Minimal Scleroderma with Extensive Calcinosis Cutis

Refer to: Rabens SF, Bauer M: Minimal scleroderma with extensive calcinosis cutis. Calif Med 118:69-71, May 1973 failure with atrial flutter and a 2:1 block. A carbon dioxide study demonstrated a pericardial effusion. Physical examination revealed numerous subcutaneous and peri-articular nodules. X-ray exam- Minimal Scleroderma with ination showed dense soft tissue opacities consistent with calcification (Figures 1, 2 and 3). Extensive Calcinosis Cutis Tlhere was an abscess on the right elbow. Cardiac symptoms were relieved by cardioversion and STEVEN FISHER RABENS, MD administration of digoxin, quinidine and diuretics. MARJORIE BAUER, MD In the following year, several ulcerations ap- Los Angeles peared about areas of calcification on the finger- tips (Figure 4). Although the patient's hands felt DEPOSITION OF CUTANEOUS CALCIFICATION OC- cold, they were never painful. No sclerodactyly or curring during the course of the so-called collagen telangiectasia was present, and there was good disease was reported as early as 1878 by Weber' capillary filling in all digits. A sinus tract persisted and in 1911 by Thibierge and Weissenbach. near the right elbow. From it the patient could Scleroderma and dermatomyositis have been the easily express a chalky, white liquid containing two diseases most frequently linked with calcinosis calcium as determined by chemical analysis cutis. Association with lupus erythematosus has (Figure 5). been described as well, although much more Another abscess appeared on the left knee and rarely.34 Calcinosis cutis in collagen disease in- the patient was again admitted to hospital in creases in incidence with the duration of disease November 1971. Physical examination, at this and with the severity of cutaneous involvement. It time, revealed a few firm hypopigmented patches is unusual for extensive calcification to occur early with pigmented follicular macules in both inguinal in scleroderma when clinical evidence of collagen areas, on the forehead, and on the back of the damage is minimal. neck (Figure 6). There was some recession of the hairline. The skin on the forearms was taut, Report of a Case but could be lifted. The facial skin was smooth The patient, a 49-year-old black woman, first with little wrinkling. There was no restricted mo- noticed hard nodules on all extremities and on the tion of the face or mouth, and the facial skin was buttocks in 1968. Two years later, she was ad- easily pinched. All hand and finger movements mitted to hospital because of congestive heart were intact. Hemoglobin was 12.4 grams per 100 ml. Leu- From the Department of Medicine (Dermatology), University of Southern California, School of Medicine. kocytes numbered 11,400 per cu mm with a nor- Submitted July 6, 1972. mal differential. Platelets were normal. Urinalysis Reprint requests to: S. F. Rabens, MD, Los Angeles County/USC Medical Center, 1200 N. State St., Box 648, Los Angeles, Ca. 90033. showed no albuminuria, glycosuria, casts, or red Figure 2.- Figure I.-Soft Extensive tissue caicification in _ w 1 l | calcifications both upper about left knee. * ~~~extremities. CALIFORNIA MEDICINE 69 The Western Journal of Medicine lins, antinuclear antibody tests, and lupus erythematosus preparations were negative in three determinations. Serum complement was 960 units. Culture of the abscess on the knee revealed Kleb- siella aerobacter and Staphylococcus aureus coag- ulase positive. Upper gastrointestinal tract and barium swallow roentgen studies were normal. On chest and abdominal films, very slight cardiac enlargement and minimal calcification in the abdominal aorta were noted. An intravenous pyelo- gram showed no renal calcification or other abnormality. Studies of bones and of the hands showed much soft tissue calcification with no bone resorp- Figure 3.-Normal joint spaces and soft tissue calci- tion and normal joint spaces. fication. On histopathologic studies of skin from areas of calcification, thickening of the dermis was noted, blood cells. The blood sedimentation rate was with dermal deposits which stained black with a 15 mm per hour. Electrolytes and blood sugar von Kossa stain for calcium. The collagen bundles were normal. Albumin content was 3.4 grams per were large and closely packed, with a reduction 100 ml, with globulin 4.8 grams. Serum protein of the interfibrous spaces. The fat around eccrine electrophoresis was within normal limits aside sweat glands as well as the subcutaneous fat ap- from a slight elevation of alpha-2 globulin. Bili- peared to be replaced almost entirely by connec- rubin, protein-bound iodine, T-3 uptake, uric acid, tive tissue. Sebaceous glands and hair follicles cholesterol, aldolase, creatine phosphokinase, and were sparse. The epidermis appeared normal, and serum glutamic oxalic transaminase were all nor- there was no inflammatory infiltrate (Figure 7). mal. Serum calcium was 10.3 to 10.6 mg per 100 These findings were consistent with the histo- ml and serum phosphate was 3.7 to 4.0 mg per pathological changes in scleroderma recently de- 100 ml on three determinations. Twenty-four-hour scribed by Fleischmajer et al.5 urine calcium was 0.6 mEq, 1.6 mEq, and 5.9 mEq on three occasions (2.5 to 20 mEq normal); Discussion and 24-hour urine phosphate was 0.19 mEq (up Calcification seen in the collagen diseases is to 3.5 mEq normal). Alkaline phosphatase was termed dystrophic calcification and the deposits 2.5 units (1 to 3 units normal). The VDRL test are a local response to tissue injury. The serum was nonreactive. Rheumatoid factor, cryoglobu- calcium and phosphorus are normal, and no evi- Figure 4.-Finger and finger tip Figure 5.-Sinus tract, right elbow. Figure 6.- Inguinal area. Hypopig- calcifications. Note chalky, white material contain- mented firm patch with pigmented ing calcium expressed from this follicular macules. area, with many deposits. 70 MAY 1973 * 118 * 5 weakness and enzyme elevation. In scleroderma, extensive calcinosis cutis is a rare sequela. It Figure 7..,- occurs most frequently with severe acrosclerosis Forearm. Note and is usually a late complication-on an average, thick dermis with 11 years after onset of the disease. It is more fre- closely packed in and is said to offer a more g,f,,;X collagen bundles, quent women, sweat glands favorable prognosis because it is a sign of long- trapped by standing disease.'2-'7 collagen, dermal A calcifications, and The patient in the present case is of interest absence of hair because she has extensive calcinosis cutis without K>follicles, acrosclerosis or many other signs of scleroderma. ; sebaceous glands, and subcutaneous Calcinosis cutis was diagnosed before scleroderma, fat. (Hemotoxylin thus reversing the usual sequence. The only mani- and eosin, X 50) festation of internal disease in this case has been one episode of congestive heart failure, arrhythmia and. pericardial effusion. dence of a systemic abnormality of calcium or The lack of clinical and laboratory evidence of phosphorus metabolism, such as occurs in hyper- systemic lupus erythematosus and dermatomyo- parathyroidism, is seen. Calcinosis universalis is a sitis, the appearance of the few firm, hypopig- term sometimes reserved for calcium deposition in mented areas with pigmented follicular macules, the dermis, subcutaneous tissue, or muscles with- the taut skin of the forearm, the cardiac manifes- out any known predisposing local tissue damage tations, and several confirmatory biopsies suggest or systemic metabolic disorder.f'7 scleroderma as the most probable explanation for In all cases, when nodules or plaques are de- the extensive calcification. posited, the surrounding tissue may become ery- Sunimary thematous, infected, tender and painful, with ul- ceration of overyling skin and discharge of a Calcinosis cutis complicating scleroderma usu- chalky material containing predominantly calcium ally occurs as a secondary event following exten- phosphate with some calcium carbonate. sive disease of many months' duration. The case Dystrophic calcifications are usually confined reported is noteworthy because the extent of the to areas of local connective tissue or lipid damage. calcinosis cutis seems disproportionate to the min- There are several explanations for this event, some imal amount of demonstrable scleroderma. of which are based on animal experiments. De- REFERENCES 1. Weber H: Cutaneous calcification. Korresp Bi Schweizer struction and lysis of fat cells results in cellular Arzte 8:623, 1878 2. Thibierge G, Weissenbach AJ: Concretions calcaires souscu- debris which may combine with calcium ions to tanees et scierodermie. Ann Dermat et Syph 2:129-155, 1911 3. Kabir D, Malkinson F: Lupus erythematosus and calcinosis produce calcium soaps in affected areas. cutis. Arch Derm 100:17-22, 1969 4. Savin J: Systemic lupus erythematosus with ectopic calcifica- In addition, tissue injury releases alkaline phos- tion. Brit J Derm 84:191-192, 1971 5. Fleischmajer R, Damiano V, Nedwich A: Alteration of sub- phatase, which initiates the calcification process. cutaneous tissue in systemic scleroderma. Arch Derm 105: 59-66, 1972 The explanation for this phenomenon is based 6. Rook A, Wilkinson DS, Ebling FJG: Textbook of Derma- tology. Oxford and Edinburgh, Blackwill Scientific Publications, upon the interaction betwen alkaline phosphatase 1969, p 1637 7. Briggs, I: Calcinosis universalis. Lancet 2:800, 1952 and polyphosphates. Polyphosphates have been 8. Cornbleet T, Struck HC: Calcium metabolism in scleroderma. Arch Derm Syph 35:188-201, 1937 shown to inhibit the precipitation of calcium phos- 9. Fleisch N: Mechanisms of calcification, role of collagen, polyphosphates, and phosphatase. Am J Physiol 200:1296-1300, 1961 phate. The alkaline phosphatase released inacti- 10. Johnson WC, Forbes PD Graham JH, et al: Experimental cutaneous calcinosis-A histopathologic and histochemical study. vates the polyphosphate compounds, thereby al- JID 43:453-466, 1974 11. Moss U: Experimental cutaneous calcinosis. Arch Path lowing calcification to occur. 78:127-134, 1964 12. Muller S, Brunsting L, Winkelmann R: Calcinosis cutis- Calcification is thought to occur in areas of Its relationship to sclerodernMa.

Load more