Acrokeratosis paraneoplastica, or Bazex syndrome, is a unique cutaneous eruption associated with internal malignant conditions, most commonly squamous cell carcinoma of the aerodigestive tract. While classic stages and sites of involvement are most often reported, atypical presentations must not be overlooked.
A man in his 60s presented with a 9-month history of slightly itchy bumps on the chest and back and 3 weeks of nail tenderness. When specifically questioned, the patient reported noticing a penile lesion 3 months earlier. Physical examination revealed multiple hyperkeratotic, erythematous and hyperpigmented papules and plaques on the chest, back, and arms (Figure 1). Hyperkeratotic patches with slight hyperpigmentation were scattered on the bilateral palms with no involvement of the soles. All fingernails had onychomadesis without dystrophy, while the toenails were normal. An erythematous scaly plaque with satellite papules was present on the glans penis (Figure 2). Face, scalp, and oral mucosa were uninvolved. A 1-cm tender, firm cervical lymph node was palpated, and on further questioning the patient reported odynophagia and dysphagia.
Erythematous, hyperkeratotic papules and plaques on the back.
Erythematous scaly plaque on the glans penis with satellite papules.
Punch biopsy findings from a lesion on the back were nonspecific, showing perivascular lymphohistiocytic infiltrate with psoriasiform hyperplasia and testing negative with syphilis, acid-fast bacilli, and Grocott methenamine silver stains. Topical clobetasol, 0.05%, ointment provided mild relief of itch but did not improve the cutaneous lesions. The patient was referred to otolaryngology with concern for acrokeratosis paraneoplastica. A computed tomography scan confirmed the presence of a supraglottic mass, and laryngoscopy with biopsy revealed basaloid squamous cell carcinoma. A positron emission tomography scan demonstrated diffuse metastases to the bones and viscera. The patient was subsequently treated with palliative chemotherapy and irradiation with no improvement of the cutaneous lesions.
Acrokeratosis paraneoplastica, or Bazex syndrome, was first described in 1965 as a characteristic papulosquamous eruption associated with internal malignant conditions.1 Bazex syndrome classically follows 3 stages.2 The first stage involves erythematous, hyperkeratotic lesions on the nose, helices, and digits with nail dystrophy. The second stage demonstrates similar lesions on the face as well as hyperkeratosis of the palmar and plantar surfaces. Finally, the third stage includes erythematous hyperkeratotic lesions on the trunk and extensor surfaces. The lesions tend to be asymptomatic and are refractory to treatment, with improvement seen in most cases only with resolution of the tumor.
Bazex syndrome occurs in the setting of underlying malignant conditions, most frequently squamous cell carcinomas of the head, neck, or esophagus, although neoplasms of bladder, prostate, and breast and liposarcoma have been described.3,4 Skin lesions tend to precede the diagnosis of the tumor by 1 year.3 The pathogenesis of Bazex syndrome is unknown, but proposed mechanisms include cross reactivity of tumor antigens with the skin, excessive growth factor production by the tumor, and zinc deficiency associated with the neoplasm.3,5
The present patient followed an atypical course for Bazex syndrome. Initial presentation of the lesions on the chest and back is unusual, as is complete lack of involvement of the nose, ears, and face. Onychomadesis in the absence of onychodystrophy is not typical, and reports of genital involvement are exceedingly rare.3,6 Since this condition preferentially affects acral areas (thus the name acrokeratosis), involvement of the penis would not be surprising. The low incidence of genital involvement may be owing in part to reluctance of patients such as ours to report genital disease. Health care professionals should be aware of potential varying presentations of this condition because the earlier the underlying neoplasm is diagnosed, the more likely it will be resectable. Appropriate referral for evaluation of the aerodigestive tract should be expedited, and if necessary, exploration for more unusual underlying tumors should be initiated.
Corresponding Author: Michael S. Graves, MD, Section of Dermatology, Medical College of Georgia at Georgia Regents University, 1004 Chafee Ave, FH-100, Augusta, GA 30912 (MikeSGraves@gmail.com).
Published Online: September 24, 2014. doi:10.1001/jamadermatol.2014.2110.
Conflict of Interest Disclosures: None reported.
Graves MS, Hardin ME, Davis LS. A Case of Bazex Syndrome With Genital Involvement. JAMA Dermatol. 2014;150(12):1368-1370. doi:10.1001/jamadermatol.2014.2110