Malignant Atrophic Papulosis Clinical Presentation

Updated: Apr 10, 2019
  • Author: L Campbell Levy, MD; Chief Editor: BS Anand, MD  more...
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Presentation

History

Most patients have cutaneous involvement, and it is these manifestations that prompt patients to seek clinical evaluation. Patients usually describe multiple small papular skin lesions. Typically, the lesions involve primarily the trunk and limbs; they are rarely painful, tender, or pruritic. The lesions appear in crops with a new group of lesions developing intermittently. Palms, soles, face, scalp, and genitalia tend to be spared, but exceptions have been noted. Specifically, multiple cases of painful genital ulceration have been reported.

Gastrointestinal manifestations most often appear several weeks, months, or even years after a cutaneous eruption, although there are infrequent reports of gastrointestinal symptoms preceding the skin lesions. The gastrointestinal manifestations are most often nonspecific and include abdominal pain, abdominal distention, nausea, vomiting, diarrhea, or constipation. Patients with extensive involvement of the gastrointestinal tract also may experience weakness, fatigue, weight loss, or symptoms of malabsorption. In late stages of malignant atrophic papulosis, gastrointestinal hemorrhage, bowel infarction, fistulae, and perforation may be observed.

Neurologic involvement is also common. As with the gastrointestinal symptoms, neurologic symptoms are usually nonspecific. Involvement of both central and peripheral nervous systems can occur and can cause paresthesias of the face and extremities, headaches, dizziness, seizures, hemiplegia, aphasia, paraplegia, and gaze palsy.

Symptoms from involvement of other organ systems are rare. Chest pain and dyspnea may occur with involvement of the lungs, pleura, pericardium, and myocardium. Involvement of the eyes can result in diplopia, blurred vision, and visual field defects.

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Physical Examination

The recognition of the skin lesions is critical to an accurate diagnosis. The early lesions are pinkish papules that appear in bouts, are about 2-5 mm in size, and occur on the trunk and extremities while usually sparing the face, palms, soles, and scalp. Within a few days, these papules become umbilicated, with depressed centers. At presentation, most of the papules have reached the atrophic stage and appear as porcelain-white lesions covered with a fine scale and surrounded by a 1- to 2-mm erythematous border. Individual lesions usually remain stable, without a tendency to spread or coalesce with neighboring lesions. However, a spectrum of presentations has been posited, including the evolution of red, painful, subcutaneous red nodules to the typical atrophic papule.

Lesions comparable to those on the skin occasionally may be observed on the conjunctiva or on the genital and buccal mucosa. Lesions have also been observed on the vocal cords.

Patients with widespread systemic disease may exhibit signs of neurologic involvement such as hemiparesis, cranial nerve abnormalities, and paresthesias. Ocular abnormalities (eg, ptosis, optic neuritis, subcapsular cataracts) may occur; papilledema also may be found.

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