ARTICLE
Acrokeratoelastoidosis
Auteur(s) : Zhifang ZHAI, Xichuan YANG, Fei HAO
Department of Dermatology,
Southwest Hospital, the Third Military
Medical University, Chongqing,
400038, P.R. China
<zhaoyj@mail.tmmu.com.cn>
In 2003 Yoshinaga et al. [1] described a 45-year-old
man with acrokeratoelastoidosis (AKE) associated with nodular
scleroderma, a variant of localized scleroderma and Tajima et
al. [2] in 2002 reported a variant of AKE in 7 out of
26 cases with systemic scleroderma, suggesting a high rate of
frequency in the association of AKE with systemic scleroderma.
Acrokeratoelastoidosis is a rare papular palmoplantar keratosis
characterized by small, round, firm papules occurring over the
dorsal hands, knuckles, and lateral margins of the palms and soles.
They are most often asymptomatic. The lesions appear in early
childhood and progress slowly. The disease appears to have no
racial, age or gender predilection. At present it is generally
acknowledged as a autosomal dominant genodermatosis and it has a
possible linkage to chromosome 2 [3], though AKE is more
frequently sporadic in the literature. We reported a sporadic case
of AKE, a 37-year-old Chinese woman in whom the borders of her
hands were involved (figure 1A). Yoshinaga et
al discussed that AKE was related to localized nodular
scleroderma as well as systemic scleroderma and coexistence of both
skin disorders suggested that nodular scleroderma and AKE were
etiologically related [1]. Masse et al. [4] revealed by
electron microscopy studies that in AKE the keratotic papules might
result from a disturbance in the secretion or excretion of elastic
material by fibroblasts in the dermis. This results in
elastorrhexis and an abnormal deposition or repair of connective
tissue within the involved area [5].
It is difficult to diagnose AKE only by the clinical features. In
the early stage, as it occurs as flavescent translucent papules,
CAKE should be distinguished from hereditary papulatranslucent
acrokeratoderma, pseudoxanthoma elasticum and colloid millium. When
AKE presents as keratotic papules, its clinical features are
particularly similar to some other keratodermas, such as focal
acral hyperkeratosis, degenerative collagenous plaques of the
hands, keratoelastoidosis marginalis of the hands, acrokeratosis
verruciformis of Hopf, and palmoplantar keratoderma of the punctate
type. The main differential diagnosis of AKE is presented in the
following table (table 1).
Table 1. The main differential
diagnosis of AKE
| Disease |
Etiology and inciting factors |
Onset |
Clinical features |
Locations |
Histology |
| AKE |
Autosomal dominant genodermatosis, but some are
sporadic. |
Adolescents or older |
Asymptomatic, firm, shiny, flat papules or
coalesce to plaques. |
Peripheral margins of the palms and soles |
Hyperkeratosis and mild acanthosis, significant
elastorrhexis in the dermis. |
| PTAK |
Autosomal dominant genodermatosis, history of
trauma or friction |
Adolescents or older |
Flavescent translucent papules |
Sides of hands and feet |
Hypokeratosis, acanthosis; almost normal
dermis. |
| Focal acral hyperkeratosis (FAH) [6] |
Maybe a disorder of keratinization but may be a
variant of AKE |
Children to adolescents |
Crateriform papules |
Sides of hands and feet |
Focal hypokeratosis, acanthosis; almost normal
dermis. |
| Degenerative collagenous plaques of the hands
(DCPH) [7] |
History of chronic sun exposure |
Middle age or older |
Crateriform papules in linear form or coalesce
to bands |
Sides of hands and/or fingers and feet |
Degenerative collagen and elastin fibers;
advanced actinic damage. |
| Acrokeratosis verruciformis of Hopf [8] |
Autosomal dominant genodermatosis |
Adolescents or older |
Flat verrucous papules |
Dorsa of hands and feet, knees and/or
elbows |
Hyperkeratosis, thickened granular layer,
acanthosis and papillomatosis. |
| Keratoelastoidosis marginalis of the hands
[5] |
History of chronic sun exposure and trauma or
friction to hands |
Senior people |
Hyperkeratotic papules |
Hands |
Hyperkeratosis, marked actinic damage or solar
elastosis. |
| Palmo plantar keratoderma of the punctate type
(PPK-Pt) [9] |
Autosomal dominant genodermatosis and may be a
variant of AKE |
Childhood or older |
Round to oval dome-shaped papules |
Palms and soles |
Hypokeratosis, parakeratosis; pyknotic vaculated
epidermis with basal layer spongiosis; dilated sweat ducts, blood
vessels and lymphatics. |
These disorders are distinguished solely on the basis of
alterations in elastic tissue. In our case, the biopsy specimen
showed hyperkeratosis, mild acanthosis and mild chronic
inflammation in the upper dermis (figure 1B). The special
staining for elastic fibers (Verhoeff’s Van Gieson stain) disclosed
reduced elastic fibres and significant elastorrhexis in the dermis
(figures 1C and 1D). Highet et
al. [6] pointed out that AKE showed a combination of
hyperkeratosis with fragmentation of dermal elastic tissue, which
justifies the designation AKE. If there is no abnormality of
elastic tissue, it appears to be a separate entity.
Multiple therapies for AKE, such as liquid nitrogen, salicylic
acid, tretinoin, and prednisone, have been tried, with minimal
success. It has been acknowledged that treatment should only be
attempted if there are symptoms. n
References
1. Yoshinaga E, Ohnishi Y, Tajima S.
Acrokeratoelastoidosis associated with nodular scleroderma. Eur
J Dermatol 2003; 13: 490-2.
2. Tajima S, Tanaka N, Ishibashi A, Suzuki K. A
variant of acrokeratoelastoidosis in systemic scleroderma: report
of 7 cases. J Am Acad Dermatol 2002; 46: 767-70.
3. Greiner J, Kruger J, Palden L, et al. A
linkage study of acrokeratoelastoidosis. Possible mapping to
chromosome 2. Hum Genet 1983; 63: 222-7.
4. Masse R, Quillard A, Hery B, et al.
Costa’s acrokerato-elastoidosis. Ultrastructural study (author’s
transl).(article in French). Ann Dermatol Venereol 1977;
104: 441-5.
5. Bogle MA, Hwang LY, Tschen JA.
Acrokeratoelastoidosis. J Am Acad Dermatol 2002; 47:
448-51.
6. Highet AS, Rook A, Anderson JR.
Acrokeratoelastoidosis. Br J Dermatol 1982; 106: 337-44.
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