Clinical and Experimental Dermatology (1977) 2, 167.
Clinical meeting of the St John's Hospital Dermatological Society: 6 November 1975
Malignant acanthosis nigricans JACQUELINE CLARKE Department of Dermatology, St Thomas' Hospital, London SEi 7EH
Accepted for publication ij January 1977
Case report History. M.C., a 61-year-old male Shopkeeper, presented in November 1975 with a 15 month history of wart-like lesions on both lips. These had begun at the angles of the mouth and slowly spread. He had also noticed cracking and soreness of the tongue. In January 1973 he had had a right pneumonectomy for squamous cell carcinoma. The hilar nodes were involved at the time of operation. He remained well until July 1974 when he developed a hoarse voice. Investigations then revealed multiple benign squamous cell papillomata on the larynx. In October 1975 he became breathless due to superior vena caval obstruction and was undergoing radiotherapy for this at the time of his referral with 'warts'. Examination. There were numerous filiform wart-like lesions on both lips (Fig. i). The tongue was deeply fissured. Typical changes of extensive acanthosis nigricans were present on the skin of the neck, the axillae (Fig. 2) and natal cleft (Fig. 3). His palms were thickened and showed an early form of the *tripe' pattern (Fig. 4). General examination revealed a right thoracotomy scar with diminished air entry on that side. There were dilated superficial veins over the upper chest and a palpable lymph node in the right anterior triangle of the neck. Investigations. ESR 45 mm/h. The following were all normal; Full blood count, urea, electrolytes, liver function tests, cortisols, extended glucose tolerance test, ACTH, ^MSH and growth hormone levels. There was no evidence of a 'growth hormone-like' peptide in the blood. He had a raised plasma calcitonin of 1-25 ^g/1 (normal range o-i Histology. Biopsy from a warty lesion on the lip showed marked warty hyperplasia of the epidermis. No virus particles could be identified on electron microscopy. 167
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Jacqueline Clarke
Figure i. Malignant acanthosis nigricans. Warty lesions on lips.
Figure 2. Malignant aeanthosis nigricans affecting the axilla. Figure 3. Malignant aeanthosis nigrieans. Extensive changes in the natal cleft.
Malignant acanthosis nigricans
169
Figure 4. Malignant acanthosis nigrieans showing velvety thiekening on the dorsum of the hand with 'tripe' palmar changes.
Comment In 1890 two independent observers, Pollitzer and Janovsky, first recognized that in their middle-aged patients, acanthosis nigricans could be associated with cancer. Since then numerous case reports have suggested that only 50X of patients with this skin lesion have a neoplasm, the remainder being either genetically determined or related to obesity (pseudo acanthosis nigricans, Curth, 1952). The 'malignant form' is usually associated with an adenocarcinoma, which in 90% of cases is in the gastro-intestinal tract. The skin lesions may precede other symptoms by up to 5 years, and may improve when the primary tumour is removed. The mechanism by which a carcinoma induces acanthosis nigricans is uncertain. Millard & Gould (1976) have reported three patients with late onset palmoplantar hyperkeratosis, clinically similar to the 'tripe palm' abnormality. These patients had no other changes of acanthosis nigricans, but all had internal malignancy, and two had raised growth hormone levels. This report strengthens the view that classical tnalignant acanthosis nigricans may be caused by ectopic hormone production by the tumour mass. Our patient is interesting in several respects. His original tumour was of squamous cell type and was removed 2 years before the onset of cutaneous signs. The appearance of acanthosis nigricans was therefore presumably related to metastatic spread. His axillary, palm and perianal lesions were typical of acanthosis nigricans but he has unusually extensive mucous membrane involvement, on the lips, tongue and larynx.
170
Jacqueline Clarke
.•'
The hypertrophic warty appearance of his lips prompted investigations for (i) the presence of wart virus or (2) excess circulating growth hormone. No wart virus was detected on electron microscopy and his cortisol, ACTH, /?MSH and growth hormone levels were normal. The raised calcitonin level is compatible with ectopic hormone production by the tumour (Sonksen, 1976). Despite these investigations, the mechanism of production of the extensive acanthosis nigricans remains obscure. It is of interest that this man's skin showed slight improvement during two short courses of radiotherapy, but is now deteriorating as further metastases develop. References CuRrH, H.O. (1952) Significance of acanthosis nigricans. Archives of Dermatology and Syphilology, 66, 80-100.
V. (1890) Acanthosis nigricans. In: Internationaler Atlas Seltener Hautkrankheiten, Heft IV, nr. XI. Leopold Voss, Leipzig. MILLARD, L.G. & GOULD, D.J. (1976) Hyperkeratosis of the palms and soles associated with internal malignancy and elevated levels of immunoreactive human growth hormone. Clinical and Experimental Dermatology, i, 363-368. POLLITZER, S. (1890) Acanthosis nigricans. In: Internationaler Atlas Seltener Hautkrankheiten, Heft IV, nr. X. Leopold Voss, Leipzig. SONKSEN, P. (1976) Personal communication. JANOVSKY,
Clinical meeting of the St John's Hospital Dermatological Society: 6 November 1975
Malignant acanthosis nigricans JACQUELINE CLARKE Department of Dermatology, St Thomas' Hospital, London SEi 7EH
Accepted for publication ij January 1977
Case report History. M.C., a 61-year-old male Shopkeeper, presented in November 1975 with a 15 month history of wart-like lesions on both lips. These had begun at the angles of the mouth and slowly spread. He had also noticed cracking and soreness of the tongue. In January 1973 he had had a right pneumonectomy for squamous cell carcinoma. The hilar nodes were involved at the time of operation. He remained well until July 1974 when he developed a hoarse voice. Investigations then revealed multiple benign squamous cell papillomata on the larynx. In October 1975 he became breathless due to superior vena caval obstruction and was undergoing radiotherapy for this at the time of his referral with 'warts'. Examination. There were numerous filiform wart-like lesions on both lips (Fig. i). The tongue was deeply fissured. Typical changes of extensive acanthosis nigricans were present on the skin of the neck, the axillae (Fig. 2) and natal cleft (Fig. 3). His palms were thickened and showed an early form of the *tripe' pattern (Fig. 4). General examination revealed a right thoracotomy scar with diminished air entry on that side. There were dilated superficial veins over the upper chest and a palpable lymph node in the right anterior triangle of the neck. Investigations. ESR 45 mm/h. The following were all normal; Full blood count, urea, electrolytes, liver function tests, cortisols, extended glucose tolerance test, ACTH, ^MSH and growth hormone levels. There was no evidence of a 'growth hormone-like' peptide in the blood. He had a raised plasma calcitonin of 1-25 ^g/1 (normal range o-i Histology. Biopsy from a warty lesion on the lip showed marked warty hyperplasia of the epidermis. No virus particles could be identified on electron microscopy. 167
i68
Jacqueline Clarke
Figure i. Malignant acanthosis nigricans. Warty lesions on lips.
Figure 2. Malignant aeanthosis nigricans affecting the axilla. Figure 3. Malignant aeanthosis nigrieans. Extensive changes in the natal cleft.
Malignant acanthosis nigricans
169
Figure 4. Malignant acanthosis nigrieans showing velvety thiekening on the dorsum of the hand with 'tripe' palmar changes.
Comment In 1890 two independent observers, Pollitzer and Janovsky, first recognized that in their middle-aged patients, acanthosis nigricans could be associated with cancer. Since then numerous case reports have suggested that only 50X of patients with this skin lesion have a neoplasm, the remainder being either genetically determined or related to obesity (pseudo acanthosis nigricans, Curth, 1952). The 'malignant form' is usually associated with an adenocarcinoma, which in 90% of cases is in the gastro-intestinal tract. The skin lesions may precede other symptoms by up to 5 years, and may improve when the primary tumour is removed. The mechanism by which a carcinoma induces acanthosis nigricans is uncertain. Millard & Gould (1976) have reported three patients with late onset palmoplantar hyperkeratosis, clinically similar to the 'tripe palm' abnormality. These patients had no other changes of acanthosis nigricans, but all had internal malignancy, and two had raised growth hormone levels. This report strengthens the view that classical tnalignant acanthosis nigricans may be caused by ectopic hormone production by the tumour mass. Our patient is interesting in several respects. His original tumour was of squamous cell type and was removed 2 years before the onset of cutaneous signs. The appearance of acanthosis nigricans was therefore presumably related to metastatic spread. His axillary, palm and perianal lesions were typical of acanthosis nigricans but he has unusually extensive mucous membrane involvement, on the lips, tongue and larynx.
170
Jacqueline Clarke
.•'
The hypertrophic warty appearance of his lips prompted investigations for (i) the presence of wart virus or (2) excess circulating growth hormone. No wart virus was detected on electron microscopy and his cortisol, ACTH, /?MSH and growth hormone levels were normal. The raised calcitonin level is compatible with ectopic hormone production by the tumour (Sonksen, 1976). Despite these investigations, the mechanism of production of the extensive acanthosis nigricans remains obscure. It is of interest that this man's skin showed slight improvement during two short courses of radiotherapy, but is now deteriorating as further metastases develop. References CuRrH, H.O. (1952) Significance of acanthosis nigricans. Archives of Dermatology and Syphilology, 66, 80-100.
V. (1890) Acanthosis nigricans. In: Internationaler Atlas Seltener Hautkrankheiten, Heft IV, nr. XI. Leopold Voss, Leipzig. MILLARD, L.G. & GOULD, D.J. (1976) Hyperkeratosis of the palms and soles associated with internal malignancy and elevated levels of immunoreactive human growth hormone. Clinical and Experimental Dermatology, i, 363-368. POLLITZER, S. (1890) Acanthosis nigricans. In: Internationaler Atlas Seltener Hautkrankheiten, Heft IV, nr. X. Leopold Voss, Leipzig. SONKSEN, P. (1976) Personal communication. JANOVSKY,
