ARTICLE
Auteur(s) : E. YOSHINAGA, Y. OHNISHI, S. TAJIMA
Department of Dermatology, National Defense Medical College,
3‐2 Namiki, Tokorozawa, Saitama 359‐8513, Japan
Reprints: E. Yoshinaga Fax: (+81)‐42‐996‐5209 E‐mail:
srg1731gr.ndmc.ac.jp
Article accepted on 27\05\2003
Scleroderma can have systemic and localized types. Nodular
scleroderma or keloidal scleroderma is considered to be a rare
variant of localized scleroderma. Unlike a typical form of
localized scleroderma, nodular scleroderma is characterized by
keloid‐like sclerotic and well‐defined nodules and histological
hyalinization of coarse collagen fibers in the dermis [1‐5]. The
pathogenesis of nodular scleroderma may be related to unique
cytokine or extracellular matrix proteins [6, 7].
Acrokeratoelastoidosis (AKE) is a rare skin disorder originally
described by Costa [8‐10]. It consists of small, firm papules seen
on the dorsa of the fingers. Histologically AKE is characterized by
focal marked hyperkeratosis, epidermal hyperplasia and dermal
elastorrhexis. Familial cases of autosomal dominant inheritance
have been described, and a possible linkage to chromosome
2 has been suggested [11]. However, many sporadic reports of
similar cases with the acquired form seen in older individuals have
appeared in the literature [12, 13]. Etiology of AKE is unknown. It
is not certain whether the primary feature of the disease is a
dermal elastorrhexis or epidermal change [14, 15]. Here, we report
acrokeratoelastoidosis associated with nodular scleroderma.
Coexistence of both skin disorders in the patient suggests that
nodular scleroderma and acrokeratoelastoidosis are etiologically
related.
Patient
A 45‐year‐old Japanese man presented in December 2001 with
multiple nodules on the trunk which appeared three months
previously. Physical examination revealed discrete erythematous
nodules on the breast, abdomen and upper arms. The lesions were
slightly elevated and sclerotic (Fig. 1a). On the
fingers, small, firm papules coalescing to plaque were noted,
although he was not aware of the lesions (Fig. 1b). No clinical
signs indicative of systemic scleroderma such as sclerodactylia,
Raynaud‘s phenomenon, ulceration of fingertips were noted. There
was no keloid family history, and he had no past history of keloid.
There is no family antecedent of such lesions.
.
Laboratory data were as follows: peripheral blood cell count and
hemoglobin were within normal limits. A slightly abnormal liver
function was noted: asparate aminotransferase: 46 IU
L ‐1 [normal range; 8 30 IU
L‐1], alanine aminotransferase: 87 IU
L‐1 [normal range; 5 35 IU
L‐1], alkaline phosphatase: 345 IU L‐1
[normal range; 100 340 IU L‐1]. He
suffered from diabetes mellitus (fasting blood sugar: 281 mg
dL‐1 [normal range; 65 110 mg
dL‐1], hemoglobin A1C: 7.4% [normal range;
4.3 5.8%]). Serum urea nitrogen, creatinine, C‐reactive
protein, serum immunogloblin content and serum complement level
were normal. Rheumatoid factor was positive, but autoantibodies,
anti‐nuclear antibody, anti‐scl‐70 antibody, anti‐centromere
antibody, anti‐RNP antibody were all negative. Urinary glucose was
1 +, but urinary protein was negative. Electrocardiogram
revealed a sinus arrhythmia. Chest X rays showed no remarkable
abnormalities. Computed tomography of chest and abdomen revealed no
remarkable abnormal findings except fatty liver.
Histopathology
A keloid‐like nodule on the abdominal region was removed by
punch biopsy for histological examination. On hematoxylin‐eosin
staining, thickened dermis was extended to the subcutaneous fat
tissue and collagen fibers in the mid to lower dermis were markedly
homogenized Fig. 2a). In the deep
dermis, thick, homogenized and hyalinized collagen bundles formed a
whorl‐like pattern (Fig. 2b). Atrophy of
the appendages was noted. Small papules on the dorsal side of thumb
were also taken for histological examination. The epidermis showed
a mild focal hyperkeratosis and regular acanthosis, although a
concaved epidermis was not observed. No fibrotic change of collagen
fibers in the dermis was seen (Fig. 3a). Elastic
fibers in the dermis were decreased in number in the upper and
middle dermis (Fig. 3b) and
fragmented, particularly in the lower dermis (Fig. 3c).
.
.
Discussion
The hyalinized collagen bundles typical of localized scleroderma
(morphea) were seen histologically in this case. However, the
clinical features of the lesions on the abdomen and breast were
hard, well‐demarcated nodules which indicated nodular scleroderma
rather than morphea.
Acrokeratoelastoidosis has three related entities, focal acral
hyperkeratosis, degenerative collagenous plaques of the hands and
keratoelastoidosis marginalis of the hands [16‐19]. The differences
among the four skin conditions are not well‐defined at present. We
diagnosed the lesion on the fingers as acrokeratoelastoidosis based
on the histological features with epidermal changes of
hyperkeratosis and acanthosis, and dermal elastorrhexis.
There is one paper describing the association of
acrokeratoelastoidosis with systemic scleroderma at a high rate of
frequency (7 cases in 26 systemic scleroderma) [20]. On
the basis of clinical and laboratory data our case showed no sign
of systemic scleroderma, but showed skin‐limited nodular
scleroderma suggesting that acrokeratoelastoidosis may be related
to systemic scleroderma as well as localized scleroderma.
Coexistence of nodular scleroderma and acrokeratoelastoidosis in
this patient, therefore, suggests that both skin disorders are
etiologically related.
The patient had a mild degree of diabetes mellitus. A close
relationship between diabetes mellitus and scleredema, which shows
sclerotic skin change in the deep dermis, has been described [21].
The relationship between diabetes mellitus and these fibrotic skin
disorders (acrokeratoelastoidosis and scleroderma) is uncertain.
There have been no previous reports concerning the correlation
between diabetes mellitus and nodular scleroderma, nor between
diabetes mellitus and acrokeratoelastoidosis. Long‐term diabetes
mellitus is considered to lead to connective tissue abnormalities
such as Dupuytren‘s contracture, carpal tunnel syndrome or stiff
hand syndrome [22]. These complications are thought to result from
non‐enzymatic advanced glycation endproducts (AGE)‐modified
proteins [23]. Immunohistochemical studies of the lesions of
nodular scleroderma and acrokeratoelastoidosis with anti‐AGE
antibody failed to show a positive staining (not shown), suggesting
no direct correlation between these lesions and diabetes
mellitus.
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