Scleredema of childhood

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Scleredema of childhood A report o] 5 cases

Five cases of scleredema in children are presented. Thirty-one additional pediatric cases are summarized, and a possible relationship to preceding streptococcal injection is emphasized. Fourteen o[ 36 cases reviewed showed evidence o[ a preceding streptococcal illness. In the 4 patients in whom biopsies were per/ormed, the pathologic findings were similar and consistent with the diagnosis o[ scleredema. The literature is briefly reviewed and salient clinical and pathologic [eatures are indicated which differentiate this sell-limited apparently benign disease [rom scleroderma and dermatomyositis.

William D. Bradford, M.D., Charles D. Cook, M.D.,* G o r d o n F. Vawter, M.D., and William Berenberg, M.D. BOSTON, MASS.

SCL~REDE~A has been described as a disease of u n k n o w n etiology "characterized by firm, non-pitting edema, usually affecting the face, neck, scalp, conjunctivae, and thorax, occasionally the arms and more rarely the legs, sparing the hands and feet. ''1 The purpose of the present report is to describe 5 cases of scleredema in children and to indicate that this rare disease should be readily differentiated from dermatomyositis and scleroderma, with which it is frequently confused. I n addition, these and previously reported cases indicate that at least a majority of the cases of scleredema m a y repreFrom the Departments o[ Medicine and Pathology, the Children's Hospital Medical Center, Boston, and the Departments o[ Pediatrics and Pathology, Harvard Medical School. eAddress, Department of Pediatrics, Yale University School o[ Medicine, 333 Cedar Street, Nero Haven, Conn. 06510,

sent another type of altered reactivity to streptococcal infection.

CASE R E P O R T S Case 1. This 2~-year-old white boy was well until 4 months before admission when he developed pharyngitis, cervical adenitis, bilateral otitis media, and a fever of 104 ~ F. His condition gradually improved, but 3 weeks before admission the skin over the face and neck became firm. Two days before admission the boy had a febrile convulsion. On admission, in August, 1932, he had an exudative tonsillitis, purulent rhinitis, and bilateral cervical adenitis. The skin over the face, neck, shoulders, and flexor surfaces of the arms had a hard leathery consistency. A small "whitish spot with a rim of violet color and a hyperpigmented border" was noted on the back of his neck. Past history and family history were noncontributory.

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Laboratory examination results were: hemoglobin, 10.8 Gm. per 100 ml.; WBC, 11,500, with 75 per cent polymorphonuclear cells, 23 per cent lymphocytes, and 2 per cent monocytes. Throat and blood cultures were negative. Urinalysis was normal. Tuberculin test, Wasserman test, and a lumbar puncture were negative. Evidence of bronchopneumonia in the right lung and a left maxillary sinusitis were found on roentgen examination. The child's temperature fluctuated between 103 and 104 ~ F. for 3 days and then, without specific therapy, became normal on the fourth. During the 2 weeks of hospitalization, the induration of the skin persisted and he exhibited malaise and anorexia, aIthough he remained afebrile. Four weeks later he had a recurrence of pharyngitis and, in addition, leg pains and definite but minimal induration of the skin of the legs. Eight months following the initial appearance of the scleredema he was asymptomatie and without abnormal findings on physical examination; 5 years later, physical examination was entirely normal. Case 2. This 6-year-old white boy was well until 8 weeks prior to admission when he had malaise, "soreness of the neck," and several episodes of vomiting. Seven weeks prior to admission he had induration of the skin over the head and neck and pain in the axillary region upon elevation of his arms. No discoloration was noted. During the next few weeks the induration extended until it involved the back, lower abdomen, and lateral aspect of both arms. This was associated with increased fatigability. On admission to the hospital in July, 1945, the temperature and blood pressure were normal. Physical examination confirmed the presence of the skin lesions and revealed marked limitation of neck, shoulder, and mandibular movement. The involved areas of skin were described as "hidebound." The liver edge was palpated 2 cm. below the right costal margin. Past history and family history were noncontributory. Laboratory examination results were: hemoglobin, 12 Gm. per 100 ml.; WBC, 12,400, with 66 per cent polymorphonuclear cells, 31 per cent lymphocytes, 2 per cent monocytes, and 1 per cent eosinophils. Nose and throat culture were negative; urinalysis, tuberculin, and Hinton tests were negative. The sedimentation rate was 29 mm. per hour. Roentgenograms of skull and chest were normal. Two electrocardiograms were

March 1966

within normal limits. A biopsy was obtained from the left forearm. On the thirteenth hospital day, the boy had an episode of tonsillitis and pharyngitis with a fever of 102 ~ F. Nose and throat cultures at that time were again negative. Physiotherapy was used in an attempt to increase the range of motion of his neck and shoulders. He was discharged on the thirty-first hospital day with little change in his physical findings. One month later he was improved but still had indurated skin and limited movement of the neck and shoulders. Six months after his initial admission, the boy could open his jaw more widely, and the involved skin was less firm. Twenty-one months after the onset, the skin over the scalp, neck, and back was still "tight and firm," although he was continuing to improve and was active. When examined 16 years later, at 22 years of age, he was found to be well and without any remaining signs of scleredema. Case 3. This 11!/2-year-old white boy was well until 14 days prior to admission when he developed coryza and malaise. Eight days before admission he was afebrile but complained of a sore area in the posterior portion of the neck; 2 days later the neck "felt stiff" and he was treated with one injection of penicillin. Two days prior to admission, indurated skin was present over the neck and shoulders and he had difficulty opening his mouth fully. On the day of admission he was lethargic, had pain on swallowing, and swelling of the face was present. On admission, in April, 1950, the boy had a normal temperature and blood pressure. He had brawny induration of his scalp, face (including eyelids), trunk, and arms decreasing to the level of his wrists. There was no discoloration. Mandibular movement was limited to approximately 50 per cent of normal and the tongue was swollen and firm. Movement of the neck was limited as was flexion of the back. The skin over the hands, legs, genitals, and buttocks was not involved. Muscle examination revealed mild generalized weakness. Past history and faraily history were noncontributory. Laboratory examination results were: hemoglobin, 13.4 Gin. per 100 ml.; WBC, 13,700 with 92 per cent polymorphonuclear cells, 4 per cent lymphocytes, 2 per cent monocytes, 1 per cent eosinophils, 1 per cent basophils. Urinalysis, tuberculin, and Hinton tests were negative. Nose

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and throat culture were negative for beta hemolytic streptococci. Corrected sedimentation rate, 23 ram. per hour; Antistreptolysin (ASL) titer was 1,585 units (normal 250 or less). Roentgen studies of the chest, barium swallow, and electrocardiogram were normal. A skin biopsy was obtained from the back of the neck. The patient remained afebrile. He was treated with physiotherapy and testosterone propionate linguets, 50 rag. per day. Five weeks later the induration had decreased and no contractures or muscle weakness was noted. His sedimentation rate had decreased to 5 ram. per hour and his ASL titer was 1,250 units. He gradually improved, and the testosterone dosage was decreased and finally discontinued after 9 weeks of therapy. The ASL titer decreased to 833 units 2 ~ months after the onset of the scleredema and to 317 units, 2 months later. Seven months after admission the boy was playing baseball, had a full range of motion of all joints, but still had slight induration in the region of the sternocleidomastoid. One year after the onset, physical examination was entirely within normal limits, and 10 years later he had remained well and was serving in the Air Force. Case 4. This 4-year-old white boy was well until 3 months before admission when he had what was clinically diagnosed as a streptococcal sore throat and was treated with penicillin for one week. Thereafter, he was lethargic and irritable but afebrile. One month before admission, his voice was less loud than usual, he had difficulty swallowing, the eyelids appeared "tight," and the skin around the neck was stiff. Three weeks before admission, he had pharyngitis and fever and was treated symptomatically. Two weeks before admission, the skin of his face and neck was definitely thickened and he was treated with penicillin and chloramphenicol. The temperature ranged between 100 and 101 ~ F. and he was given adrenocorticotropic hormone (20 rag. per day) and prednisone (15 rag. per day) with no apparent improvement. A paternal aunt had had rheumatoid arthritis; past and family history were otherwise noncontributory. At the time of admission in December, 1957, he was afebrile but appeared lethargic and irritable. Blood pressure was normal. A "waxy" induration of the skin and subcutaneous tissues was noted over the scalp, forehead, eyelids,

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cheeks (he was described as having masklike facies), neck, and trunk as well as upper arms. Extension of the elbows was slightly limited. No discoloration of the skin lesions was noted. Laboratory examination results were: hemoglobin, 13 Gin. per 100 ml.; WBC, 12,000 with 72 per cent polymorphonuclear cells, 27 per cent lymphocytes, and 1 per cent monocytes. Urinalysis and spinal fluid were normal; tuberculin test was negative. Nose and throat culture was negative for beta hemolytic streptococci. Corrected sedimentation rate was 24 ram. per hour and ASL titer, 317 units. Roentgen studies of the chest and barium swallow were normal. The electrocardiogram was normal. Frednisone was discontinued within 4 days of hospitalization. By the seventh hospital day, the findings were decreasing and the lesions disappeared entirely within 3 to 4 weeks. Five years after discharge the boy had remained well and free of skin lesions. Case 5. This 7~-year-old white girl was well until 3 weeks before admission when she developed pharyngitis. One and a half weeks later the skin over her face and neck felt thick. By the time of admission, the induration had spread to the trunk and extremities, sparing only the hands and feet. On admission in May, 1963, she was afebrile. The lesions were described as having a "woody feeling"; no pigmentation was present. The child had no contractures and no muscle tenderness or weakness. Past history and family history were noncontributory. Laboratory examination results were: hemoglobin, 13 Gin. per 100 ml.; WBC, 12,400 with 42 per cent polymorphonuclear cells, 40 per cent lymphocytes, 6 per cent monocytes, and 12 per cent eosinophils. Urinalysis was normal; tuberculin, Hinton, and lupus erythematosus cell tests were negative. Throat culture was positive for beta hemolytic streptococci; ASL titer was positive 1:1,250. EIectrophoretic pattern was normal. A biopsy was obtained from the right deltoid region. On the second hospital day a 10 day course of penicillin was started and prednisone, 40 rag. per day, was begun and continued at home for a total of 8 weeks. No immediate change was noted. During the succeeding 4 weeks, however, there was a gradual diminution in the lesions and 8 months later she had just barely detectable induration in the upper extremities without other physical abnormalities.

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PATHOLOGICAL

FINDINGS

The four biopsies were consistent in their salient features with minimal variation in degree of involvement. There was minimal edema of the epidermis. Swollen collagen bundles, usually evident in deeper corium, in fascia, in perimysium, and to a lesser degree in connective tissue trabeculae of subcutaneous fat were found in each case. Close examination showed that these swollen bundles were composed of discrete fibrils (sometimes swollen) thrown into relative prominence by a matrix material which was closely associated with the collagen bundles and sharply defined from surrounding elements except in the densest collagen of fascia. This material was homogenous or finely granular and took all stains which demonstrate collagen but always was less densely stained than collagen fibrils and had an affinity for metachromatic stains. There was an apparent decrease in anisotropism in involved areas. Evidence of vasculitis (apart from some swelling of nuclei and a mild increase in perivascular mononuclear elements), diffuse inflammation, activation of fibroblasts, sclerosis, atrophy of dermal appendages, or major changes in epidermis, fat, skeletal muscle, or elastic tissue were not evident. These findings distinguish scleredema from scleroderma and dermatomyositis (Table I), both

of which show more vasculitis and tissue destruction (fibrosis and sclerosis). The 5 cases show a striking similarity in their clinical and pathologic features. The onset followed weeks to months after an acute upper respiratory tract infection; in all cases there was a sweater distribution of involvement (Fig. 1), and the course was consistently benign. The 3 patients in whom the antistreptolysin titer was measured all showed evidence of having had streptococcal infections. In the 4 patients in whom biopsies were performed, the pathological findings were similar and consistent with the diagnosis of scleredema (Figs. 2, 3, and 4). DISCUSSION Scleredema was first described by Piffard, 2 in 1871, who distinguished it from scleroderma and called it "acute scleriasis." T h e term scleredema adultorum applied by Buschke, 3 in 1900, is a misnomer since the greatest incidence occurs in the first two decades of life (51 per cent of the cases are 20 years or under according to the report of Greenberg and co-workers~). A review of the literature reveals 31 cases of scleredema in children and adolescents in whom the diagnosis appears reasonable on the basis of the available description. Data from these and the present 5 cases are tabulated in Table II. These cases were selected

Table I. Comparison of pathologic findings in scleredema, scleroderma, and dermatomyositis

I

Scleredema~

I

Scleroderma

I

Derraatomyositis

Epidermis

Minimal edema only

Atrophy

Vacuolation Atrophy, hyperkeratosis (extensor surfaces)

Corium

Swelling of collagen bundles

Sclerosis Endarteritis Active marginal infiltrate

Fibrosis Active infiltrate

Vascular sclerosis or normal Occasionally fibrosis or

Increased muscle nuclei Perivascular infiltrate Increased interstitial tissue Degenerated muscle

with homogeneous rectachromatic matrix material Minimal perivascular mononuclear infiltrate Fascia a n d muscle

Deposition of homogeneous metachromatic material in fascial layers

hyalinization ~On

the basis of biopsies from 4 of the present cases.

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5 case

2of5

Fig. 1. Diagram of distribution of skin changes in the 5 children with scleredema.

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without regard to sex and included all individuals under 20 years of age. T h e onset of illness was generally insidious and the skin lesions were usually preceded by an u p p e r respiratory tract infection. All h a d nonpitting induration of skin and subcutaneous connective tissues, usually without discoloration, over the neck, face, and shoulders. Frequently the trunk, upper arm, and occasionally the legs were involved. H a n d s and feet were spared. T h e duration of illness was generally 6 to 24 months without progression or specific systemic involvement. Of the 36 pediatric patients, 19 were males and 15 females (2 reports failed to list sex). This is at variance with the data of Greenberg and co-workers, 4 who found a 2:1 ratio of females to males in the combined group of children and adults. I n 8 of 9 instances in which the A S L titer was measured, it was elevated. Of the 7 patients with throat cultures, 3 revealed beta hemolytic streptococci. Eight of 13 patients had an elevated sedimentation rate. Taken on an individual basis, eight children had elevated antistreptolysin titer and 2 of these children also had beta hemolytic streptococci in their throats. One other child

Fig. 2. Photomicrograph of biopsy from back of neck of Case 3 showing individual strands of collagen surrounded by homogeneous or faintly granular material which is discrete from adjacent fibers. (Toluidine blue and eosln, x510.)

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Table II. Thirty-six

Date

March 1966

pediatric

Author

Sex

cases of scleredema

Age at onset (yr.)

Preceding illness

Time ~ (wk.)

ASL

ESR

Duration of illness (months)

Therapy

1903

Baginsky 7

F

59/12 G e n e r a l m a l a i s e

1909

Nobl s

F

6

Parotitis

1923

Hoffmann 9

F

7

Grippe

1925

GrilnmandeP ~

?

4

Scarlet fevernephritis

1927

NaegelP 1

F

14

P y o d e r m a of arms

1928

Beck 12

?

7

Grippe

1928

yon Graevenitz~3

M

4

Pharyngitis I m p e t i g o 8 days after onset

2-3

Thyroid

1928

y o n Graevenitz 13

F

5

Otitis m e d i a

2-3

Thyroid

1929

R u m m e r t 14

F

13

Tonsillopharyngitis

1929

Lean~, DryfysLee, a n d gaunaa15

M

1930

SeiferO6

1934

4~2

Thyroid

Pituitary

Tonsillopharyngifts-nephritis

3

M

None

1

Guy and AmscheNr

F

Scarlatina fi-strepttonsilitisnephritis

4

1937

Vero a n d PingitorO s

M

11

Tonsillopharyngitis

4

1938

Sweitzer a n d L a y m o n 19

F

14

Pharyngitis Grippe-arthralgia-myalgia

4

~ 12

Thyroid

1938

Sweitzer a n d Laymonl9

M

16

Scarlet fever

4

)12

None

1939

Barer 2~

M

10

Febrile illness

2

1940

O ' L e a r y , Waisman, and I-Iarrison21

M

18

Influenzamastoiditis

6

9

1940

O ' L e a r y , Waisman, and H a r r i s o n 21

M

2

Scarlet fever Otitis m e d i a mastoiditis

5

8

1940

O ' L e a r y , Waisman, and H a r r i s o n 21

F

6

None

~-~

37

9

Thyroid

Symptomatic

1~

4

None

12

*Time in weeks between preceding illness and onset of scleredema. ~Patient had otitis media, sinusitis, bronchopneumonia on admission. :~ASL not available in 1934, 1945. Upper limits of normal in our laboratory is 250 units. In Greenberg and Robinow's laboratories upper li~it of normal is 166. w method. HThis pharyngitis was described as "strept throat" clinically.

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Sclerederna of childhood

39 7

Table II. Cont'd

Date

Author

Sex

Age at onset (yr.)

1940 O'Leary, Waisman, and Harrison 21

M

6

1946 Vallee 5

F

16

Preceding illness

Time ~ (wk.) ASL

ESR

2

Pneumonia with emphysema

4

N

1I~

5

1950 Morris 22

M

10

URI

M

13

None

1960 Knierim 2s

F

5

None

1961 Paseker and

M

9

None

1962 Colver, Emory, and Graham 25

M

5%2 fl-streptococcus-pharyngitis-adenitis

4

1963 Robinow 6

M

7

1~

Therapy

3

Rubeola

1960 Knierim 2a

Duration of illness (months)

36

9 I0

39

10

Hyperthermia

Prednisone None

3

E a n e t 24

1963 Robinow 6

F

13

Rubeola Tonsillopharyngitis Cervical adenitis Tonsillopharyngitis Cervical adenitis

1,200

250

46

3

42

Prednisone

Methyl prednisolone Diphtheria antitoxin

3

1

Hydrocortisone

1963 Greenberg and associates 4

F

4

URI

4

250

N

30

Symptomatic

1963 Greenberg and associates 4

M

9~2

Pharyngitisotitis media

6

333

N

24

Symptomatic

1963 Greenberg and associates 4

F

7

URI, cough, fever

2

500

77

3

Symptomatic

1963 Present series

M

2~z

Pharyngitist

16

:~

8

Physiotherapy

1963 Present series

M

6

Cervical adenitis

8

:~

29 w

18

Physiotherapy

1963 Present series

M

11

Physiotherapy Testosterone

1963 Present series

M

4

1963 Present series

F

78~2 fl-streptoeoecus-pharyngifts

116A2 URI Pharyngitis H

had beta streptococci by t h r o a t culture, 3 ha d scarlet fever, one h a d nephritis, and one h a d impetigo. Thus, of 36 cases reviewed, 14 h a d evidence of a p r e c e d i n g streptococcal illness. A similar relationship

2

1,585

23 w

12

317

24w

3~

1,250

I~

ACTH-Prednisone

4

Prednisone

between scleredema and streptococcal infection has been noted by Vallee, ~ R o b i n o w , ~ an d G r e e n b e r g and co-workers 4 in adults an d children. Since the course and prognosis of scler-

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Fig. 3. Photomicrograph of biopsy from back of neck of Case 4 showing swollen and wideiy spaced collagen bundles in deeper corium. (Hematoxylin and eosin, x37.)

Fig. 4. Photomicrograph of biopsy from shoulder of Case 5 showing deeply staining focally swollen collagen fibers at junction of mid- and deep corium. Note lack of vasculitis. (Toluidine blue and eosin, x50.)

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edema is benign, it is useful to differentiate this condition f r o m s c l e r o d e r m a a n d dermatomyositis. S c l e r o d e r m a does n o t c h a r a c teristically follow p r e c e d i n g infections, is usually a s y m m e t r i c a l , a n d is not usually confined to the face, neck, shoulders, and u p p e r arms. M o s t frequently, scleroderma in children is localized a n d consists of whitish atrophic plaques. D e r m a t o m y o s i t i s is a generalized disease w i t h an insidious onset a n d usually a p r o l o n g e d course. E r y t h e m a t o u s or h y p e r k e r a t o t i c a t r o p h i c lesions are typically p r e s e n t over the extensor surfaces of the joints. T h e r e is f r e q u e n t l y generalized i n d u r a t i o n of the skin, subcutaneous tissue, a n d muscle w i t h tenderness a n d weakness. Thus, t h e history, the physical e x a m i n a t i o n , and course (as well as the f r e q u e n t presence of an elevated A S L titer) t e n d to distinguish scleredema from s c l e r o d e r m a a n d d e r m a t o myositis. T h e r e a r e no controlled studies on the t r e a t m e n t of children with scleredema. M a n y of the e a r l y cases were t r e a t e d w i t h t h y r o i d p r e p a r a t i o n s , while recent authors r e p o r t cases t r e a t e d with antibiotics a n d steroids. It is possible t h a t steroids m a y i m p r o v e the m o r b i d i t y of the a c u t e phase of scleredema, but because of their toxicity a n d the benign course of the disease, t h e y do not seem indicated. S t r e p t o c o c c a l infection should be suspected a n d t r e a t e d w h e n found. Physiothera p y m a y increase the m o b i l i t y of the involved areas. SUMMARY

Five cases of scleredema in children are presented. T h i r t y - o n e a d d i t i o n a l a c c e p t a b l e p e d i a t r i c cases a r e s u m m a r i z e d a n d a possible relationship to p r e c e d i n g streptococcal infection is emphasized. T h e literature is briefly reviewed a n d the salient clinical a n d p a t h o l o g i c features which differentiate this self-limited a p p a r e n t l y benign disease from s c l e r o d e r m a a n d d e r m a t o m y o s i t i s a r e indicated. REFERENCES

I. Epstein, N. N.: Scleredema adultonlm, J. A. M. A. 99: 820, 1932.

Scleredema of childhood

399

2. Piffard, H. G.: Acute scleriasis; spontaneous recovery. Scleroderma treatment by galvanization, Gazz. med. 7: 51, 1871. 3. Buschke, A.: Vorstellung eines Falles yon Skler6dem, Arch. f. Dermat. u. Syph. 53: 383, 1900. 4. Greenberg, L. M., Geppert, C., Worthen, H. G., and Good, R. A.: Scleredema "adultorum" in children, Pediatrics 32: 1044, 1963. 5. Vallee, B. L.: Scleredema: A systemic disease, New England J. Med. 235: 207, 1946. 6. Robinow, M.: Scleredema adultorum--a children's disease, Am. J. Dis. Child. 105: 265, 1963. 7. Baginsky, A.: Berl. klin. Wchnschr. 40: 441, 1903. 8. Nobl, G.: l~ber das benigne SklerSdem Erwachsener, Wien. med. Wchnschr. 59: 2244, 1909. 9. Hoffmann, E.: t.)ber Skleroedema (Scleremia) adultorum nach Grippe mit Gewebsver;inderungen an den cutanen Nerven, Klin. Wchnschr. 2: 963, 1923. 10. Grtlnmandel: Scleredema adultorum, Zentralbl, f. Haut- u. Geschlechtskr. 17: 270, 1925. 11. Naegeli, T.: Skler6dem (Buschke), Zentralbl. f. Haut- u. Geschlechtskr. 23: 638, 1927. 12. Beck, J.: Scleroedema adultorum (Buschke), Zentralbl. f. Haut- u. Geschlechtskr. 27: 751, 1928. 13. von Graevenitz, N.: "Ober 3 F~ille von Sklergdema adultorum (Buschke), Monatsschr. f. Kinderh., Leipz. & Wien. 39: 257, 1928. 14. Rummert, O.: Ein Beitrag zur Kenntnis des Skleroedema adultorum, Dermat. Wchnschr. 89: 1563, 1929. 15. Lean6, E., Dryfys-Lee, G., and Launan, C.: (quoted from Epstein) Bull. Soc. p6diat, de Paris 27: 518, 1929. 16. Seifert, A.: Arch. f. Kinderh. 91: 220, 1930. 17. Guy, W. H., and Amschel, F.: Scleredema, Arch. Dermat. & Syph. 29: 777, 1934. 18. Vero, F., and Pingitore, N. E.: Scleredema adultorum, with involvement of the muscles in an 11 year old boy, Am. J. Dis. Child. 54: 322, 1937. 19. Sweitzer, S. E., and Laymon, C. W.: Scleredema adultorum, Arch. Dermat. & Syph. 37: 420, 1938. 20. Barer, M.: Case of scleredema adultorum, Proc. Roy. Soc. Med. 32: 1379, 1939. 21. O'Leary, P., Waisman, M., and Harrison, M. W.: Scleredema adultorum, Am. J. M. So. 199: 458, 1940. 22. Morris, G. E.: Scleredema adultorum, Arch. Dermat. & Syph. 61: 1069, 1950. 23. Knierim, H. G.: Scleredema adultorum: A report of 2 cases, Ohio M. J. 56: 193, 1960. 24. Pascker, F., and Eanet, L.: Scleredema "adultorum" in a child, Arch. Dermat. & Syph. 84: 853, 1961. 25. Colver, T., Emory, J. L., and Graham, J. T.: Scleredema with hematuria and a raised antistreptolysin titre: Recovery with cortisone._ Case report, Arch. Dis. Childhood 37: 103, 1962.