemoglobin at 11.1 g/dl (RR: 10.5-13.5), with mean cell volume of 75.9 fl (RR: 71.0-90.0), and mean cell hemoglobin of 25.5 pg (RR: 24.0-29.0). Associated with low serum iron at 46 ug/dl (RR: 60-160), low Serum Ferritin at 11.6 ng/ml (RR: 13.0-150.0), and normal Serum Transferrin at 264 mg/dl (RR: 192-382)
There were no significant changes in the level of the muscles enzymes: Myoglobin <21 ng/ml (RR: 25-72), Creatine Phosphokinase (CPK) 60 U/l (RR: 39-400), Creatine Kinase MB (CK-MB) 6.3 U/l (RR: 2.3-9.5), Troponin T hs STAT 0.004 ng/ml (RR: 0.013-0.025), and Lactate Dehydrogenase 509 U/l (RR: 280-500)
Inflammatory markers showed a high erythrocyte sedimentation rate (ESR) of 34 mm/hr. (RR: 1-20), and normal C-reactive protein (CRP).
Radiological examination showed a pelvis X-ray with small focal globs of amorphous calcification in the muscles (Figure. 6). Lumbar and knee X-rays with osteoarthritis changes.
Methotrexate 20 mg/week (wk.) had been started. As well as alendronate 70 mg/wk, CaHco3 500 mg TDS, Vit D (1 Alfa) 0.25 microgram OD (as DEXA showed osteopenia). Iron fumarate 200 mg BID, and artificial tears and saliva. In 6 months, prednisolone and omeprazole stopped, AZT reduced to 50mgOD.
Mrs. M.A.T responded well to medications, with no clinical symptoms, and normal muscle enzymes and inflammatory markers.
Conclusion(s): Both Calcinosis and lipodystrophy can be seen in the adult dermatomyositis.
