12-vuotiaalla tytöllä esiintyi borrelioosi käden, ranteiden ja kantapään alueen iholla (adrodermatitis chronica atrophicans). Iho oli ylipigmentoitunuttta ja atrofioitunutta. Ihosta löytyi joitakin tulehdusssoluja, lievää fibroosia ja borreliabakteereita.
Spirochetes in atrophic skin lesions accompanied by minimal host response in a child with Lyme disease.
Journal of The American Academy of Dermatology Volume 25-Number 2,
Part 2 August 1991
Stephen E. Gellis, MD, Miguel J. Stadaecher, MD,PhD, and ALLEN C.
"Acrodermatitis chronica atrophicans, which has rarely been observed in the United States, is a late skin manifestation of Lyme borreliosis. A 12-year-old girl who spent summers on Cape Cod presented with a 2-year history of hypepigmentation and atrophy of the skin on the hands, wrists, and ankles. The skin biopsy specimen of an affected area showed mild dermal fibrosis, a few inflammatory cells, and spirochetes orphologically compatible with Borrelia burgdorferi. An IgG antibody response to B. burgdorferi could be elicited by immunoblotting, but not by enzyme-linked immunosorbent assay. We conclude that this patient had chronic Lyme borreliosis manifested only by indolent infection of the skin."
Acrodermatitis chronica atrophicans, a late skin manifestation of Lyme borreliosis, is caused by the tick-borne spirochete, Borrelia burgdorferi.
The disorder usually begins insidiously with an ill-defined area of erythema, edema and induration, most commonly at an acral site. Gradually, the erythema fades and is replaced by hyperpigmented atrophic skin. The final picture evolves over months to years, producing a wrinkled area of skin resembling "cigarette paper". Although some patients have features of Lyme borreliosis before the onset of acrodermatitis chronica atrophicans, others do not....We report unusual atrophic skin lesions on the hands, wrists, and ankles in a child from an area endemic for Lyme disease....the infection was accompanied by only a minimal humoral immune response to the organism.
A 12-year old girl who spent the summers on Cape Cod had a 2-year history of hyperpigmentation on the hands and ankles. She had no other history of tick bite, erythema migrans, or other signs of Lyme disease. She had not travelled outside the United States. Physical examination revealed that the skin was atrophic, without induration or inflammation, on both hands and wrists and on small areas along both inner malleoli. These lesions appeared to have a blue-gray hyperpigmentation because of the prominet underlying VENOUS pattern.
Two skin biopsy specimens were obtained from adjacent affected and unaffected areas at the border of a lesion near the wrist. In the affected area, there were mild dermal fibrosis and a few perivascular, mononuclear inflammatory cells, but no plasma cells. the unaffected area was normal. However, both specimens, when stained with the Warthin-Starry silver stain, showed spirochetes morphologically compatible with B. burgdorferi........
The patient was treated with intravenous penicillin, 12 MILLION U/day in six divided doses for 2 weeks, followed by a two-week course of tetracycline, 500 mg four times a day. No change was noted in the skin lesions. At the end of treatment, another skin biopsy specimen was obtained from an area adjacent to the previous lesional biopsy site. Spirochetes were not seen in this specimen.
Acrodermatitis chronica atrophicans was described in Germany in 1902. Years later, it was recognized that the skin disease may be accompanied by systemic manifestations and may sometimes be preceded by erythema migrans or Ixodes rincus tick bites. The disorder, as we know it today, was characterized in the 1980s by Asbrink and Hovmark in Sweden. They cultured B. burgdorferi from the lesions of acrodermatitis, in one instance, as much as ten years after disease onset, and their patients had high antibody titers to the spirochete. In addition to chronic skin involvement, underlying structures were frequently affected, and systemic involvement of the nervous system, joints and/or bones was common. Histologically the lesions showed telangiectasia and a patchy interstitial symphoplasmacellular infiltrate in the dermis. The diagnosis was usually based on the clinical picture and elevated antibody titers to B. burgdorferi. Most patients responded to PROLONGED courses of oral or intravenous antibiotic therapy. Although the illness has been described primarily in Europe, several cases have now been recognized in the United States. Chronic infection of the skin with B. burgdorferi may have a greater range of presentations because spirochetes have been seen in the skin biopsy specimens, and elevated antibody titers to B. burgdorferi have been found in a few patients with localized scleroderma, lichen sclerosus et atrophicus, or progressive facial hemiatrophy (Parry-Romberg syndrome).
The presentation in our patient was unusual for Lyme disease, acrodermatitis, or for any other illness: she was a child; she had no preceding signs of Lyme disease; her sites of skin atrophy were ILATERAL and SYMMETRIC; she had no history of inflammation at these sites; and her antibody response to B. burgdorferi, determined by standard ELISA, was negative. However, spirochetes morphologically compatible with B.
burgdorferi were seen in lesions, and immunoblotting showed that she had an IgG antibody response primarily to the outer-surface proteins of the
spirochete. Although an immune response ot outer-surface proteins of the spirochete is often found later in the illness, this patient's pattern of reactivity was unusual because the responses to other immunodominant antigens, the 41, 58, and 66 kD polypeptides, were minimal. It is puzzling that this antibody response was not detectable by standard ELISA. However, in patients with minimal antibody responses, immunoblotting is more sensitive and specific than ELISA for detecting such a response. We do not know why this patient had only a limmited immune response to B. burgdorferi, but we assume that either strain variation of the spirochete or genetically determined factors in the host immune response played a role.
Although her skin lesion did not change after treatment, presumably because of the degree of atrophy, spirochetes were not seen in the followup skin biopsy specimen.
We believe this child had acrodermatitis accompanied by only a minimal humoral immune response to the spirochete. We report her case to encourage recognition, appropriate evaluation, and treatment of this unusual presentation of Lyme disease.