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Disseminated superficial porokeratosis with amyloid deposits

European Journal of Dermatology. Volume 15, Number 4, 298-300, July-August 2005, Clinical report

Summary

Author(s) : Manuel Ginarte, Álvaro León, Jaime Toribio , Department of Dermatology, Complejo Hospitalario Universitario de Santiago, Faculty of Medicine Santiago de Compostela, Spain.

Summary : We report a 65-year-old Caucasian man who exhibited a disseminated superficial porokeratosis. Histological examination with haematoxylin and eosin stain revealed the presence of dermal deposits of an eosinophilic amorphous material that stained positively with crystal violet and showed bright yellow fluorescence with thioflavin-T under the polarizing microscopy, indicating that it was amyloid. Colloid bodies from porokeratotic lesions can degenerate into amyloid, but it is not usually detected with haematoxylin and eosin stains because of its small quantity. Probably amyloid deposits could be detected more frequently in porokeratosis if histochemical techniques were carried out in a routine way.

Keywords : amyloidosis, cutaneous amyloidosis, disseminated superficial porokeratosis, porokeratosis

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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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2 Runne U, Orfanos CE. Amyloid production by dermal fibroblasts. Br J Dermatol 1977; 97: 155-66.

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12 Jee MS, Chang SE, Suh HS, et al. Porokeratosis ptychotropica associated with dermal amyloid deposits. Clin Exp Dermatol 2003; 28: 450-2.

13 García-Villalta MJ, Daudén E, Ruiz-Genao D, et al. Dermal amyloid deposits in disseminated superficial porokeratosis. Acta Derm Venereol 2004; 84: 173-4.

14 Aso M, Hagari Y, Nakamura K, et al. A case of secondary cutaneous amyloidosis: epidermal keratinocytes produce amyloid in the cytoplasm. J Cutan Pathol 1990; 17: 176-81.