Download Scleredema of Buschke: A Rare Post-Streptococcal

Case Report
Scleredema of Buschke: A Rare Post-Streptococcal
Complication
S Majumder*, SK Mandal**, S Roy Chowdhury*, D Bandyopadhyay**,
R Bandyopadhyay**, PP Chakraborty***
Abstract
A 19-year male presented with acute onset, gradually progressive symmetric indurations involving the skin over
the face, neck, shoulders and upper part of chest following an upper respiratory tract infection. Detailed history
and examination did not reveal evidence of Raynauds’ phenomenon, nail changes, digital ulcers, pigmentation
or any systemic involvement. Autoantibodies for systemic sclerosis were absent. Histopathology of skin biopsy
documented scleredema. Antistreptolysin O (ASO) titer was elevated. We diagnosed a case of Scleredema adultorum
of Buschke following a streptococcal throat infection. We report this case to highlight the importance of clinically
differentiating this relatively benign, self-limiting disorder from systemic sclerosis. ©
INTRODUCTION
S
treptococcal throat infection is common. We
report a case of scleredema adultorum of Buschke, a rare
complication of streptococcal infection. It closely resembles
systemic sclerosis.
CASE REPORT
A 19 year male presented in January 2006 with difficulty
in opening his mouth, turning his head and raising his
arms above the shoulders due to a gradually progressive
stiffening of the skin over the last six weeks. The skin
indurations were painless and began from the region of the
neck and gradually progressed to involve the face, upper
part of the trunk and the upper part of both arms. There
was no involvement of the distal parts of the upper limbs
or the lower extremities. There was no history suggestive
of Raynaud's phenomenon or swelling of hands or feet. He
specifically denied dysphagia, dyspnea, easy fatigability,
weight loss or joint pains. There was no history of prolonged
drug intake or toxin exposure. He was not a known
diabetic and had not undergone organ transplantation.
He gave a history of a febrile illness preceding the onset
of his symptoms by two weeks. The fever was associated
with a sore throat that lasted for about a week before it
subsided with drug therapy. Examination revealed the
presence of painless induration of the skin over his face
(Fig. 1), neck, upper trunk and the upper part of both
arms. No acral involvement (Fig. 2), digital ulcers, infarcts,
plaques or pigmentation or nail changes were observed.
*Post Graduate Trainee; **Assistant Professor; ***R.M.O. cum Clinical
Tutor, Department of Medicine, Medical College, Kolkat.
Received : 14.5.2007; Revised : 13.7.2007; Accepted : 29.8.2007
© JAPI • VOL. 55 • OCTOBER 2007
Anemia and lymphadenopathy were absent. Pulse was 84/
minute, regular and equally palpable in all four limbs. His
blood pressure was 124/76 mm Hg. Systemic examination
revealed no specific abnormality. Investigations revealed
hemoglobin 14.5gm/dl, total white cell count 6800/cmm
with normal differentials, platelet count 190 x 109/L and ESR
12mm. The fasting and postprandial plasma glucose levels
were 74mg/dl and 100 mg/dl. The serum urea, creatinine,
electrolytes and liver function tests were within normal
limits. Urinalalysis was normal. Total serum protein was 7.2
gm/dl of which globulin was 2.9gm/dl. Ultrasonography
of the abdomen and chest radiograph were within normal
limits. Assays for antinuclear antibody, anticentromere
antibody and anti Scl-70 were negative. Skin biopsy from
involved sites revealed normal epidermal lining with
short rete ridges. The mid and reticular dermis showed
relatively dense eosinophilic collagen as compared with
papillary dermis indicating dermal sclerosis. There was
some separation of collagen fibers in reticular dermis (Fig.
3). Special stains for amyloid were negative. Histology
was compatible with scleredema. Serum and urine
immunofixation electrophoresis were within normal limits.
The ASO titer was elevated (800IU/ml). Throat swab cultures
revealed the presence of streptococcus. He was diagnosed
to be a case of scleredema adultorum of Buschke following
streptococcal infection. The patient was put on three-weekly
intramuscular long acting penicillin therapy. He was on
regular follow-up and has completely recovered over a
period of four months and is leading a normal life.
DISCUSSION
Scleredema is a connective tissue disorder characterized
by widespread induration and thickening of the skin resulting
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737
Fig. 3 : Histopathology of skin showing dense eosinophilic collagen in
the dermis.
Fig. 1 : Skin induration over face.
dermatological manifestation of diabetes mellitus3 and
association with plasma cell dyscrasias has been reported.4
It has been reported occasionally following trauma and
tubercular lymphadenitis. Streptococcal hypersensitivity
injury of lymph channels, alteration of pituitary function
and peripheral nerve abnormalities have all been proposed
but none have been substantiated. An autoimmune
pathophysiology of the disease related to streptococcal
infection has also been proposed. There is no effective
treatment. Most cases are self resolving within a period of
six months to two years. Significant improvement has been
reported recently with the use of electron beam therapy.
The patient showed gradual marked improvement and has
now completely recovered from his skin lesion. Therein lies
the importance of distinguishing this relatively benign selflimiting condition from systemic sclerosis.
Fig. 2 : Relative sparing of distal extremities.
from the accumulation of collagen and proteoglycan in the
dermis.1 It is characterized by stiffening and hardening of
subcutaneous tissue as if infiltrated with paraffin. The onset
is frequently sudden and consists of marked nonpitting
symmetric induration of the skin usually affecting the
posterior and lateral aspects of the neck and spreading to
the face, shoulders, back, arms and thorax.2 The disease
usually reaches maximum involvement in one to two
weeks. The induration is of wooden-like consistency, waxy
white or shiny in appearance and rather diffuse so that
there is no sharp line of demarcation between involved
and non-involved regions. Involvement of the neck with
acral progression, sparing of hands and feet, absence of
Raynaud’s phenomenon and visceral involvement permit
clinical differentiation of this rare disease from systemic
sclerosis. A few days to six weeks before the onset, 65%
of patients develop an infection, usually of streptococcal
origin.2 Most laboratory tests are within normal limits.
Autoantibodies associated with both the systemic and
limited forms of scleroderma are absent. Histopathology
shows minimal epidermal changes with markedly thickened
dermis due to collagen deposits separated by spaces of
hyaluronic acid deposition. The cause of scleredema is
unknown Insidious onset. Scleredema is a well recognized
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© JAPI • VOL. 55 • OCTOBER 2007