British Journal of Dermatology (1979) lOi, 71.
Mycobacterium avium infection of the
skin resembling lepromatous leprosy GARY W.COLE AND JOHN GEBHARD Dermatology Section, Long Beach Veterans Medical Center, Hospital, Long Beach, California 90822, U.S.A. Accepted for publication 19 October 1978
SUMMARY
A patient on systemic steroid therapy developed a cutaneous papule which histologically resembled lepromatous leprosy. Cultures of this lesion grew Mycobacterium avium. Since there was no evidence of disseminated infection the lesion was excised and the patient continues to do well.
Human infections caused by Mycobacterium avium complex organisms (M. battey, M. intercellulare, Runyon group III) are generally manifest as pulmonary disease, osteomyelitis or lymphadenitis (Harris & McClement, 1977). Only rarely has skin involvement been reported and this is ahiiost always associated with disseminated disease (Koenig, Colhns & Heyssel, 1966; Lincoln & Gilbert, 1972). It is distinctly unusual to encounter a patient with a purely cutaneous M. avium complex infection (Schmidt et al., 1972). We recently diagnosed and treated such a patient. CASE REPORT
A 65-year-old white male appeared at the dermatology clinics of the Long Beach Veterans Administration Hospital in April 1977 complaining of a 3-4 month history of an asymptomatic growth on his right forearm. He was otherwise in good health except for chronic asthmatic bronchitis of 20 years duration which has required prcdnisone, or its equivalent, fror control (20 mg/day). Physical examination revealed a slightly Cushingoid elderly gentleman with a i cm^ yellow-orange, scaling papule on his right forearm. There was no axillary adenopathy and the rest of the physical evaluation was unremarkable. Histological examination of a punch biopsy showed a thinned epidermis with loss of the rete ridges. The papillary and reticular dermis were massively infiltrated with foamy histiocytes (Fig. i). Fite's stain showed enormous numbers of acid-fast bacilli singly and in clumps (Fig. 2). The pathological picture was interpreted as lepromatous leprosy and the patient was begun on dapsone (25 mg/day). The patient was re-evaluated in December 1977. Normal laboratory findings at that time included a complete blood count, sedimentation rate, urinalysis, antinuclear factor, rheumatoid factor, VDRL, serum creatine, calcium, protein, albumin, bilirubin, lactic dehydrogenase, alkaline phosphatase, ooo7-0963/79/o70o-cx)7i$02.oo
r' 1979 British Association of Dermatologists 71
"•' ^A i*»"% '••• Ti-^^ FIGURE I. High power view demonstrarcs lepra cell-like character of the histiocytes (H & E, x 200).
FIGURE 2. Acid-fast bacilli in 'globe-like' clumps in histiocytes (Fite, x 200).
Mycobacterium avium infection resembling leprosy
73
transaminase, and protein electrophoresis. Intradermal injections of PPD, PPD-B, trichophytin, Candida, and tetanus toxoid showed no induration at 38 h. Buffy coat, normal skin and ear scrapings were reported as showing no acid-fast organisms. A biopsy of the lesion obtained for mycobacterial culture grew a non-chromogenic add-fast organism. Chest films were unchanged since 1975 and an X-ray of the right forearm showed no osteomyelitis. Cultures of sputum and urine repeated in triplicate grew no acid-fast organisms. Dapsone was discontinued and the lesion was excised. The patient is doing well at the time of this writing. RESULTS
The acid-fast organism cultured from onr patient's skin proved to be an atypical nonchromogenic mycobacterium of the M. avium complex. Some of its biochemical parameters are listed in Table i. Agglutination testing revealed this organism to be M. avium complex serotype i. TABLE I. Some biochemical parameters of the Mycobacterium avium complex Test
Results
Urease Arylsulfatase Nitrate reduction Niacin Tween 80 hydrolysis Catalase Pigment Growth 37-C 25=C
Negative Negative Negative Negative Negative Negative Negative
45X Slow ( >7 days) Drug resistance
Positive Negative Negative Positive Positive
DISCUSSION
Negative cultures of sputum and urine, negative X-ray examination of the chest and right arm, and negative laboratory studies support the contention that this infection was confined only to our patient's skin. Few reports of purely cutaneous M. avium complex infection have appeared in the medical literature (Schmidt et al.-, 1972; Privat ei a!., 1968). The lesions in the two patients described were composed of multiple ulcers. Pathological findings consisted of acute granulomatous inflammation^ acid-fast bacilli were demonstrable in one case but not in the other. In contrast, our patient had a single granulomatous papule which his to logically resembled lepromatous leprosy. Feldman & Herschfield (1974) reported twenty-nine patients who developed cutaneous involvement due to an 'unculturable mycobacterial species.' Many of their cases resembled ours both clinically and histologically and two of them were erroneously diagnosed as leprosy. Our patient was on long-term systemic steroid therapy and had negative intradermal skin tests. This lack of cutaneous delayed hypersensitivity probably reflects an overall defect in cell mediated immunity induced by systemic steroid therapy. Likewise, there appears to be a failure to mount an
74
G.W.Coh andJ.Gebhard
immune response to M. leprae in patients who develop lepromatous leprosy (Godal, Myklestad & Samuel, 1971). Perhaps, the inability to deal effectively on a cellular level with M. avium complex mycobacteria could account for the leprous appearance of our patient's biopsy. Af. avium complex mycobacteria are resistant to the standard anti-mycobacterial chemotherapeutic agents. Surgical intervention is a common mode of therapy in the treatment of pulmonary disease or lyphadenitis due to this organism (Harris & McClement, 1977). Our patient's single lesion was excised and no further treatment is contemplated at this time. ACKNOWLEDGMENTS
Dr Irving Krasnow cultured and identified the organism. REFERENCES FELDMAN, R.A. & HERSHFIELD, E. (1974) Mycobacterial skin infection by an unidentified species. Anrials of Internal Medicine, 80, 445, GODAL, T., MYKLESTAD, B. & SAMUEL, D.R. C197O Characterization of ihe cellular immune defect in lepromaious leprosy. Clinical and Experimeriral Immunology, 9, 821. HARRIS, H . W , & MCCLEMHNT, J . H . (1977) Nontuberculous mycobacterioses. In: Infectious Diseases (Ed, by P. Hocprlch), p. 350, Harper & Row, Maryland, KoENiG, M.G., COLLINS, R . D . & HEVSSEL, R . M . (1966) Disseminated mycobacteriosis caused by battery type mycobacteria. Annals 0/ Internal Medicine, 64, 145. LINCOLN, E . M . & GILBERT, L.A. (1972) Disease in children due to Mycobacceria other than Mycobacterium tuberculosis. American Review of Respiratory Diseases, 105, 6S3. PRIVAT, Y., SARRAT, H . , FAYH, I. & BELLOSSI, A. (1968) Ulcerations cutanees multiples dues tres probablemeni a Mycobacterium aviiort. Societe de dermatogic el de syphiligraphie, 75, 304, S C H M I D T , J . D . , YAEGER, H . , S M I T H , H . B . & R A L E I G H , J . W . (1972) C u t a n e o u s infection d u e t o a R u n y o n g r o u p I I I
atypical mycobacterium. American Review of Respiratory Diseases, 106, 469.
Mycobacterium avium infection of the
skin resembling lepromatous leprosy GARY W.COLE AND JOHN GEBHARD Dermatology Section, Long Beach Veterans Medical Center, Hospital, Long Beach, California 90822, U.S.A. Accepted for publication 19 October 1978
SUMMARY
A patient on systemic steroid therapy developed a cutaneous papule which histologically resembled lepromatous leprosy. Cultures of this lesion grew Mycobacterium avium. Since there was no evidence of disseminated infection the lesion was excised and the patient continues to do well.
Human infections caused by Mycobacterium avium complex organisms (M. battey, M. intercellulare, Runyon group III) are generally manifest as pulmonary disease, osteomyelitis or lymphadenitis (Harris & McClement, 1977). Only rarely has skin involvement been reported and this is ahiiost always associated with disseminated disease (Koenig, Colhns & Heyssel, 1966; Lincoln & Gilbert, 1972). It is distinctly unusual to encounter a patient with a purely cutaneous M. avium complex infection (Schmidt et al., 1972). We recently diagnosed and treated such a patient. CASE REPORT
A 65-year-old white male appeared at the dermatology clinics of the Long Beach Veterans Administration Hospital in April 1977 complaining of a 3-4 month history of an asymptomatic growth on his right forearm. He was otherwise in good health except for chronic asthmatic bronchitis of 20 years duration which has required prcdnisone, or its equivalent, fror control (20 mg/day). Physical examination revealed a slightly Cushingoid elderly gentleman with a i cm^ yellow-orange, scaling papule on his right forearm. There was no axillary adenopathy and the rest of the physical evaluation was unremarkable. Histological examination of a punch biopsy showed a thinned epidermis with loss of the rete ridges. The papillary and reticular dermis were massively infiltrated with foamy histiocytes (Fig. i). Fite's stain showed enormous numbers of acid-fast bacilli singly and in clumps (Fig. 2). The pathological picture was interpreted as lepromatous leprosy and the patient was begun on dapsone (25 mg/day). The patient was re-evaluated in December 1977. Normal laboratory findings at that time included a complete blood count, sedimentation rate, urinalysis, antinuclear factor, rheumatoid factor, VDRL, serum creatine, calcium, protein, albumin, bilirubin, lactic dehydrogenase, alkaline phosphatase, ooo7-0963/79/o70o-cx)7i$02.oo
r' 1979 British Association of Dermatologists 71
"•' ^A i*»"% '••• Ti-^^ FIGURE I. High power view demonstrarcs lepra cell-like character of the histiocytes (H & E, x 200).
FIGURE 2. Acid-fast bacilli in 'globe-like' clumps in histiocytes (Fite, x 200).
Mycobacterium avium infection resembling leprosy
73
transaminase, and protein electrophoresis. Intradermal injections of PPD, PPD-B, trichophytin, Candida, and tetanus toxoid showed no induration at 38 h. Buffy coat, normal skin and ear scrapings were reported as showing no acid-fast organisms. A biopsy of the lesion obtained for mycobacterial culture grew a non-chromogenic add-fast organism. Chest films were unchanged since 1975 and an X-ray of the right forearm showed no osteomyelitis. Cultures of sputum and urine repeated in triplicate grew no acid-fast organisms. Dapsone was discontinued and the lesion was excised. The patient is doing well at the time of this writing. RESULTS
The acid-fast organism cultured from onr patient's skin proved to be an atypical nonchromogenic mycobacterium of the M. avium complex. Some of its biochemical parameters are listed in Table i. Agglutination testing revealed this organism to be M. avium complex serotype i. TABLE I. Some biochemical parameters of the Mycobacterium avium complex Test
Results
Urease Arylsulfatase Nitrate reduction Niacin Tween 80 hydrolysis Catalase Pigment Growth 37-C 25=C
Negative Negative Negative Negative Negative Negative Negative
45X Slow ( >7 days) Drug resistance
Positive Negative Negative Positive Positive
DISCUSSION
Negative cultures of sputum and urine, negative X-ray examination of the chest and right arm, and negative laboratory studies support the contention that this infection was confined only to our patient's skin. Few reports of purely cutaneous M. avium complex infection have appeared in the medical literature (Schmidt et al.-, 1972; Privat ei a!., 1968). The lesions in the two patients described were composed of multiple ulcers. Pathological findings consisted of acute granulomatous inflammation^ acid-fast bacilli were demonstrable in one case but not in the other. In contrast, our patient had a single granulomatous papule which his to logically resembled lepromatous leprosy. Feldman & Herschfield (1974) reported twenty-nine patients who developed cutaneous involvement due to an 'unculturable mycobacterial species.' Many of their cases resembled ours both clinically and histologically and two of them were erroneously diagnosed as leprosy. Our patient was on long-term systemic steroid therapy and had negative intradermal skin tests. This lack of cutaneous delayed hypersensitivity probably reflects an overall defect in cell mediated immunity induced by systemic steroid therapy. Likewise, there appears to be a failure to mount an
74
G.W.Coh andJ.Gebhard
immune response to M. leprae in patients who develop lepromatous leprosy (Godal, Myklestad & Samuel, 1971). Perhaps, the inability to deal effectively on a cellular level with M. avium complex mycobacteria could account for the leprous appearance of our patient's biopsy. Af. avium complex mycobacteria are resistant to the standard anti-mycobacterial chemotherapeutic agents. Surgical intervention is a common mode of therapy in the treatment of pulmonary disease or lyphadenitis due to this organism (Harris & McClement, 1977). Our patient's single lesion was excised and no further treatment is contemplated at this time. ACKNOWLEDGMENTS
Dr Irving Krasnow cultured and identified the organism. REFERENCES FELDMAN, R.A. & HERSHFIELD, E. (1974) Mycobacterial skin infection by an unidentified species. Anrials of Internal Medicine, 80, 445, GODAL, T., MYKLESTAD, B. & SAMUEL, D.R. C197O Characterization of ihe cellular immune defect in lepromaious leprosy. Clinical and Experimeriral Immunology, 9, 821. HARRIS, H . W , & MCCLEMHNT, J . H . (1977) Nontuberculous mycobacterioses. In: Infectious Diseases (Ed, by P. Hocprlch), p. 350, Harper & Row, Maryland, KoENiG, M.G., COLLINS, R . D . & HEVSSEL, R . M . (1966) Disseminated mycobacteriosis caused by battery type mycobacteria. Annals 0/ Internal Medicine, 64, 145. LINCOLN, E . M . & GILBERT, L.A. (1972) Disease in children due to Mycobacceria other than Mycobacterium tuberculosis. American Review of Respiratory Diseases, 105, 6S3. PRIVAT, Y., SARRAT, H . , FAYH, I. & BELLOSSI, A. (1968) Ulcerations cutanees multiples dues tres probablemeni a Mycobacterium aviiort. Societe de dermatogic el de syphiligraphie, 75, 304, S C H M I D T , J . D . , YAEGER, H . , S M I T H , H . B . & R A L E I G H , J . W . (1972) C u t a n e o u s infection d u e t o a R u n y o n g r o u p I I I
atypical mycobacterium. American Review of Respiratory Diseases, 106, 469.
