Palmoplantar filiform hyperkeratosis with Darier’s disease. Association or coincidence?

ARTICLE

Palmoplantar orthokeratotic filiform hyperkeratosis (PPOFH) is a rare disorder, characterized by multiple, spiny, keratotic projections. In 1992, Zarour et al. [1] described the first case of PPOFH in a patient with Darier's disease. We report a similar observation.

Case report

An 85-year-old woman was referred to us because of papulokeratotic plaques on her chest and groin, that she had had for 30 years. She had warty follicular papules, distributed over the central and seborrhoeic areas of the back (Fig. 1), and over the groin, suggestive of Darier's disease. Examination of palms and soles revealed multiple, spiny, hyperkeratotic projections (Fig. 2). The patient could not state precisely the date of onset of the lesions and reported that her daughter had similar lesions on her chest. Each keratotic lesion on the palms and soles measured about 3 mm in length and 1.5 mm in diameter. The base was firmly attached to the underlying skin. Apart from osteoporosis, the patient was in good general health, general physical examination being otherwise normal.

Histopathological examination of a skin biopsy specimen of a chest lesion showed dyskeratosis with suprabasal acantholysis, typical of Darier's disease (Fig. 3). Histological examination of a biopsy specimen performed on a palmar plug showed a column of orthokeratotic hyperkeratosis without acantholysis (Fig. 4).

The patient refused any therapy or follow-up.

Discussion

Spiny keratoderma is a rare disorder, described in 1967 by Goldstein [2]. It is characterized by multiple, thin, spiny keratotic projections on the palms and soles or disseminated on various regions of the body. Its clinical differential diagnoses include punctate porokeratosis, common warts, arsenical keratoses, acanthosis nigricans and lichen nitidus [3]. Zarour et al. [1] have proposed a classification of this keratinization disorder, on the basis of clinical (three patterns: palmar and plantar, disseminated with palmoplantar sparing, and linear), and histological (two types: parakeratosis or orthokeratosis) features. According to this definition, we report the fifth case of PPOFH. There was no apparent clinical association in the first reported observation [4]. Two further cases were associated with cancer of the rectum [5, 6]. The fourth case, which involved a 70-year-old man, described by Zarour et al. [1], was clinically similar to ours, and occurred in association with Darier's disease.

Filiform palmoplantar hyperkeratosis, either orthokeratotic or parakeratotic, is frequently associated with malignant neoplasms [3, 5-11]. As a rule, it cannot be considered as an actual paraneoplastic syndrome, since it often begins several years before the diagnosis of cancer, and persists after its removal. Nevertheless, it is associated with an underestimated risk of malignancy, and patients should be carefully examined to detect any underlying neoplasia. In view of the age of our patient, and because of the normal physical examination, we did not performed any further investigations.

In some Darier's disease lesions, such as acrokeratosis verruciformis, there is no acantholysis. Similarly, PPOFH may be considered histologically as a clinical manifestation of Darier's disease, that appears in older patients. This hypothesis could explain the rarity of the association in the literature. In addition, patients, who have become accustomed to their cutaneous disease, are not necessarily inclined to seek advice when palmoplantar lesions occur.

Alternatively, Darier's disease and PPOFH may also be considered as two different dermatoses. Of course, the coexistence of the two diseases may be a coincidence. This new observation, however, underlines the possibility of an association which may be not fortuitous. If similar
cases are reported, molecular studies would be helpful to understand the transmission of this keratinization disorder.

REFERENCES

1. Zarour H, Grob JJ, Andrac L, Bonerandi JJ. Palmoplantar orthokeratotic filiform hyperkeratosis in a patient with associated Darier's disease. Classification of filiform hyperkeratosis. Dermatology 1992; 185: 205-9.

2. Goldstein N. Multiple minute digitate hyperkeratoses. Arch Dermatol 1967; 96: 692-3.

3. Kaddu S, Soger P, Kerl H. Palmar filiform hyperkeratosis: a new paraneoplastic syndrome? J Am Acad Dermatol 1995; 33: 337-40.

4. Knobler EH, Grossman ME, Rabinowitz AD. Multiple minute palmar-plantar digitate hyperkeratoses. Br J Dermatol 1989; 121: 239-42.

5. Fegueux S, Bilet S, Crickx B, Perron J, Grassin M, Belaïch S. Hyperkératose palmo-plantaire filiforme et cancer recto-sigmoïdien. Ann Dermatol Venereol 1988; 115: 1145-6.

6. Le Guyadec T, Beaulieu P, Darie H, Fournier B, Gros P, Millet P. Hyperkératose palmaire filiforme orthokératosique: un nouveau cas associé à un cancer rectal. Ann Dermatol Venereol 1994; S123: 91.

7. Herman PS. Punctate porokeratotic keratoderma. Dermatologica 1973; 147: 206-13.

8. Beylot C, Taïeb A, Bioulac P, Doutre MS, Foix P. Hyperkératose palmo-plantaire filiforme et néoplasie viscérale. Ann Dermatol Venereol 1982; 109: 747-8.

9. Bianchi L, Orlandi A, Iraci S. Punctate porokeratotic keratodermal: its occurrence with internal neoplasia. Clin Exp Dermatol 1994; 19: 139-41.

10. Hillion B, Le Bozec P, Moulonguet-Michau I, Blanchet-Bardon C, Petit A, Stephan J, Civatte J. Hyperkératose palmo-plantaire filiforme et cancer du sein. Ann Dermatol Venereol 1990; 117: 834-6.

11. Rault S, Salmon-Ehr V, Cambie MP, Armingaud P, Barhoum K,
Ploton D, Kalis B. Hyperkératose palmo-plantaire filiforme parakératosique et adénocarcinome digestif. Ann Dermatol Venereol 1997; 124: 707-9.