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Chronic slowly enlarging skin lesion in 6-year-old female
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A 6-year-old female is referred to evaluate a chronic, slowly enlarging skin lesion on her right lower leg. The lesion is not associated with any symptoms or other complaints, such as fever, nausea, vomiting, cough, pain or other skin lesions. It was first noted as a light-colored spot when she was adopted from an orphanage in India at 4 years of age.
Figure 1. Yellowish Tan Plaque on the patient's right lower extremity
While her past medical history is mostly unknown, she is thought to be healthy. She apparently was in an orphanage from birth until her adoption. Her review of systems is positive only for the skin finding of this spot. Her immunizations are up-to-date, and fairly well-documented. Her tuberculin skin test is confirmed negative, and she has a bacillus Calmette-Guérin vaccination scar on her right arm.
Examination reveals a very bright, happy and healthy appearing female, who is alert, with normal vital signs, with the only positive finding being the yellowish-tan plaque on her right proximal lateral lower leg (Figure 1), measuring 2 cm and round. There are two smaller and darker spots at the edges of the lesion opposite each other where skin biopsies were previously taken. The lesion itself had some minor altered sensation, in that the patient could feel an object scraping across the area, but the skin felt “thicker” — the sensation of the surrounding skin was unaffected.
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Case Discussion
The biopsies confirmed the diagnosis to be Mycobacterium leprae; (B) Leprosy, or more appropriately, Hansen disease. This is a very complex topic, beyond the scope of a 15-minute case report, but essentially, the disease results from infection with M. leprae, and, depending on the extent of the infection and the immune response, the patient is classified in one of five categories.
James H. Brien
The diagnosis should be based on a skin biopsy, which should be sent to the National Hansen Disease Programs (NHDP) in Carville, Louisiana (800-642-2477; www.hrsa.gov/hansens). The stains, cultures and PCR testing will be done there. They are also a good source for advice and provide medication assistance at no charge to the patient. Treatment guidelines are based on the number of lesions: multibacillary (≥ 6 patches) or paucibacillary (1-5 patches). Basically, the medications Aczone (dapsone, Allergan), rifampin, Lamprene (clofazimine, Novartis) and clarithromycin are used in various combinations for 12 to 24 months, depending on the patient’s classification.
Figure 2. Hansen Disease after 12 months of therapy
Figure 3. Nummular Eczema
Figure 4. Tinea Corporis
Source: Brien JH
Physicians should refer to the AAP Red Book and the NHDP for specific recommendations.
One must remember to test the patient for glucose-6-phosphate dehydrogenase (G6PD) deficiency before using dapsone, as it may result in severe hemolytic anemia in those affected. Our patient was G6PD deficient, and was treated with rifampin plus clarithromycin for 12 months with good results (Figure 2). A clinical clue is to consider Hansen disease in a patient if a skin lesion is numb or has decreased sensation, especially if they have any connection with southern or southwest Asia. Hansen disease also is frequently found in Brazil and several African countries, as well as others. A useful website for global information is leprosymission.org.uk.
The cause of leprosy was found by the Norwegian physician, Gerhard Hansen, MD, in 1873. In 1931, a patient, Stanley Stein, at the inpatient facility in Carville, Louisiana, began a campaign to rename the disease “Hansen disease” to help lessen the stigmata of the diagnosis of leprosy. The National Hansen Disease Museum is now located at the facility and is a great resource for those interested in the history of this historic disease.
Nummular eczema is an eczematous lesion that is round, or “coin shaped” (Greek for nummus) (Figure 3). These lesions are treated the same as generalized eczema, with topical anti-inflammatory creams (steroid) and moisturizers.
Tinea corporis is a skin infection with one of the dermatophytes (from dermatos, Greek for skin plus phutón, Greek for plant). These are some of the anamorphic (asexual) fungi, mostly from Microsporum or Trichophyton species. Infection usually begins as an erythematous papule that grows into a ring with raised edges, leaving behind a scaling center (Figure 4). It is usually acquired from a household or other close contact, or a pet dog or cat. Patients usually respond to topical treatment with miconazole or clotrimazole, but it may take several weeks; if unresponsive, physicians may try oral griseofulvin, or refer to a dermatologist or infectious disease consultant.
Granuloma annulare is an uncommon skin condition of unknown etiology, characterized by circular lesions, with erythematous papular lesions about the periphery. It is often associated with underlying autoimmune disorders, such as lupus, rheumatoid arthritis and diabetes thyroiditis. This must be uncommon, as I have no pictures of my own to show, but there are an abundant number of good pictures on the Internet. It could be that those who take care of the patients with these underlying conditions are making the diagnosis or referring to dermatologists. Granuloma annulare also can be seen in otherwise healthy people; it is self-limiting, and requires no treatment, and usually does not last longer than a few months to a couple of years.
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James H. Brien, DO, is with the department of infectious diseases at McLane Children’s Hospital, Baylor Scott & White Health, Texas A&M College of Medicine in Temple, Texas. He also is a member of the Infectious Diseases in Children Editorial Board. Brien can be reached at: jhbrien@aol.com.
Disclosure: Brien reports no relevant financial disclosures.