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Indian Journal of Dermatology, Venereology and Leprology
Medknow Publications on behalf of The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL)
ISSN: 0378-6323 EISSN: 0973-3922
Vol. 71, Num. 2, 2005, pp. 137-138
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Indian Journal of Dermatology, Venereology, Leprology, Vol. 71, No. 2, March-April, 2005, pp. 137-138
Quiz
Bilateral, asymptomatic scaly and fissured cutaneous lesions of the fingers in a patient presenting with myositis
Bugatti Leonardo, Angelis Rossella De, Filosa
Giorgio, Salaffi Fausto
Dermatology Unit, "A Murri" Hospital, Jesi (Ancona)
Correspondence Address: Dermatology Unit, "A. Murri" Hospital, Via
dei Colli, 52, 60035 Jesi-AN-
dermjesi@yahoo.it
Code Number: dv05047
A 69-year-old woman was admitted because of sudden onset of fever and symmetrical arthro-myalgias, predominantly in the upper limbs. She also had global muscle weakness and wasting, as well as dyspnea and loss of appetite. On clinical examination, active mobilization of the joints was impossible, except for the elbows, hands and wrists. On thoracic auscultation slight bilateral crackles were revealed over the lower chest. Unusual cutaneous findings were noted, consisting of scaling and fissuring on the lateral sides of the second and third finger of both hands, a picture similar to that produced by manual labor [Figure - 1]. The dermatological examination was otherwise unremarkable. A skin biopsy specimen was obtained from an involved finger and stained with Hematoxylin-Eosin [Figure - 2]. Histological examination showed an interface psoriasiform dermatitis consisting of parakeratotic hyperkeratosis, epidermal hyperplasia, necrotic keratinocytes with focal exocytosis and vacuolar degeneration of the basal layer, perivascular lymphocytic infiltrate and telangiectasias. ESR was 18 mm in the first hour. Muscle enzymes were raised: creatine kinase 3712 U/l (normal range 15-210), aspartate transaminase 108 U/l (normal range 5-44), alanine aminotransferase 114 U/l (normal range 5-48), lactate dehydrogenase 1848 U/l (normal range 230-460), aldolase 40 U/l (normal range 2-7.6).
WHAT IS YOUR DIAGNOSIS?
Diagnosis: Anti-synthetase syndrome (ASS) with mechanic′s
hand
Immunological tests revealed the presence of anti-extractable nuclear
antigen (ENA) antibodies with anti Jo-1 specificity. Electromyography
showed small amplitude, brief actions potentials and increased insertional
activity was consistent with irritable myopathy. A muscle biopsy was
not performed. Lung disease was present with interstitial lesions on
CT scans and a pulmonary function test showing an obstructive disease.
The following tests were normal or negative: urinalysis, serum creatinine,
rheumatoid factor, antinuclear antibodies, anti-dsDNA, ANCA and anticardiolipin
antibodies, cryoglobulins, nailfold capillary microscopy and echocardiography.
Search for an occult neoplasm was negative.
A high dose of corticosteroids, methylprednisolone 2.5 mg/kg/day, was
required and cyclophosphamide 2 mg/kg/day was added for the presence
of an interstitial lung disease. Myalgias and muscle weakness improved
significantly as the cutaneous lesions gradually disappeared.
DISCUSSION
Antibodies binding to aminoacyl-tRNA synthetases have been identified
in the serum of 25% to 40% of adults with inflammatory
myopathies.[1] The first to
be discovered and the commonest of these antibodies, anti-Jo-1, (in 15% to
30% of
adults with polymyositis or dermatomyositis) is directed against histidyl
t-RNA synthetase.[2] At least
four other anti-synthetase antibodies have been described.[1] The
anti-synthetase antibodies are specific for a clinically distinctive
syndrome characterized by myositis along with non-erosive arthritis,
interstitial lung disease, skin changes (Mechanic′s hand, Raynaud′s
phenomenon) and fever.[3] Isolated
clinical manifestations such as interstitial lung disease have been reported
in about 10% of cases.[4] Compared
to classic dermatomyositis (DM), in which such reported similar lesions
were sporadically described in association with other skin manifestations,
mechanic′s hand seems to be a distinctive feature of the ASS,[5] present
in up to 70% of patients,[6] though
sporadically reported in classic DM in association with other cutaneous
findings.[7]
Light microscopy findings[7] are
similar to those observed in DM. In the present case, the distinguishing
cutaneous feature is the presence of mechanic′s hand in the absence
of any other skin lesion generally observed in typical DM (heliotrope
rash, malar rash and Gottron′s papules). The differential diagnosis
includes irritant contact dermatitis, tinea manuum and dry dishydrotic
eczema. Since myositis and cutaneous manifestations may not follow a
parallel course, the finding of mechanic′s
hand should prompt a search for anti-synthetase antibodies and may serve
as a clinical diagnostic aid in patients with an underlying idiopathic
inflammatory myopathy.
REFERENCES
1. | von Muhlen C, Tan EM. Autoantibodies in the diagnosis of systemic rheumatic diseases. Semin Arthritis Rheum 1995;24:323-58. Back to cited text no. 1 |
2. | Bernstein RM, Morgan SH, Chapman J, Bunn CC, Mathews MB, Tumer WM, et al. Anti-Jo-1 antibody: A marker for myositis with interstitial lung disease. BMJ 1984;289:151-2. Back to cited text no. 2 |
3. | Targoff IN. Immune manifestations of inflammatory muscle disease. Rheum Dis Clin North Am 1994;20:857-80. Back to cited text no. 3 [PUBMED] |
4. | Friedman AW, Targoff IN, Arnett FC. Interstitial lung disease with autoantibodies against aminoacyl-tRNA synthetases in the absence of clinically apparent myositis. Semin Arthritis Rheum 1996;26:459-67. Back to cited text no. 4 [PUBMED] |
5. | Mitra D, Lovell CL, Macleod TI, Tan RS, Maddison PJ. Clinical and histological features of 'mechanic's hands' in a patient with antibodies to Jo-1. A case report. Clin Exp Dermatol 1994;19:146-8. Back to cited text no. 5 [PUBMED] |
6. | Love LA, Leff RL, Fraser DD, Targoff IN, Dalakas M, Plotz PH, et al. A new approach to the classification of idiopathic inflammatory myopathy: Myositis specific autoantibodies define useful homogeneous patient groups. Medicine 1991;70:360-74. Back to cited text no. 6 [PUBMED] |
7. | Mittal R, Sharma VK, Prasad HR, Singh MK. Mechanic's hand: A clinical diagnostic aid in dermatomyositis. Acta Derm Venereol 2001;81:65-6. Back to cited text no. 7 [PUBMED] |
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