Case Report
Scleredema
of Buschke: A Rare Post-Streptococcal Complication
S Majumder*, SK Mandal**,
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
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. 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 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 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 self-limiting condition
from systemic sclerosis.
REFERENCES
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7th Edition.
2. Fleischmajer R. Scleredema. In: Freedberg IM,
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I, Goldstein B. Diabetes mellitus. Clin Dermatol 2006;24:237-46.
4. Dziadzio M, Anastassiades CP,
Hawkins PN, et al. From scleredema
to
5. Alp
H, Orbak Z, Aktas A. Scleredema adultorum due to
streptococcal infection. Pediatr Int 2003;45:101-3.
6. Cron RQ, Swetter SM. Scleredema revisited. A poststreptococcal complication. Clin Pediatr (Phila) 1994;33:606-10.