Malakoplakia D84.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

malacoplakia

Definition
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Very rare granulomatous disease due to reduced phagocytosis ability of bacteria. Manifestation on numerous internal organs, rarely also on the skin.

Etiopathogenesis
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Acquired defect of macrophages (immunosuppression) concerning the intracellular, lysosomal degradation of phagocytic bacteria. Bacteria cannot be eliminated from the tissues. Escherichia coli, more rarely Staphylococcus aureus or other bacteria can usually be isolated from malacoplakia foci.

Manifestation
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Mainly in immunosuppressed patients, e.g. in immunosuppressive therapy after organ transplantation.

Localization
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Skin changes preferably perianal, genital, in the glutaeal area. Most frequently affecting the urogenital tract, followed by the gastrointestinal tract, lymph nodes, female genital organs, brain, bones, lungs.

Clinical features
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Skin infestation (rare): uncharacteristic, circumscribed, pyodermaic foci, abscesses, ulcerative nodules and aggregation of painful, solid, coarse papules.

Histology
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Granulomatous infiltrates from large histiocytes with foamy, eosinophilic cytoplasm (so-called von Hansemann cells). Sometimes they contain basophilic, PAS-positive target-like inclusions (so-called Michaelis-Gutmann corpuscles). Electron microscopy: detection of intracellular bacteria in macrophages and granulocytes.

Therapy
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  • In case of skin involvement: If possible, generous surgical removal of the skin lesions.
  • Therapy attempt with clofazimine (initial 400 mg/day, slowly decrease to 100 mg/day).
  • Alternatively ciprofloxacin (e.g. Ciprobay) 500 mg/day p.o. if necessary in combination with trimethoprim-sulfamethoxazole(e.g. Cotrimox-Wolff 2 times/day 2 tbl. p.o.).
  • In which form (and if at all) antibiotics improve the course is not clear. A reduction of the immunosuppressive therapy should be considered as far as possible.
  • The use of ascorbic acid has been described.

Literature
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  1. Lowitt MH et al (1996) Cutaneous malacoplakia: A report of two cases and review of the literature. J Am Acad Dermatol 34: 325-332
  2. Mehregan DR et al (2000) Cutaneous malakoplakia: a report of two cases with the use of anti-BCG for the detection for micro-organisms. J Am Acad Dermatol 43: 351-354
  3. Pang LC (2003) Malacoplakia manifesting as a chronic inflammatory mass at the site of a nonhealing surgical wound. Ear Nose Throat J 82: 876-878, 880
  4. Sarkell B et al (1994) Cutaneous malacoplakia. J Am Acad Dermatol 30: 834-836
  5. Schaller J et al (1996) Cutaneous malacoplakia in a patient with psoriasis vulgaris. dermatologist 47: 763-736
  6. Van Furth R et al (1992) Ciprofloxacin for treatment of malakoplacia. Lancet 339: 148-149
  7. Wittenberg PG et al (1998) Cutaneous malacoplakia in a patient with the acquired immunodeficiency syndrome. Arch Dermatol 134: 244-245

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Last updated on: 29.10.2020