Dermatologica 143: 95-99 (1971)

Acrokeratosis Verruciformis: Malignant Transformation

M. D ogliotti and A. Schmaman

Departments of Dermatology and Pathology Baragwanath Hospital and

the South African Institute for Medical Research, Johannesburg

Abstract. A case of acrokeratosis verruciformis in which squamous carcinoma occurred

is described. No previous report of this occurrence could be found. The authors consider
acrokeratosis verruciformis as a distinctive disease and not a variation of Darier’s disease.

Acrokeratosis verruciformis is an uncommon disorder of the skin first

described in 1930 by H opf [4] who reported 2 cases at a meeting of the
‘Dermatologische Gesellschaft Hamburg-Altona’. In a recent survey of this
condition C osta [2] pointed out that although 40 cases have been recorded in
various papers its anatomic patterns have not yet been defined. Including the
disease amongst the epithelial nevi (hyperplasia of epithelial cells in absence
of ordinary nevus cells), M ontgomery [5] described acrokeratosis verruci­
formis as follows: ‘Verrucous lesions on the dorsum of the hands and feet
with some involvement of the palms and flexural surface of the fingers,
wrists, and forearms.’
In a detailed study on verrucoid changes in Darier’s disease W aisman [8]
described acral lesions histologically indistinguishable from either Unna’s
hard nevus and Hopf’s disease. In his conclusion W aisman pointed out that
acrokeratosis verruciformis may be a variant of keratosis follicularis (Darier’s)
and seborrheic keratosis. This view is not shared by M ontgomery who
draws attention to the fact that benign dyskeratosis is very rarely seen in
Hopf’s disease.
Present at birth or developing during childhood or even in adults, acro­
keratosis verruciformis has shown its familial character in the reports of
N iedelman [6] in which 14 cases were described in 20 members of 4genera-

Received: October 30, 1970; accepted: December 22, 1970.

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96 Dogliotti/S chmaman

tions, D anbolt [3] 3 cases in 3 generations and Rinsema [7] in siblings of the
same generation. It is an autosomal dominant rather than an x-linked type
of inheritance. In a recent review of this subject Bureau et al. [1] stressed the
benign course of the disease and its persistency during the entire life of
patients despite the most modern methods of treatment: 'Aucune thérapeu­
tique ne les influence, mais aucune complication n’a été signalé, en particulier
il ne se produit pas de dégénérescence epithéliomateuse.' Acrokeratosis verruci­
formis is therefore a benign nevoid condition without tendency to become
malignant.
A recent case seen in a Bantu male with malignant changes in a lesion in
the right malleolar area is, therefore, worthy of description.

Case Report

Piet N., a 48-year-old Bantu male bom in Botswana was admitted to the Dermatology
division of Baragwanath Hospital on 23rd October, 1969. There was no history of con­
sanguinity. At the age of 15 years he noticed a few warty patches on both hands and feet.
They increased in size and number very slowly during the past 32 years. Six months before
attending the hospital, a tumor-like lesion appeared on the lateral aspect of the right
malleolus where previous warty elements were present. This was unrelated to trauma or
any local treatment. Very recently the tumor-like lesion started ulcerating.
Examination. His general condition was good. He was cooperative and well orientated.
No abnormality of the scalp, teeth or nails were present. On the dorsal aspect of the
metacarpophalangeal joints of some fingers there were scattered warty hard lesions,
moderately raised, hyperkeratotic and showing tendency to group in patches. Similar
elements, irregularly distributed, were present on the lateral aspect of both ankles. No
other skin disorders were evident on the trunk or mucous membranes. The palms and
soles showed moderate hyperkeratosis. Just posterior to the right lateral malleolus there
was a ring-like ulcer about 2 cm in diameter. The edges were raised and hard and the floor
w'as hard and moderately granular but showing no tendency to heal. On microscopic and
cultural examinations the latter yelded cellular debris and a growth of Proteus tnirabilis,
S. pyogenes, E. coli and S. faecalis on 3 specimens.
Histological investigation showed considerable hyperkeratosis with prominence of the
granular layer. There was a mild papillomatosis with several peaks of epidermis pushing
up into the thick keratin layer. A moderate degree of acanthosis was present with uni­
form elongation of the retc ridges (fig. I). In the ulcerated area there was a transition to a
well differentiated squamous-cell carcinoma, with invasion of the dermis by irregular is­
lands and strands of markedly pleomorphic epidermal cells. Abnormal mitoses were
present and nuclear variation and hyperchromatism, was a prominent feature. Keratin-
ization with the formation of horn cells was present in several areas (fig. 2, 3).
The patient was referred for radiotherapy. He did not return for follow-up examinations
after treatment.
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 Acrokeratosis Verruciformis: Malignant Transformation 97

Fig. I. Marked hyperkeratosis with slight papillomatosis and acanthosis (H E x 30).

Fig. 2. Well differentiated area showing a keratin pearl at the bottom right side
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(H-E x 300).
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98 D ogliotti/S chmaman

Fig. 3. An area showing marked nuclear pleomorphism and abnormal mitoses

(H-E x 600).

Discussion

To our knowledge, this is the first reported case of acrokeratosis verruci­

formis in which malignancy has occurred in one of the lesions. Different
views on the prognosis and treatment should, therefore, be considered.
Although one cannot draw conclusions from a single isolated occurrence,
our case would support the view that acrokeratosis verruciformis is an indi­
vidual disease and not a form fruste of Darier’s disease. There may be some
resemblance on clinical and histological grounds to Darier’s disease as well
as seborrheic keratosis. No malignant change has been reported in these two
conditions. There may also be some resemblance to epidermodysplasia
verruciformis. This disease may be distinguished histologically by the cons­
picuous vacuolation of epidermal cells.

A cknowledgement

We wish to thank the Superintendent of Baragwanath Hospital Dr. C. H. K nikp for

permission to publish this case and Prof. J. H. S. G ear, Director of the SA Institute for
Medical Research for facilities granted, and Mr. U lrich for the photomicrographs.
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 Acrokeratosis Verruciformis: Malignant Transformation 99

References

1. Bureau, Y.; Barriere, H .; L itoux , P. et Bureau, L.: Acrokératose verruciforme.

Discussion de son individualité á propos d'une observation. Ann. Derm. Syph., Paris
92: 269 (1965).
2. C osta, O. G.: Acrokeratosis, vol. I (1962).
3. D anbolt : Cited by Bureau et at. (1965).
4. H opf , G.: Warzenartige Keratosen an Hand- und Fussriicken (acrokeratosis verruci­
forme). Congr. int., Copenhagen (Engelsen and Schroder, Copenhagen 1922).
5. Montgomery, H .: in H ober Dermatology, vol. 2, p. 869 (Harper and Row, New
York 1967).
6. N iedelman, M. I.: Acrokeratosis verruciformis (Hopf). Arch. Derm, and Syph.,
Bcrl. 55: 598 (1947).
7. R insema: Cited by Bureau et al. (1965).
8. W aisman, M.: Verruciform manifestation of keratosis follicularis including a reapp­
raisal of hard nevi (Unna). Arch. Derm., Chicago 81: I (I960).

Authors' address: Dr. M. D ogliotti and Dr. A. Schmaman, Department of Dermatology,

Baragwanath Hospital, South African Institute for Medical Research, Johannesburg
(South Africa)
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