Thiemann disease

Thiemann
disease and familial digital arthropathy – brachydactyly: two sides of the same coin?. (a1, a2) patient (II). Multiple bony abnormalities present, particularly involving the heads of the middle phalanges characterized by irregularity, flattening, and radial angulation. Bilateral symmetric marked joint space loss of the 2nd-5th DIP and PIP joints, 1st IP joints, left 5th MCP and right 2nd MCP joints. Osteophyte formation in a similar distribution with a subchondral cyst in the left 3rd DIP. Shortening of the middle phalanges, worst at the second and fifth digit is seen. (b1, b2) patient (I). Short and broad middle phalanges bilaterally, worst at the 2nd and 5th fingers. Irregularity and sloping of the 2nd -5th middle phalangeal heads with resultant radial deviation. No secondary degenerative changes. (c) 19-year-old man reported by Jawad et al. with Thiemann disease. There is flattening and irregularity of the phalangeal epiphyses and broadening of the PIP and DIP joints. There are signs of secondary osteoarthritis including joint space loss and osteophyte formation. Mild shortening of 2nd-5th middle and distal phalanges. (d) 17-year-old boy reported by Nurdan Kotevoglu-Senerdem et al. with Thiemann disease. There is irregularity, fragmentation and flattening of the 2nd-4th PIP and DIP joints. (e) 25-year-old women reported by Seçkin et al. with Thiemann disease. Note irregularity and flattening of the epiphyses and flexion deformity of both fifth finger PIP joints. There is thickening at the base of all proximal phalanxes and all middle phalanxes were broad. There is narrowing at the third and fourth DIP and fifth PIP joints of both hands. (f) Thiemann disease case report of a 10-year-old female by Molloy et al. There are dense sclerotic distal phalangeal epiphyses (ivory epiphyses). There is broadening and irregularity of the 2-5th middle phalangeal bases with premature physeal fusion and relative shortening. Mild ulnar deviation of the right third PIP joint. Note: permissions were obtained from the copyright holder to reuse the images c,d,e and f
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Thiemann disease (also called familial osteoarthropathy of the fingers or osteonecrosis of the base of phalanx) is a non-inflammatory disorder of unknown etiology and refers to osteonecrosis of the epiphyses of the phalanges which leads to deformity of the fingers.

Epidemiology

Thiemann disease is a rare form of avascular necrosis which involves proximal interphalangeal (PIP) and distal interphalangeal (DIP) joints in the second decade of life. The base of the proximal phalanges of the index and middle fingers are usually affected.

Cases generally arise in children and young adults (<25 years old) .

Thiemann disease is usually familial (with an autosomal dominant inheritance pattern), usually with complete penetrance, and a balanced male:female case distribution . Sporadic cases have also been reported and are three times as common in males .

Clinical presentation

Clinical presentation and radiologic appearance of Thiemann disease correspond closely with those of Mauclaire disease (osteonecrosis of the metacarpal heads).

An initial painless swelling of the proximal interphalangeal joints, with eventual development of pain and deformity is the typical clinical presentation.

Occasionally the toes, primarily the big toes, and the first tarsometatarsal (TMT) joints are also affected, in which case it may be called osteochondritis ossis metacarpi et metatarsi .

Blood tests are normal - including erythrocyte sedimentation rate - with no elevation in the level of acute phase proteins, e.g. C-reactive protein.

Pathology

Histopathology demonstrates an aseptic osteonecrosis with unremarkable vasculature, and lacking inflammatory changes .

Radiographic features 

Irregularity of the epiphyses of the phalanges is seen. The bones appear broadened, sclerotic and fragmented. Joint space narrowing may be seen later.

Treatment and prognosis

Treatment is usually conservative, although in severe disease arthrodeses or arthroplasties may be needed.

History and etymology

The condition was first reported by a German physician H Thiemann in 1909 .

Siehe auch:
Assoziationen und Differentialdiagnosen zu Morbus Thiemann:
Aseptische
Knochennekrose
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