Import of an Extinct Disease?

OBSERVATION Pinta in Austria (or Cuba?) Import of an Extinct Disease? Ingrid Woltsche-Kahr, MD; Bruno Schmidt, PhD; Werner Aberer, MD; Elisabeth Aberer, MD Background: Pinta, 1 of the 3 nonvenereal treponema- detection of spirochetes in the trunk lesion indicated early toses, is supposed to be extinct in most areas in South and secondary syphilis, but an extensive case history and the Central America, where it was once endemic. Only scat- clinical appearance fulfilled all criteria for pinta. tered foci may still remain in remote areas in the Brazilian rain forest, and the last case from Cuba was reported in 1975. Conclusion: The acquisition of a distinct clinical en- tity, pinta, in a country where it was formerly endemic Observation: A native Austrian woman, who had lived but now is believed to be extinct raises the question of for 7 years in Cuba and was married to a Cuban native, whether the disease is in fact extinct or whether syphilis developed a singular psoriasiform plaque on her trunk and pinta are so similar that no definite distinction is pos- and several brownish papulosquamous lesions on her sible in certain cases. palms and soles during a visit to her home in Austria. Positive serological findings for active syphilis and the Arch Dermatol. 1999;135:685-688 HE NONVENEREAL trepone- after the appearance of pintids), lesions matoses yaws, endemic marked by vitiligolike depigmentation are syphilis (bejel), and pinta the leading feature. These lesions are not are caused by an organism believed to be infectious. Histopathologi- that is morphologically and cal investigations show moderate acan- Tantigenically identical to the causative agent thosis, spongiosis, sometimes hyperkera- of venereal syphilis, Treponema pallidum. tosis and hypergranulosis, and liquefaction However, they differ significantly from generation of the basal layer in the epi- syphilis in their mode of transmission, epidermis, and a perivascular dermal infil- demiology, and clinical manifestation. trate composed of lymphocytes, plasma Pinta, which is endemic only in the cells, and neutrophils in the upper der- western hemisphere, is the most benign mis. The main reservoir of pinta is young spirochetal disease, showing only skin adults, who have chronic skin lesions. Di- manifestations and occasionally mild sys- rect skin-to-skin contact is the likely temic symptoms (Table).1 The lesions of mechanism of transmission. pinta are characterized by first hyperkera- Several reports of cases of yaws and totic and later dyschromic eruptions. Like bejel imported to Europe as well as the venereal syphilis, pinta chronically re- ever-increasing ease of travel to the world’s lapses. The primary lesion appears as a remotest areas legitimate the demand to small papule or erythematous macule 7 to consider a diagnosis of nonvenereal trepo- 70 days after inoculation and extends nematoses in certain clinical situations. within several weeks to an erythematous Lack of evidence in a recent case that pinta From the Divisions of Allergy infiltrated plaque of up to 12.5 cm in di- is completely eradicated in areas of Cen- and Venereology ameter. Several months to years after ap- tral America where it was once endemic2 (Drs Woltsche-Kahr and pearance of the initial lesion, small scaly seemed to justify this diagnosis in a case W. Aberer) and General papules that enlarge to psoriasiform that fulfilled all historical, clinical, and se- Dermatology (Dr E. Aberer), plaques, “pintids,” may develop. These rological criteria of this disease. Department of Dermatology, University of Graz, highly infectious secondary lesions may be Graz, Austria; and sparse or numerous and, after healing, REPORT OF A CASE Ludwig-Boltzmann Institute, leave areas of gray, brown, or slate blue hy- Hospital of Vienna-Lainz, perpigmentation or depigmentation. In the A 28-year-old white Austrian woman, who Vienna, Austria (Dr Schmidt). late stage of pinta III (months to 10 years was married to a native Cuban and had ARCH DERMATOL / VOL 135, JUNE 1999 685 ©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 Differential Diagnosis of Pinta and Venereal Syphilis* Characteristics Pinta Venereal Syphilis Transmission .95% Nonsexually (extragenital pattern) .95% Sexually; hematogenous; diaplacental (3-9 mo) Clinical manifestations Stage I Extragenital; erythematous infiltrated plaque Genital/extragenital; indolent ulcer; regional lymphadenopathy Stage II Pintids (small, scaly papules that enlarge to psoriasiform Fever, joint pain, lymphadenopathy, exanthema, plaques with peripheral accentuation); no systemic condylomata lata, corona veneris, “plaques symptoms muqueuses,” angina specifica, alopecia areolaris specifica, and luetic leukoderma Stage III Vitiligolike depigmentation; no involvement of other Gummata; involvement of skin, bones, central nervous organs system, and visceral organs (liver, kidney, etc) Histopathology Histological criteria for the differentiation of Histological criteria for the differentiation of treponematoses are unreliable: acanthosis, treponematoses are unreliable: acanthosis, intraepidermal neutrophilic microabscesses, upper intraepidermal neutrophilic microabscesses, upper dermal inflammatory infiltration around blood vessels dermal inflammatory infiltration around blood vessels Serology No serologic test can distinguish between the No serologic test can distinguish between the treponematoses: TPHA, FTA-ABS, VDRL, IgM ELISA, treponematoses: TPHA, FTA-ABS, VDRL, IgM ELISA, immunoblot† immunoblot† Molecular techniques Treponema differ in a single nucleotide? Treponema differ in a single nucleotide? Epidemiology Endemic foci and isolated cases Worldwide Therapy #2-y duration: penicillin G benzathine, 2.4 million U #2-y duration: penicillin G benzathine, 2.4 million U intramuscularly, 1 application; .2-y duration: intramuscularly, 1 application; .2-y duration: penicillin G benzathine, 2.4 million U intramuscularly, penicillin G benzathine, 2.4 million U intramuscularly, 3 times in 1 wk (in case of allergy to penicillin: 3 times in 1 wk (in case of allergy to penicillin: doxycycline, erythromycin) doxycycline, erythromycin) *From Koff and Rosen.1 †TPHA indicates Treponema pallidum hemagglutination; FTA-ABS, fluorescent treponemal antibody absorption test; and ELISA, enzyme-linked immunosorbent assay. Figure 2. Close-up view showing a hyperkeratotic annular plaque. Figure 1. A single nummular plaque in the left thoracolumbar region. denied having had any extramarital relations in the recent past. lived for the past 7 years in an urban area of Cuba, de- Clinical inspection of the patient disclosed a soli- veloped a solitary erythematosquamous plaque on the left tary erythematous, hyperkeratotic annular plaque of 2.5 side of her trunk. This lesion had lasted for almost 1 month cm in diameter in the left thoracolumbar area, resem- when she was examined at our outpatient clinic. She did bling tinea corporis, a psoriatic plaque, granuloma an- not remember any fever, tick bites, changes in oral or geni- nulare, or hyperkeratotic lichen planus (Figure 1 and tal mucous membranes, joint pain, or lymphadenopa- Figure 2). Furthermore, several discrete erythemato- thy. On questioning, she told us that her husband had squamous lesions on the palms and soles (Figure 3), observed a similar lesion in his right subaxillary region each about 1 cm in diameter, were found; the patient had about 4 months before but that it had disappeared spon- not been aware of them. The remaining skin as well as taneously; a routine test of her husband’s serum before oral and genital mucous membranes showed no abnor- minor surgery in Cuba had disclosed a positive serologi- malities; the lymph nodes were not enlarged, and no alo- cal test result for syphilis, and the husband had then been pecia or any other signs of secondary syphilis could be treated with penicillin. Both our patient and her spouse detected. ARCH DERMATOL / VOL 135, JUNE 1999 686 ©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 IgM IgG 1012 3456789 1012 3456789 47 kd Figure 3. Several brownish red papulosquamous lesions, each about 0.5 to 1 cm in diameter, on the palms. 17 kd 15.5 kd Figure 5. Western blots showing nearly identical bands in serum from our patient with pinta (lane 1) and serum from patients with syphilis II (lane 9), syphilis I (lanes 2-6 and 8), and latent syphilis (lane 10). Lane 7 contains serum from a healthy person without syphilis. T pallidum hemagglutination test, 1:5120; fluorescent treponemal antibody absorption test for IgM and IgG; Cap- tia-Syphilis-M IgM enzyme-linked immunosorbent assay (Biotech; Gull Laboratory, Bad Homburg, Ger- many); and VDRL test, 1:32. Western blot analysis with a T pallidum IgM and IgG test kit (Marblot; VIRAMED, Munich, Germany) showed bands of 15.6, 17, 47, 61, and Figure 4. Numerous spirochetes in the epidermis of the hyperkeratotic trunk 83 kd in the IgG blot and 17, 37, 41, and 47 kd in the lesion (immunoperoxidase method with polyclonal anti–Treponema pallidum IgM blot, a pattern typical of Treponema (Figure 5). As antibody, oil immersion, original magnification 31000). controls, serum samples from 8 patients with syphilis and 1 healthy person were investigated. Serum from our pa- Wound serum was harvested from the hyperkera- tient (lane 1) demonstrated bands nearly identical to those totic plaque on the trunk after mechanical erosion of the of serum from patients

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