Dermatology Online Journal

Acrokeratosis paraneoplastica (Bazex syndrome): Case report and review of literature
Vikas Sharma, M.D., Nand Lal Sharma, M.D., Nitin Ranjan, M.B.B.S., Geeta Ram Tegta, M.D., Sandip Sarin, M.B.B.S.
Dermatology Online Journal 12 (1): 11

Department of Dermatology, Venereology & Leprosy, Indira Gandhi Medical College, Shimla, India. drvcsharma2003@rediffmail.com

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Abstract

Acrokeratosis paraneoplastica of Bazex is a rare syndrome. This condition is a distinct skin marker of supra-diaphragmatic neoplasia. This syndrome is important because the cutaneous findings preceed the onset of symptoms referable to the underlying neoplasm by several months in majority of the cases. We report here a case of a 63-year-old cachectic female who had classic psoriasiform lesions of acrokeratosis of Bazex with underlying bronchogenic carcinoma of the right lung.

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Introduction

First described by Bazex et al. [1] in 1965 as a skin marker of supra-diaphragmatic neoplasia, this syndrome is a rare but distinct and an important sign of internal malignancy. It has been reported previously with squamous cell carcinoma of the upper aerodigestive tract, most commonly with neoplasia of lower lip, tongue, esophagus, pharyngolaryngeal region, upper third of the lung, and carcinoma of the stomach metastasizing to the cervical region [2].

This syndrome is important because the cutaneous findings precede the onset of symptoms referable to the underlying neoplasm by several months in majority of the cases, thus providing an essential clinical and diagnostic clue for the search of occult neoplasm [1]. We present here a case of an elderly female who was being treated for her chest symptoms on the lines of chronic obstructive pulmonary disease. She was sent to us for consultation regarding her skin lesions.

Clinical synopsis

A 63-year-old female was referred to our department with a 10-month history of multiple, well-defined, scaly, erythematous patches and plaques, distributed symmetrically over dorsa of both her hands and feet (Figs. 1 and 2 ).

Figure 1	Figure 2
Figure 1. Well-defined psoriasiform lesions, distributed symmetrically over dorsa of both hands.
Figure 2. Well-defined psoriasiform lesions, distributed symmetrically over dorsa of both feet.

Figure 3
Figure 3. Close-up view of the psoriasiform lesion present over dorsum of hand showing presence of a violaceous hue at the periphery.

These psoriasiform lesions had a peculiar violaceous hue at the periphery (Fig. 3). Similar lesions were also present over the helix of right ear. The palms and soles showed keratoderma-like changes.

She was admitted in the department of medicine with the complaints of significant weight loss (10-12 kg over 4 months), cough with expectoration (2 months), shortness of breath, anorexia, and chest pain (12-15 days). She had been a life-long smoker.

Her chest Roentgenogram showed a patch of irregular consolidation in the lower zone of right lung and a dense opaque shadow at the left lung root. Her plain and contrast CT chest findings (Figs. 4 and 5) were consistent with bronchogenic carcinoma right lung with secondary involvement of the lymph glands at the left lung root.

Figure 4	Figure 5
Figure 4: Plain CT chest with arrows highlighting the findings.
Figure 5: Contrast CT chest with arrows highlighting the findings.

Blood tests were normal. Biopsy from a skin lesion revealed hyperkeratosis, acanthosis, parakeratosis and a perivascular lympho-histiocytic infiltrate in the upper dermis.

Discussion

The key components of Bazex syndrome include an underlying internal malignancy with one or more of these cutaneous signs as follows: papulosquamous lesions, hyperpigmentation, keratoderma, paronychia, and nail dystrophy [3]. The classic cutaneous findings in Bazex syndrome are in the form of well-defined, scaly, erythematous to violaceous patches or plaques, distributed symmetrically over the acral locations [4]. Nail changes may include ridging, thickening, yellowish discoloration, and onycholysis [5]. Swelling of the digits and rarely bullae may be present [6].

A wide range of histological findings have been described in acrokeratosis of Bazex, commonly hyperkeratosis, parakeratosis, acanthosis, and a perivascular lympho-histiocytic infiltrate. Rarely dyskeratotic keratinocytes, vacuolar degeneration, band-like infiltrate, and melanin incontinence may be present [3,4].

If we correlate the temporal relationship between onset of the skin lesions and diagnosis of neoplasm, the psoriasiform lesions are known to precede the diagnosis of neoplasm in 65-70 percent of the patients. Cutaneous manifestations follow the diagnosis of neoplasm in 10-15 percent of patients. There is a simultaneous onset of skin lesions and diagnosis of tumor in 15-25 percent of the cases. Skin lesions either improve significantly or resolve completely when the underlying neoplasm is treated in 90-95 percent of the patients whereas they remain unchanged in the setting of persistent disease. The reappearance of skin lesions signal a recurrence of the tumor[6].

Therefore, all patients presenting with such lesions should undergo a thorough diagnostic workup that includes complete physical examination including pelvic examination, complete blood counts, blood biochemistry, chest Roentgenogram, a thorough head and neck examination, including endoscopic evaluation of larynx and pharynx, upper gastro-intestinal endoscopy, bronchoscopy, computed tomography scans of chest and abdomen.

Acrokeratosis paraneoplastica of Bazex typically exhibits 3 clinical stages[4-6] as follows:

• Stage 1, the skin lesions are not well-defined and involve helices of ears, nose, fingers, toes and nails. The tumor is said to be asymptomatic in this stage.
• Stage 2, the lesions tend to spread to additional sites, such as palms and soles. The stage is characterized by appearance of local symptoms referable to the tumor.
• Stage 3, if the tumor remains untreated, areas of erythema and scaling begin to develop on the elbows, knees and trunk.

Treatment is aimed at eradication of the underlying malignancy with surgery, chemotherapy, or radiotherapy. If the tumor is unresectable or resistant to treatment, then etretinate (0.75-1 mg/kg/d) is the drug of choice [7].

References

1. Bazex A , Griffiths A . Acrokeratosis Paraneoplastica Ð a new cutaneous marker of malignancy. Br j Dermatol 1980 ;103: 301Ð6. PubMed

2. Viteri A, Munoz A, Barcelo R. Acrokeratosis paraneoplastica (Bazex syndrome) preceeding the diagnosis of metastatic squamous cell carcinoma of the esophagus. J Am Acad Dermatol. 2005 Apr;52(4):711-2. PubMed

3. Pecora AL , Landsman L , Imgrund SP, et al. Acrokeratosis Paraneoplastica (Bazex' Syndrome):Report of a case and Review of the Literature. Arch Dematol 1983 ;119: 820- 6. PubMed

4. Bolognia JL . Bazex Syndrome : Acrokeratosis Paraneoplastica. Seminars Dermatol 1995 ;14: 84-9. PubMed

5. Baran R . Paraneoplastic acrokeratosis of Bazex. Arch Dematol 1977 ;113: 1613. PubMed

6. Jacobsen FK , Abildtrup N , Laursen SO, et al. Acrokeratosis Paraneoplastica (Bazex' Syndrome). Arch Dematol 1984 ;120: 502-4. PubMed

7. Wishart JM . Bazex paraneoplastic acrokeratosis: a case report and response to Tigason. Br j Dermatol 1986 ;115: 595-9. PubMed

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