April 1987, Vol. 26, No. 3

Cameo

Cutaneous Malakoplakia With Dermatomyositis

MOHAN SINGH, M.D., S. KAUR, M.D., B. K. VAIPAYEE, M.D., AND A. K. BANERIEE, M.D.

from the Departments of Dermatology and Pathology,Postgraduate Institute of Medical Education and Research,Chandigarh, India

A 30-year-old man was hospitalized with painful perianalabscesses and draining sinuses for 5 months and fea-

tures of dermatomyositis of 3 years' duration. Perianal skinrevealed erythematous nodules, abscesses, and dischargingsinuses arranged in a linear fashion and shaped like an In-verted V, the junction of two linear lesions formed by fourfirm, tender nodular lesions on the undersurface of scrotum(Fig. 1).

Blood biochemistry was within normal limits. Urine showed8-10 erythrocytes and 4-5 pus cells/HPF. Escherichia coliwas grown on culture. Cultures from the perianal abscessesgrew Staphylococcus aureus and Proteus vulgaris. LDH (346lU per liter; normal 70-240 lU per liter), aldolase (51.0 sigmaunits; normal, 25 sigma units), and electromyography weresuggestive of dermatomyositis. Skin tests with PPD, fungalrecall antigens (candidin, trichophytin, aspergillin, histoplas-min), and DNCB challenge were unresponsive. T lympho-cytes (71 %). B lymphocytes (12%), T-helper cells (61 %), andT-suppressor cells (8%) were within normal range. Leukocytefunction tests were within normal range. Histopathologic ex-amination revealed a normal epidermis and a dense collec-tion of cells in the underlying dermis. The predominant cellswere large with abundant pale-staining cytoplasm and smallnuclei (Von Hanseman cells). Several macrophages containedtypical intracytoplasmic inclusions (Michaelis-Gutmannbodies) showing positive Von Kossa's reaction for calcium(Figs. 2 and 3). These were PAS-positive and showed positivePearl's reaction for iron.

Comments

Malakoplakia is a rare, chronic granulomatous dis-ease of unknown etiology, which usually affects theurinary tract. It is characterized histopathologically by

Address for correspondence: Mohan Singh, M.D.. Department ofDermatology & Venereology, Postgraduate Institute of Medical Ed-ucation and Research, Chandigarh-160 012 India.

the presence of histiocytes (Von Hansmann cells) con-taining peculiar intracytoplasmic, rounded, laminatedinclusions called Michaelis-Cutmann (MG) bodies.'Cutaneous malakoplakia is very rare. There are only13 case reports of cutaneous malakoplakia in the lit-erature.' These have been seen in six females andseven males aged 5 months to 75 years. A predominantassociation with diseases of the altered immune re-sponsiveness or immunosuppressive therapy has beendocumented.'

The etiology of malakoplakia is uncertain. Associatedurinary tract infection is frequently seen in malako-plakia of urinary tract.' E. coli, S. aureus, and Pseudo-monas aeruginosa have been cultured from the cuta-

Eic. 1. Linear arrangement of discharging abscesses convergingat a red, tender nodule in the scrotal wall.

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No. 3 MALAKOPLAKIA WITH DERMATOMYOSITIS Singh el al. 191

• , . * • • *«

FIG. 2. Microphotograph showing normal epidernus and infiltra-tion of the dermis by pale cells. (H&E; original magnification, X60)

FIG. 3. The predominant cells are pale histocytes with cytoplasmicinclusions or MC bodies. (H&E; original magnification, X160; inset:von Kossa; original magnification, X640)

neous lesions.' Several authors have tried to implicatealtered macrophage response to infection in the cau-sation of disease.'^" Abdou et al.^ reported a patientof extensive malakoplakia whose monocytes showeddecreased bactericidal activity toward E. coli in vitroand low levels of cyclic GMP. Bactericidal activity,chemotaxis, phagocytosis, and NBT reduction werenormai in the present patient. These findings are similarto those of Lewin et al." and McClure et al.,^ who alsocould not demonstrate these defects.

References1. McClure ). Malakoplakia. I Pathol. 1983;140:275-330.2. Schreiber BG, Maderazo EG. Leucocyte function in malako-

plakia. Arch Pathol Lab Med. 1978;102:534-537.3. Abdou NI, NaPombejara C, Sagawa A, et al. Malakoplakia: ev-

idence for monocyte lysosomal abnormality correctable bycholinergic agonist in vitro and in vivo. N Engl) Med. 1977;297:1413-1419.

4. Lewin K), Fair WR, Steigbigel RT, et al. Glinical and laboratorystudies into the pathogenesis of malakoplakia. ) Clin Pathol.1976;29:354~363.

5 McClure ), Cameron CHS, Garrett RG. The ultrastructural fea-tures o( malakoplakia.) Pathoi. 1981;134:13-25.

The Complexity of Modern Medicine

One of the great obstacles to an understanding of modern medicine is Its complexity, its in-credible wealth of seemingly unrelated details. This complexity has led to specialization, whichin turn has further intensified the complexity. There is no better way than the study of medicalhistory to bring some order and coherence into this oppressive mass of details. As the details areordered historically, the fundamental ideas that govern modern medical thinking and action beginto stand out; and the observer is enabled to apply them to the modern picture. The student ofmedicine must grasp the role of anatomy in the growth of medicine since Vesalius, the periodicvictories ofthe chemical approach, the part played by the laboratory in clinical medicine sincethe middle of the last century, and the recurrent idea that the healing force of nature can bereplaced by the curative activities of the physician. Only then will he have a clear understandingofthe essential features and trends of present-day medicine. As the only discipline that presentsmedicine as a whoie, medical history is a valuable antidote to certain mental attitudes growingout of the unavoidable specialization of which medical men rightly complain.—Ackerknecht EH.A Short History of Medicine. Baltimore: Johns Hopkins, 1982:xvi.