ARTICLE
Auteur(s) : Koji SUMI Toshiyuki YAMAMOTO Hiroo
YOKOZEKI Kiyoshi NISHIOKA
Department of Environmental Immunodermatology, Tokyo Medical and
Dental University Postgraduate School, Yushima 1‐5‐45, Bunkyo‐ku,
Tokyo, Japan
Reprints: K. Sumi Fax: (+81) 3‐5803‐0143 E‐mail:
kojidermtmd.ac.jp
Article accepted on 10\7\2003
Key words: Generalized morphea‐like scleroderma was
first reported by Yamakage and Ishikawa [1, 2]. The clinical
features of generalized morphea‐like scleroderma are different from
those of systemic sclerosis. Generalized morphea‐like scleroderma
clinically involves the upper arms, lateral forearms, lumbar area,
and back, which shows symmetrical and lesional sclerosis with
pigmentation and depigmentation intermingled within the sclerotic
plaques as well as redness on the borders. Frequently, there is a
history of exposure to organic solvents [2]. Our patient presented
not only the clinical features of generalized morphea‐like
scleroderma but also lesional miliary papules on the lateral
aspects of sclerotic forearms. Skin biopsies from the forearm
showed amyloid deposition in the papillary dermis, which was
identified by Congo‐red stain.
Case report
A 62‐year‐old Japanese man was referred to our hospital
complaining of dyspnea and coldness of his limbs. He had no
particular past history nor family history. His job was sale of
building materials and organic solvents at his office. Three years
ago, he noticed itching of the head and face, sclerosis of the
back, lumbar area and superior limbs, and Raynaud‘s phenomenon.
Furthermore, he had been conscious of dyspnea for one year.
Physical examination showed comparatively‐circumscribed shiny
plaques on the cervix, lumbar area, back and lateral upper arms. In
the V‐neck zone, redness and small depigmented patches were
diffusely recognized (Fig.1). Diffuse sclerosis and
pigmentation could be observed on his head and face. In addition,
miliary papules were found at the lateral aspects of sclerotic
forearms (Fig.2).
.
.
Laboratory studies showed that the anti‐nuclear antibody was
positive (1:320, speckled pattern) and serum KL‐6 levels were
3540 U\ml (normal; < 500 U\ml). The
anti‐ribonucleoprotein (RNP) antibody, the anti‐Scleroderma‐70
(Scl‐70) antibody, the anti‐SS‐A antibody, and the anti‐SS‐B
antibody were negative. The rheumatoid factor was 245 IU\ml
(normal: < 20 IU\ml). Serum immunogloblin E
levels were 2745 IU\ml (normal: < 250 IU\ml). The
arterial oxygen tension (PaO2) was 75.3 torr and the arterial
carbon dioxide tension (PaCO2) was 36.2 torr. Therefore, the
ventilation\perfusion mismatch of O2‐CO2 exchange was disordered at
alveoli. Data of the %VC (75.3%) and the FEV1.0 (77.2%)
demonstrated the restrictive lung disorder. The computed tomography
(CT) showed a honeycomb pattern at bilateral lower lungs near the
pleurae. Gallium scintigraphy and the esophagography showed no
abnormal findings.
Skin biopsies were taken from the left middle finger and the left
lateral forearm, both of which showed sclerosis with thickened and
homogenous collagen bundles in the thickened dermis. Histological
features of the specimen from the lateral forearm showed that the
epidermis showed irregular hyperplasia, orthohyperkeratosis, and
scattered dyskeratotic cells. In the papillary dermis amorphous,
eosinophilic deposits were presented (Fig. 3a). Congo‐red stain
showed positive findings at papillary dermis (Fig. 3b)..
Discussion
Most interesting features in the presented case were the
coexistence of miliary papules at the lateral aspects of sclerotic
forearms in a patient with generalized morphea‐like scleroderma. In
the papillary layer, positive findings were demonstrated by
Congo‐red dye. He had no features of cutaneous amyloidosis other
than on the forearms. Systemic amyloidosis was excluded by detailed
investigation. Cases of localized cutaneous amyloidosis associated
with systemic sclerosis [3] and sclerodermatomyositis [4] have been
reported. Ogiyama et al. [3] reported 6 cases of
cutaneous amyloidosis associated with progressive systemic
sclerosis, and the involved sites were the upper back in all cases.
To our knowledge, this case is the first report of cutaneous
amyloidosis associated with generalized morphea‐like scleroderma.
Although the onset of amyloidosis is unclear, amyloid deposition in
localized cutaneous amyloidosis was suggested to be derived from
degenerated epidermal keratinocytes through filamentous
degeneration or apoptosis [5, 6]. Humphreys et al. [8]
reported that amyloid deposition was made by frequent scratching in
atopic dermatitis. Amyloid deposition may be caused by a similar
mechanism in our case, because our patient complained of itching on
the lateral forearms, face and trunk. We speculate that amyloid
deposits may be induced by recurrent stimuli of the friction damage
through scratching in this case.
References
1 . Yamakage A, Ishikawa H. Generalized morphea‐like
scleroderma occurring in people exposed to organic solvents.
Dermatologica 1982; 165: 186‐93.
2 . Yamakage A, Ishikawa H, Saito Y, Hattori A.
Occupational scleroderma‐like disorder occurring in men engaged in
the polymerization of epoxy resins. Dermatologica 1980; 161:
33‐44.
3 . Ogiyama Y, Hayashi Y, Kou C, Matsumoto Y, Ohashi M.
Cutaneous amyloidosis in patients with progressive systemic
sclerosis. Cutis 1996; 57: 28‐32.
4 . Orihara T, Yanase S, Furuya T. A case of
sclerodermatomyositis with cutaneous amyloidosis. Br J
Dermatol 1985; 112: 213‐9.
5 . Kumakiri M, Hashimoto K. Histogenesis of primary
localized cutaneous amyloidosis: sequential change of epidermal
keratinocytes to amyloid via filamentous degeneration. J Invest
Dermatol 1979; 73: 150‐62.
6 . Maeda H, Ohta S, Saito Y, Nameki H, Ishikawa H.
Epidermal origin of the amyloid in localized cutaneous amyloidosis.
Br J Dermatol 1982; 106: 345‐51.
7 . Kobayashi H, Hashimoto K. Amyloidogenesis in
organ‐limited cutaneous amyloidosis. An antigenic identity between
epidermal keratin and skin amyloid. J Invest Dermatol 1983;
80: 66‐72.
8 . Humphreys F, Spencer J, Mclaren K, Tidman MJ. A
histological and ultrastructual study of the dirty neck‘ appearance
in atopic eczema. Clin Exp Dermatol 1996; 21: 17‐9.
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