Acrokeratosis paraneoplastica (Bazex syndrome): An atypical presentationDepartment of Dermatology, Kasturba Medical College, Manipal, Karnataka India. firstname.lastname@example.org
Raghavendra Rao, and Shrutakirthi D Shenoi
Dermatology Online Journal 10 (1): 21
A 62-year-old male presented with a 2-year history of hyperkeratotic lesions of the hands and feet. Previous treatment with topical steroids was unsuccessful. A complete physical examination revealed the presence of blood in the stool, and sigmoidoscopy showed an ulcerative growth at the rectosigmoid junction. The histopathology showed adenocarcinoma.
Acrokeratosis paraneoplastica is a rare but distinct dermatosis described by Bazex et al. in 1965 . It is characterized by psoriasiform-erythematous plaques, typically affecting hands, feet, nose, and earlobes. This condition is associated with internal malignancy, usually squamous cell carcinoma of the upper-aerodigestive tract, or cervical lymph node metastases from an unknown-primary tumor . Other rare associations include adenocarcinoma of the stomach, esophagus, or uterus, anaplastic small-cell carcinoma of the lung, Hodgkin's lymphoma , transitional-cell carcinoma of the bladder , adenocarcinoma of the colon , and squamous cell carcinoma of the lower leg .
A 62-year-old male presented with complaints of itchy, scaly lesions of hands and feet for 2 years. He initially developed pruritic vesicles and scaly plaques in the periungual areas of the right foot. Later, similar lesions appeared on the left foot and on both hands. Various topical steroids had no effect on the lesions.
Examination disclosed multiple hyperpigmented scaly papules and plaques over the dorsum and palmoplantar surfaces of the hands and feet (Fig. 1). Examination of the nails showed thickening and brownish discoloration of nail plates with subungual hyperkeratosis involving a few nails of the hands and feet.
Routine laboratory examination of blood and urine was normal, but an examination of stool showed frank blood on 2 occasions. Sigmoidoscopy showed an ulcerative growth at the rectosigmoid junction. A biopsy of that lesion revealed well-differentiated adenocarcinoma.
Skin biopsies of palmar and plantar lesions showed gross hyperkeratosis, acanthosis, and regular psoriasiform-elongation of rete ridges. PAS staining did not reveal dermatophytes. Although nail clippings were positive for dermatophyte, KOH preparations from palms and soles were negative. Treatment for 1 month with fluconazole (150 mg per week) did not produce any improvement in the appearance of the palms and soles.
Acrokeratosis paraneoplastica is a rare paraneoplastic syndrome . Its presence should prompt an extensive search for occult cancer. It is more common in males, with mean age of onset around age 61 years. The skin eruption precedes the detection of an underlying malignancy in 67 percent of cases, with a reported median interval of as long as 1 year between the onset of the skin changes and the recognition of malignancy. Cutaneous manifestations follow the diagnosis of malignancy in 15 percent of cases. In the remainder, the onset of skin lesions and the diagnosis of the tumor occur concurrently. Usually the course of the dermatosis parallels that of the tumor growth; the skin eruption resolves only when the tumor is effectively treated .
Bazex and Griffith  described the 3 stages of evolution of the disease:
- Stage I. Erythema and psoriasiform scaling of fingers and toes, ear helices and nose are present. There are also nail changes, including subungual hyperkeratosis, onychodystrophy, longitudinal bands of discoloration, and onycholysis.
- Stage II. Involvement of palms and soles is common. Facial lesions involve cheek and pinna. Malignancy usually becomes evident in this stage.
- Stage III. Involvement extends to legs, knees, thighs, arms, trunk and scalp.
Associated features of acrokeratosis paraneoplastica include pruritus, sterile paronychia, vesicles and bullae, hyperpigmentation, hypopigmentation, and carpal tunnel syndrome . The diagnosis is based on clinical features since the histopathology is nonspecific . Pecora et al. described IgG, IgM, IgA, and C3 along the basement-membrane zone of lesional and nonlesional skin by direct immunofluorescence . However, others could not reproduce these results .
Our case is unusual because the eruption lacked erythema and was limited to acral areas with sparing of ears and nose. The associated internal malignancy was an adenocarcinoma of the rectosigmoid junction and not the usual squamous cell carcinoma. To our knowledge, there is only 1 report of such an association.
References1. Bazex A, Griffith A. Acrokeratosis paraneoplastica--a new cutaneous marker of malignancy. Br J Dermatol. 1980 Sep;103(3):301-6. PubMed
2. Pecora AL, Landsman L, Imgrund SP, Lambert WC. Acrokeratosis paraneoplastica (Bazex' syndrome). Report of a case and review of the literature. Arch Dermatol. 1983 Oct;119(10):820-6. Review. PubMed
3. Lucker GP, Steijlen PM. Acrokeratosis paraneoplastica (Bazex syndrome) occurring with acquired ichthyosis in Hodgkin's disease. Br J Dermatol. 1995 Aug;133(2):322-5. PubMed
4. Arregui MA, Raton JA, Landa N, Izu R, Eizaquirre X, Diaz-Perez JL. Bazex's syndrome (acrokeratosis paraneoplastica)--first case report of association with a bladder carcinoma. Clin Exp Dermatol. 1993 Sep;18(5):445-8. PubMed
5. Hsu YS, Lien GS, Lai HH, Cheng YS, Hu CH, Hseih MC, Fang CL, Pan S. Acrokeratosis paraneoplastica (Bazex syndrome) with adenocarcinoma of the colon: report of a case and review of the literature. J Gastroenterol. 2000;35(6):460-4. PubMed
6. Hara M, Hunayama M, Aiba S, Suetake T, Watanabe M, Tanaka M, Tagami H. Acrokeratosis paraneoplastica (Bazex syndrome) associated with primary cutaneous squamous cell carcinoma of the lower leg, vitiligo and alopecia areata. Br J Dermatol. 1995 Jul;133(1):121-4. PubMed
7. Bolognia JL. Bazex syndrome: acrokeratosis paraneoplastica. Semin Dermatol. 1995 Jun;14(2):84-9. PubMed
8. Richard M, Giroux JM. Acrokeratosis paraneoplastica (Bazex' syndrome). J Am Acad Dermatol. 1987 Jan;16(1 Pt 2):178-83. PubMed
© 2004 Dermatology Online Journal