University of Alabama at Birmingham 2004 Case #7 Universidad Peruana Cayetano Heredia
The following patient was seen in the outpatient clinic of tropical disease unit of the Tropical Medicine Insitute in Lima.  We thank Dr. Francisco Bravo, Head of Dermatology at our institute and Gorgas Course Dermatology Consultant for his advice on this case.  (see his review - ?New and Re-emerging Cutaneous Infectious Diseases in Latin America? Dermatol Clin 2003;21:655-68.)
Image for 03/15/04History: 5 year old male with recurrent infection of the scalp, ears, neck, and less often axilla and groin since the age of 5 months presents with a typical recurrence.  Episodes occur 3-4 times per year that respond rapidly to therapy with 2-3 weeks total of oral anti-staphylococcal antibiotics.  The last episode was 3 months ago.  Staphylococcus aureus has been isolated when cultures have been performed.  Normal vaginal birth at term, normal development, breastfed until 2 years of age, has received all standard EPI vaccines.  Frequent episodes of rhinitis in recent months.

Epidemiology: Born in Ica on the coast of Perú of a mestizo father and mullato mother.  Father with pulmonary TB 4 years ago, entire family got INH.  Mother is well.  A sister of the mother and a sister of the grandmother both died of lymphoma.

Physical Examination: Afebrile.  Scaly eczematous, crusted lesions affecting the scalp, neck, and axillae as shown in images A and B.  No adenopathy, hepatomegaly, or splenomegaly.

Laboratory Examination: Routine blood work has always been normal when performed in the past.

 

 

 

 

Diagnosis: HTLV-1 Associated Infective Dermatitis (ID) of Childhood.

Image for 03/15/04 DiscussionDiscussion: Both ELISA and Western Blot for HTLV-1 were positive.

The recurrent skin disease in this child is typical of an increasingly recognized dermatologic manifestation of HTLV-1 infection (Arch Dermatol 1998;134:439-44).  An infective dermatitis (ID), manifest by a pattern of eczema, exudation and crusting around the scalp, nostrils, and ears, and which responds remarkably quickly to antibiotics, was initially described in Jamaica in the 1960s.  A watery nasal discharge without other signs of rhinitis was frequently present.  With the description of HTLV-1 and the availability of serology, the association of ID with HTLV-1 infection in Jamaica was made in the 1990s and has been confirmed elsewhere.

A number of other dermatologic conditions are caused by HTLV-1 infection: notably acute T-cell leukemia/lymphoma with cutanous infiltration (normal blood film in this patient), crusted or Norwegian scabies, and dermatophytosis.  Opportunistic infection with Strongyloides stercoralis is frequent and, as we have presented previously, tropical spastic paraparesis (also called HTLV-1 Associated Myelopathy [HAM]) is an important and often severe neurologic sequela of HTLV-1 infection.  Neither this patient nor his parents had any neurological findings.

Clinically and histologically (chronic lymphocytic psoriaform dermatitis), the skin disease cannot be differentiated from atopic dermatitis or eczema so HTLV-1 seropositivity is required for diagnosis of this entity.  In our series of 92 children presenting initially with compatible skin disease 33/92 (35%) were HTLV-1 positive.  Although long-term follow-up studies are not available, it appears that ID predominantly affects young children and is uncommon in adults with HTLV-1.  Adult patients with TSP/HAM may have skin manifestations such as icthyosis as well as xerosis and erythema that are not clinically symptomatic.  Recently investigators from Brazil were able to isolate HTLV-1 by PCR more often in abnormal compared to normal skin areas from clinically asymptomatic HTLV-1 positive patients (Am J Trop Med Hyg 2003;68:562-5).  Whether HTLV-1 is involved in the pathogenesis of the skin lesions in infected individuals or whether it is there due to the migration of inflammatory cells containing virus is still uncertain.  Whether any of these skin lesions in HTLV-1 positive individuals or the presence of HTLV-1 locally can predict the development of cutaneous T-cell lymphoma is highly speculative at present.

The prevalence of HTLV-1 in South America is generally underappreciated, normally being associated with Japanese and Caribbean populations.  In Perú, the disease is highly endemic (2-3% seropositivity) in Andean areas of the country, in Quechua populations who have had no contact with Japanese immigrants to the country.  Other South American countries with significant rates of HTLV-1 include Brazil, Colombia, and Ecuador.  Transmission appears to be mainly vertical with high associations with breastfeeding and duration of breastfeeding (probable route in our patient).  Transfusion, sexual transmission, and IV drug abuse are much less important.

Our patient responded rapidly to oral dicloxacillin and a follow-up image taken after 7 days of therapy is shown [see image C].  Image D shows a previous episode of resolving ID in the same child in 2001, demonstrating the characteristic distribution of scaling lesions with underlying erythema.  All children with a recurrent infective dermatitis who are resident in areas where HTLV-1 exists should be tested for this retrovirus.