Report

PRIMARY CUTANEOUS CRYPTOCOCCOSIS IN EGYPT ALY ABDEL-FATTAH, M.D., MOHAMMED S. ABU ZEID, M.D., F.R.C.S., AND ADLY E. GHALY, M.D. From the Departments of Dermatology, Surgery, and Pathology, Ain Shams University Faculty of Medicine, Cairo, Egypt

Case Report In 1974, a 14-year-old girl noticed a small swelling on the lower part of the left side of the chest wall a little below her left breast. Her mother thought it was something like a boil and applied steroid-antibiotic ointment to the lesion. After 2 weeks the lesion was unchanged, so the girl sought medical help. At that time she had a reddish nodule with a dark brown scab in the middle. It was firm and tender and about 1.5 cm in diameter. It looked like an atypical small carbuncle. She was given ampicillin and dicloxacillin capsules together with local glycerine magnesium sulphate dressings. For the next 15 days, the lesion was examined every few days. It was noticed that it progressed in size, the scab was lost and the surface of the lesion was reddish violet and covered with a thin layer of epithelium. One month later she had an oval elevated ulcer about 4 x 3 cm. The edge was whitish, thickened, firm and everted in parts and the margin indurated; it showed some brownish pigmentation. The surface of the lesion was reddish violet; there were no sloughs or discharge but it seemed to be covered by a smooth thin layer of epithelium (Fig. 1). The base was extending into the subcutaneous tissue but it was not adherent to the underlying muscles of the chest wall. The lesion was a bit tender although it was not painful. There were no palpable lymph nodes in the left axilla or the right axilla or in both groins. The skin of the patient had showed mild icthyosis since her babyhood. Abdominal and chest examination revealed no abnormal finding. Skull and chest films were negative.

ABSTRACT: Neither systemic nor primary cutaneous cryptococcosis has ever been reported in Egypt. The case of a 14-year-old girl with a solitary primary cutaneous cryptococcosis lesion is presented. A surgical specimen proved the diagnosis, although the lesion resembled a rapidly growing epithelioma. Follow-up for 4 months did not reveal evidence of systemic involvement. Cryptococcosis is a well known although a relatively uncommon systemic mycosis with world wide distribution. It involves mainly the central nervous system, lungs and other internal organs. The skin may be affected in about 10% of cases, usually as a manifestation of the systemic nature of the disease. Primary isolated cutaneous cryptococcosis is very uncommon. Moreover, primary cutaneous cryptococcosis had never been reported in Egypt. This does not mean that it does not exist, but most probably it is overlooked. We present the case of a 14-yearold girl with cutaneous cryptococcosis proven from the excised lesion.

The picture by that time suggested a malignant lesion of the skin and the dermatologist was consulted. Since diagnosis could not be made clinically, it was decided to excise a specimen for biopsy. The whole lesion with a margin between 2 and 3 cm of the surrounding skin was removed down to the deep fascia.

Address for reprints: Aly Abdel-Fattah, M.D., 35 Mansour St., Bab El-Louk, Cairo, Egypt. 606

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Ttie big gap which resulted could be closed easily by interrupted sutures in 2 layers due to the flexibility of the skin. The postoperative course was uneventful and the wound healed by primary intention. The lesion was a rounded ulcerated infiltrated f i r m subcutaneous disc nearly 4 cm in diameter. The whole epidermis is completely ulcerated over the lesion and more or less normal on either side. The subcutaneous tissue showed heavy granulomatous cellular infiltrate consisting of histiocytes, lymphocytes, plasma cells and fibroblasts with some multinucleated giant cells. There were many areas of necrosis, especially in the superficial part near the ulcer. Inside some large histiocytes and scattered between the inflammatory cells, there were many rounded bodies which stained pale blue in color surrounded by a refractile gelatinous capsule. Some of them showed budding. There was no evidence of neoplastic or any malignant changes. The pathological examination was typical of cryptococcosis.

Discussion Cryptococcosis (synonyms: torulosis, European blastomycosis, Busse-Buschke's disease) is caused by an encapsulated fungus, Cryptococcus neoformans. Although the respiratory tract is the usual portal of entry, C. neoformans is unique among fungi in predilection for invasion of the central nervous system.' Intracranial infection^ generally presents as subacute meningitis with headache, fever, neck stiffness and cranial nerve palsies, or less often simulates a cerebral tumor with the symptoms of raised intracranial tension. The pulmonary infect i o n is often symptomless but extensive lesions may cause breathlessness and cough, sometimes productive of sputum containing cryptococci. In the disseminated form of the disease, cutaneous lesions may precede or follow the signs of involvement of the central nervous system and lungs. The cutaneous and mucous membrane lesions which occur in about 10 and 3% of cases respectively are seldom patho-^' '• ^ They have been classiinto 4 groups: (1) cystic or firm subcutaneous swellings which may ul-

Fig. 1.

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Surface of the lesion.

cerate either spontaneously or after incision; (2) crusted granulomata which range from acneform rashes of papules or pustules through nodules, warts and tumors to large vegetating plaques; (3) dermal plaques or nodules, and (4) ulcers which may develop in any of the previous lesions or may appear in a previously healthy skin. On the other hand, primary cutaneous cryptococcosis is rarely recognized clinically and its diagnosis must depend on alert histological examination and detection of cryptococci from unusual abscesses and granulomata by direct smear, culture methods, and mouse inoculation.•"' When the lesion of the patient discussed was seen in its well developed state, the ulcer resembled very much an epithelioma; it did not even seem to be a granuloma. As it was a solitary lesion, surgical removal was possible. We did not submit scrapings for direct smear or culture as the possibility of cryptococcosis or other fungus infection had not been considered. Serial sections from the biopsy revealed the encapsulated fungus and budding in different fields. The pathological features together with the presence of the yeast were characteristic of cryptococcosis.

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Fig. 2. Heavy granulomatous infiltrate in the tissue (X 96).

Fig. 3. Higher magnification showing many rounded bodies surrounded with refractile gelatinous capsules characteristic of C. neoformans (X 240).

m It has been mentioned that in the systemic form the usual portal of entry is through the respiratory tract but rarely primarily cutaneous inoculation does occur. However, the source of infection in our case is rather obscure. It was shown by Emmons and others'' ^ that the most significant source of infection is exposure to pigeon excreta, but such history was not obtained in this case. Moreover, crytococcosis is not contagious and transmission from man to man or from the many domestic animals

which may harbor it has not been demonstrated.^ Virulent strains of C. neoformans cannot be isolated from skin, mucosa or feces of man with sufficient frequency to support the concept of an endogenous source of infection.'' Cryptococcosis often complicates Hodgkin's disease^ and occasionally sarcoidosis; this suggests that the organism is widespread in the environment, and can easily affect the susceptible patient. Normal human serum inhibits the growth of C. neoformans, a factor which may con-

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tribute to the low incidence of infections in otherwise healthy individuals.' Reduct i o n in serum anticryptococcal activity possibly plays a role in the occurrence of the infection in individuals suffering f r o m debilitating diseases. Also, the rarity of occurrence of primary cutaneous cryptococcosis and its good prognosis can be attributed to the efficiency of the healthy immune defenses in the skin.^ In our patient the only abnormality associated with the disease was her icthyotic skin, which had been present since babyhood; otherwise she was absolutely normal. After 4 months of followup, there is nothing suggestive of reticulotic or lymphoblastic manifestations.

2. Butler, W. T, Ailing, D. W., Spickard, A.,

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References 1.

Carton, C. A., and Mount, L. A., Neurological aspects of cryptococcosis. J. Neurosurg. 8: 143, 1951.

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and Utz, J. P., The diagnostic and prognostic value of clinical and laboratory findings in cryptococcal meningitis. A follow-up study of 40 patients. N. EngI. J. Med. 270:59, 1964. Cawley, E. P., Crekin, R. H., and Curtis, A. C , Torulosis, a review of the cutaneous and adjoining mucous membrane manifestations. J. Invest. Dermatol. 14:327, 1950. Crounse, R. C , and Lerner, A. B., Cryptococcosis: case with unusual skin lesions and favorable response to Amphotericin B. Arch. Dermatol. 77:210, 1958. Rook, A. J., and Woods, B., Cutaneous cryptococcosis. Br. J. Dermatol. 74:43, 1962. Rook, A. J., Wilkinson, D. S., and Ebling, F. J. C , Textbook of Dermatology. Vol. I, 2nd Edition. Oxford, Biackwell Scientific Publications, 1972. Emmons, C. W., Binford, C. H., and Utz, J. P., Medical Mycology. Philadelphia, Lea & Febiger, 1970. Littman, M. L., and Schneierson, S. S.: Cryptococcus neoformans in pigeon excreta in New York City. Am. J. Hyg. 69:49, 1959. Hildick-Smith, C , Blank H., and Sarkany, I., Fungus Diseases and Their Treatment. London, J. & A. Churchill, Ltd., 1964.

In a personal communication from Dr. H. S. Allen, of Seoul, Korea, he states that natives as well as foreigners are often attacked with what is termed in that country "varnish poisoning." It comes on after contact with furniture or other articles recently varnished. Some persons cannot pass a furniture shop where articles are being varnished without being poisoned. His personal experience is thus related: "My first eruption occurred in Nanking. The dermatitis first developed over my left eye, spreading to my forehead and nose; it itched and burned, causing great discomfort and inconvenience. Being ignorant of the effect of the Chinese varnish. I diagnosed the trouble as herpes zoster frontalis. The next attack was in Shanghai, after receiving a new chair from a Chinese cabinetmaker. The skin of the forehead became hot and itched intolerably, and was soon covered by an eruption of minute vesicles on a raised base. The swelling increased until the eyes were nearly closed. It was first diagnosed as a facial erysipelas, but consultation being called, it was pronounced 'varnish poisoning.' "—Pp. 528-529 from Morrow, P. A., "Drug Eruptions in Selected Monographs on Dermatology," New Sydenham Society, London, 1893.

Primary cutaneous cryptococcosis in Egypt.

Neither systemic nor primary cutaneous cryptococcosis has ever been reported in Egypt. The case of a 14-year-old girl with a solitary primary cutaneou...
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