ARTICLE
Auteur(s) :, Manuel Ginarte*,
Álvaro León, Jaime Toribio
Department of Dermatology, Complejo Hospitalario Universitario
de Santiago, Faculty of Medicine Santiago de Compostela, Spain
accepté le 28 Juin 2004
Porokeratosis is a disorder of keratinization of unknown etiology
that is characterized by the presence of the cornoid lamella, a
column of parakeratotic cells extending through the stratum
corneum. Disseminated superficial porokeratosis (DSP) is a distinct
form of porokeratosis that consists of numerous, small, superficial
keratotic papules with an atrophic center bordered by a peripheral
keratotic ridge involving the extremities and trunk. Occasionally,
porokeratosis has been associated with dermal amyloid deposits,
especially in medium to advanced aged males of oriental origin with
DSP. Here we report a 65-year-old Caucasian man with DSP with
secondary cutaneous amyloidosis.
Case report
A 65-year-old otherwise healthy Caucasian man had multiple,
hyperpigmented, asymptomatic papules scattered over his trunk,
thighs, legs and arms that had been present for 5 years on both
exposed and non-exposed areas. The lesions measured less than
15 mm and had raised hyperkeratotic borders with central
atrophy ( (figure
1) ). The palms, soles, mucous membranes, and nails were
spared. No flare-up occurred due to sun exposure, and no topical
treatment had been previously applied. He did not know whether
other relatives showed similar skin lesions other than his sister,
who had never visited a dermatologist before she died. Blood cell
count, serum biochemistry, renal and hepatic function tests,
urinalysis, TSH, antinuclear antibodies, and serum protein
electrophoresis were all normal or negative. Histologic examination
revealed orthokeratosis in the epidermis with typical cornoid
lamella, the underlying granular layer was lacking, as well as some
dyskeratotic cells. Hydropic degeneration of the basal cell layer
and lymphohistiocytic inflammatory infiltrate in the papillar
dermis were also observed underlying the cornoid lamella zone (
(figure 2) ). An
eosinophilic amorphous material in the upper dermis was more marked
close to the cornoid lamella, stained positively with crystal
violet, and revealed bright yellow fluorescence with thioflavin-T
under polarizing microscopy ( (figure 3) ).
Histopathologic and histochemical studies from the healthy skin
surrounding the lesions were negative for amyloid deposits. Based
on the clinicopathologic correlation, a diagnosis was made of
disseminated superficial porokeratosis with secondary localized
cutaneous amyloidosis.
Discussion
Since Pamphongsant and Sittapairoachana [1] reported the first case
of porokeratosis associated to amyloidosis in 1974, at least 18
more patients have been notified (table 1)( Table 1 )[1-13]. Most of them are medium to
advanced aged males with DSP. Since patients from oriental origins
predominate, a sizable proportion of the reported cases come from
Asiatic countries. Whenever immunohistochemical investigations with
anticytokeratin antibodies have been carried out, K-amyloid
(derived from epidermal keratinocytes) was identified [8-13]. The
two cases reported by Kuno et al. [10] did not show positive
immunohistochemical reactions to immunoglobulin light chains or
amyloid AA protein in the dermal deposits. When the normal skin
adjacent to the lesions was studied, amyloid deposits were not
found, as occurred in our patient [5, 11-13].
Several treatments have been attempted: topical corticosteroids
[6, 11], topical retinoids [12], cryotherapy [9, 11, 12],
5-fluorouracil [13], and topical dimethyl sulfoxide [7]. Usually,
the response to therapy is poor, although occasional success has
been reported with dimethyl sulfoxide [7], topical corticosteroids
[6], cryotherapy [9], and combination of topical corticosteroids
with cryotherapy [11].
The cause of the association between the two conditions and why
only a small proportion of patients with porokeratosis develop
deposits of amyloid is unknown. However, the number of cases
reported argues against a coincidence. For the vast majority of the
authors, the amyloid deposits are due to the marked tendency of
keratinocytes in porokeratosis to degenerate. Degenerated and
necrotic epidermal cells (colloid bodies) can produce abnormal
keratin proteins that become transformed into amyloid [3, 14]. This
would explain why the amyloid deposits are confined to the vicinity
of the cornoid lamella and under the areas with more evident
porokeratotic changes. Racial or sexual factors could be implicated
and this would explain the tendency for aged oriental men to be
affected. Actually, this association may be more common than so far
reported, because of the difficulty of detecting the deposits of
amyloid with haematoxylin and eosin stain, particularly if they are
very scarce. In fact, some authors [3, 8] found amyloid deposits
only by means of histochemical, immunohistochemical and
ultrastructural techniques, but not by merely carrying out
haematoxylin and eosin stains.
Table 1 Patients number 13 and 14 were brother and
sister
|
Patient n°
|
Age
|
Sex
|
Type of porokeratosis
|
Race
|
Reference
|
Country of report
|
|
1
|
51
|
F
|
Disseminated superficial actinic
|
Thai
|
Piamphogsant (1974) [1]
|
Thailand
|
|
2
|
70
|
M
|
Mibelli
|
ND
|
Runne (1977) [2]
|
Germany
|
|
3
|
66
|
F
|
Mibelli
|
ND
|
Sato (1980) [3]
|
Japan
|
|
4
|
57
|
ND
|
Mibelli
|
ND
|
Sato (1980) [3]
|
Japan
|
|
5
|
55
|
ND
|
Mibelli
|
ND
|
Sato (1980) [3]
|
Japan
|
|
6
|
ND
|
ND
|
Giant porokeratosis
|
ND
|
Masu (1981) [4]
|
Japan
|
|
7
|
81
|
M
|
Disseminated superficial
|
Chinese
|
Lee (1988) [5]
|
USA
|
|
8
|
53
|
M
|
Disseminated superficial
|
Portuguese
|
Hill (1992) [6]
|
France
|
|
9
|
63
|
M
|
Disseminated superficial
|
Japanese
|
Yasuda (1996) [7]
|
Japan
|
|
10
|
60
|
M
|
Disseminated superficial
|
Japanese
|
Yasuda (1996) [7]
|
Japan
|
|
11
|
72
|
M
|
Disseminated superficial
|
ND
|
Amantea (1998) [8]
|
Italy
|
|
12
|
63
|
M
|
Disseminated superficial
|
Japanese
|
Demitsu (1999) [9]
|
Japan
|
|
13
|
86
|
M
|
Mibelli (multiple lesions)
|
Japanese
|
Kuno (1999) [10]
|
Japan
|
|
14
|
81
|
F
|
Mibelli (solitary lesion)
|
Japanese
|
Kuno (1999) [10]
|
Japan
|
|
15
|
83
|
M
|
Disseminated superficial
|
ND
|
Kim (2000) [11]
|
Korea
|
|
16
|
51
|
M
|
Atypical type (ptychotropica)
|
ND
|
Jee (2003) [12]
|
Korea
|
|
17
|
70
|
M
|
Disseminated superficial
|
Spanish
|
García-Villalta (2004) [13]
|
Spain
|
|
18
|
70
|
F
|
Disseminated superficial
|
Spanish
|
García-Villalta (2004) [13]
|
Spain
|
|
19
|
65
|
M
|
Disseminated superficial
|
Spanish
|
Our patient
|
Spain
|
References
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