ELSEVIER Journal of the European Academy of Dermatology and Venereology

6 (1996) 187-190

Case report

Acquired ichthyosis associated with Skzary’s syndrome

Carlos de la Torre *, Antonio Losada, Manuel J. Cruces Servicio de Dermatologia, Hospital Provincial de Pontevedra, C. Loureiro Crespo s/n, 36001 Pontevedra, Spain

Abstract

We report a case of a 70-year-old woman affected by acquired ichthyosis. Morphologic studies of mononuclear cells in peripheral blood revealed that 50% were SCzary cells, and analyses of surface phenotype disclosed a CD4 predominance. Reports of association with cutaneous T cell lymphoma, mycosis fungoides type, have been described, but we have found no reports with SCzary’s syndrome.

Keywords: Cutaneous T cell lymphoma; SCzary’s syndrome; Acquired ichthyosis; Paraneoplastic disorders

1. Case report

A 70-year-old woman, without personal or family history of interest was referred to our clinic for evaluation of a dermatosis. The patient complained of itchy, dry, scaling skin, with onset on the legs two years earlier, and which had gradually worsened and included generalized ery- thema in the last two months.

Examination revealed generalized erythema- tous infiltration of the skin, prominent on the face with ectropion. On the trunk, especially on the flanks and lower back, the skin was dry with ichthyosiform 8 desquamation. The scaling was most severe on the extremities, particularly on the extensor surfaces, and mildly on the flexural creases, with large polygonal adherent scales, with

* Corresponding author. Tel: + 34 86 855500, Ext. 296; fax: + 34 86 860767.

a brownish hue on the legs (Figs. 1,2). The palms and soles were hyperkeratotic, and there were generalized palpable subcutaneous lymph nodes and hepatomegaly.

Initial laboratory data revealed a white blood cell count of 21600 (27% polymorphonuclear leukocytes, 64% lymphocytes, 9% monocytes) and the erythrocyte sedimentation rate was 83 mm/h. Results of other laboratory tests were normal with the exception of slightly raised levels of IgG and IgA and lactate dehydrogenase.

Morphologic studies of mononuclear cells in peripheral blood revealed that 50% were SCzary cells, and analyses of surface phenotype disclosed a CD4 predominance. Chest X-ray, and abdomi- nal ultrasonography were normal, as were bone marrow smears.

A skin biopsy showed an atrophic epidermis with compact hyperkeratosis and a thin granular layer, consistent with ichthyosis of the vulgaris or acquired type. In the papillary dermis there was a

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188 C. de la Torre et al. /J. Eur. Acad. Dermatol. Venereol. 6 (1996) 187-190

Fig. 1. Brownish polygonal, adherent scales, typical of ichtyosis.

band-shaped mononuclear infiltrate, some with indented nuclei, with the presence of occasional atypical epidermotropic cells (Fig. 3). Electron microscopy identified atypical cerebriform SCzary cells. The dermal and intraepidermal lympho- cytes predominantly carried CD2, CD5 and CD3 antigens, with CD4 positivity in 75% (Fig. 4) and CDS in less than lo%, being markers for B cells, CD19 and CD22, negative.

2. Discussion

The ichthyosiform dermatoses clinically and histologically similar to autosomal dominant ichthyosis vulgaris, but not genetically deter- mined, have been termed as acquired ichthyosis, although some authors prefer to define them as ichthyosiform conditions (states) or symptomatic ichthyosis [l-4]. Hyperkeratosis of palms and soles, alopecia, a tendency to affect cutaneous folds and pruritus are said to occur more fre- quently in acquired ichthyosis [5].

Fig. 2. Brownish polygonal, adherent scales, typical of ichtyosis.

C. de la Torre et al. /J. Eur. Acad. Dennatol. Venereal. 6 (1996) 187-190 189

Fig. 3. Compact hyperkeratosis with a thin granular layer. Band-like lymphoid dermal infiltrate with focal epidermotropism of am lical cells (HE 200).

Fig. 4. Lymphoid cells show a predominant helper T cell phenotype (CD 4, Leu-3a) (Immunoperoxidase stain 2.50).

190 C. de la Torre et al. /J. Eur. Acad. Dermatol. Venereol. 6 (1996) 187-190

Acquired ichthyotic changes have been de- scribed in a variety of systemic disorders, or in- duced by certain drugs, but their onset during adult life is very rare and usually indicates an underlying malignant process, especially Hodgkin’s disease [3-121.

Reports of association with cutaneous T cell lymphoma (CTCL), mycosis fungoides type, have been described, but we have found no reports with SCzary’s syndrome [3-51.

SCzary’s syndrome is a characteristic form of CTCL, defined by the classic triad of erythro- derma, generalized lymphadenopathy, and the presence of atypical circulating mononuclear cells (SCzary cells) [13-141. The erythroderma of SCzary’s syndrome usually presents as an edema- tous infiltrated one, with a tendency towards pig- mentation (melanoerythroderma), and is associ- ated with pronounced itching, hyperkeratosis of palms and soles, onychodystrophy, diffuse alope- cia and ectropion [Xl. A certain grade of scaling, lamellar or bran-like, may be present, but it is not clearly ichthyotic, thus allowing the differentia- tion [51.

A parallel course of acquired ichthyosis with that of underlying neoplasia has been described [10,13]. In the case herein reported a marked improvement of the ichthyotic skin followed con- trol of the leukemic CTCL, although no cure occurred, and the patient died three years after the diagnosis.

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