Dermatophytic disease with deficit in CARD9: A new case with a brain impairment.

INTRODUCTION: Dermatophytic disease individualized by Hadida and Schousboe in 1959 is a rare form of chronic dermatophyte infection; characterized by subcutaneous and visceral invasion and a therapeutic failure. We report a case of dermatophytic disease with brain abscess in an Algerian patient. OBSERVATIONS: The patient was 47-year-old, she was born parents first cousins. She had since the age of 10 years scaly scalp lesions that became secondarily papular and nodular. At the age of 17 years, the patient had a generalized skin involvement with multiple nodular lesions, diffuse disease of the nails (plurionyxis), poly lymphadenopathies affecting the cervical, axillary and inguinal areas. Mycological tests identified Trichophyton rubrum. Histopathological examinations showed caseiform necrosis with epithelioid and giant cells. PAS staining showed hyphae in necrosis. The rest of the blood and urine tests were normal. Sequencing CARD9 allowed to highlight a homozygous mutation Q289X. The evolution was marked by an appearance at the age of 47 of a right cerebral abscess fronto-temporo-parietal, cortico-subcortical detected by MRI. The patient was given itraconazole 200mg/day with good evolution after three months of decline. DISCUSSION: Dermatophytic disease is very rare. Fifty cases have been published, mostly from North Africa, a strong endogamy region. Signs and symptoms almost always start with a scalp ringworm with almost constant occurrence of complications: in our patient a cerebral localization with a good evolution with itraconazole. Immunologically it is characterized by an autosomal recessive deficiency CARD9. This observation is notable for the onset of the disease at an early age, the dermatophyte T. rubrum and the favourable outcome of the brain lesion with itraconazole. CONCLUSION: Dermatophytic disease is rare, often severe. Our case is original because of the occurrence of a cerebral localization with good response to itraconazole and its mechanism of action deserves to be elucidated.

[Anthrax: an unusual escharotic lesion]. / Charbon: une lésion escarrotique inhabituelle.

BACKGROUND: Anthrax is basically an animal disease rarely found in humans. We report a case occurring in a young boy who had minimal signs and a suggestive skin lesion. CASE REPORT: A 16-year-old boy living in the steppe pastures in western Algeria was hospitalized for unexplained long-duration fever. Physical examination and laboratory tests did not suggest any infectious disease among those common in our area: typhoid fever, brucellosis, meningitis. Anthrax was suggested by presence of an escarrotic black plaque measuring 2 to 3 cm localized on the left leg. The lesion was very adherent and surrounded by an inflammatory rim. The diagnosis was confirmed bacteriologically at direct examination and by culture. Pathology found epidermal necrosis without dermal inflammatory infiltration nor vascular thrombosis. Penicillin was effective in 10 days. DISCUSSION: Bacillus anthracis, the causal agent in anthrax, is a Gram positive rod. The disease is generally seen in herbivorous animals contaminated by plant or animal products. Human anthrax is very rare but should not go unrecognized. In humans, skin contamination is the rule, causing first a vesiculopustulous lesion which progresses to an escarrotic plaque with peripheral inflammation localized on exposed areas. In our case, the hidden localization retarded diagnosis.

[Dermatophytic disease: exuberant hyperkeratosis with cutaneous horns]. / Maladie dermatophytique: hyperkératose exubérante avec cornes cutanées.

BACKGROUND: Dermatophytic disease is a chronic dermatophytosis involving the skin and viscera and caused by benign dermatophytes which cross the skin barrier. We present a case presenting previously unreported giant cutaneous horns. CASE REPORT: A male Algerian patient aged 29 years consulted for chronic skin disease which had progressed for 2 years. Manifestations included warts, papulonodules, eczematiform lesions, lichenifications, alopecia, onyxis, multiple node enlargement and giant cutaneous horns on the plantar aspect of the feet making walking difficult. The patient's kinhood included 10 members and the patient's parents were first cousins; no other family member had a similar disease state. Laboratory findings included eosinophilia (11%, 550 cells/mm3), an inflammatory syndrome, and normal phosphorus and calcium levels despite demineralization of the hands and feet. The tuberculin interdermal reaction was positive but the trichophytine interdermal reaction was negative. Trichophyton violaceum was found in all mycological samples from skin and nails. The histology examination of skin biopsies revealed acanthosis, considerable hyperkeratosis, and myceleal filaments invading the horny layer, the sweat glands and hair follicles. Abscesses and granulomas were also seen in the superficial dermis. Oral griseofulvin 1 g per day and topical applications with ketoconazole gave clear improvement. DISCUSSION: Dermatophytic disease is a chronic dermatophytosis observed mainly in North Africa. The locally high rate of consanguinous marriages would suggest autosomal recessive inheritance of a genetic anomaly possibly associated with deficient cellular immunity. The lesions are polymorphous and develop progressively, probably due to diminished cellular immunity. This case with giant cutaneous horns would be a new variant. Antifungals can provide definitive cure. The pathogenesis of this severe dermatophytosis remains to be established.

[Chancroid in Algeria: the status of this sexually transmitted disease in 1995]. / Le chancre mou en Algérie: état de cette maladie sexuellement transmissible en 1995.

Absent for several decades, the chancroid reappeared in Algeria in 1988. In the unique department of Dermatology and Venereology of the University Hospital of the country of Tlemcen (more than 700,000 inhabitants), we wanted to know the state of this STD seven years after the report of the first cases. The file of the consulting patients were examined. We looked for the principal characteristics of this STD: age, sex, incubation period, place infection contact, type of relation, clinical presentation, evolution without and with treatment, other associated STD (syphilis, HIV). From August 1988 (1st case) to December 1995, 144 cases of chancroid were collected = 1988: 6, 1989: 5, 1990: 7, 1991: 18, 1992: 11, 1993: 33, 1994: 48, 1995: 16. The presentation is quite stereotyped; it concerns males only, singles in must cases, having had sexual relations with prostitutes. The incubation period is short (less than 10 days), the characteristic ulceration presents, very often, some adenopathies. The treatment by cotrimoxazole is efficient. They are no concomitant syphilis or HIV infection. The chancroid is the first cause of genital ulceration in the world. Since 1991, it is the principal STD in our department. It spreads within a male population, young singles associated with prostitutes. It is well installed in Algeria, and its role, although minor, in the transmission of the HIV infection, should not be neglected.

[Hepato-erythropoietic porphyria]. / Porphyrie hépato-érythropoïétique.

INTRODUCTION: Hepatoerythropoietic porphyria is a rare congenital form of porphyria with a biochemical pattern of porphyria cutanea tarda. The disease is caused by defect in uroporphyrinogen decarboxylase. CASE REPORT: A 3-year-old girl was born to healthy consanguineous parents. From birth she had bloody urine and developed photosensitivity with bullae at 1 year of age. Clinical examination revealed scars resulting from bullae, hypertrichosis of the face and members and abnormal pigmentation. Uroporphyrin and coproporphyrin levels in the urine and erythrocyte protoporphyrin level were elevated. Uroporphyrinogen decarboxylase activity was 37.5 p. 100 of the control, and was 82.5 p. 100 in the mother and normal in the father. DISCUSSION: This case of uroporphyrinogen decarboxylase deficiency in a child and her parents was not as severe as reported in other cases in the literature, confirming the heterogenous nature of hepatoerythropoietic porphyria. It is possible that in this case, the mutation was different than in previous cases.

[Allergic vasculitis in brucellosis]. / Vasculite allergique au cours d'une brucellose.

Brucellosis is an anthropozoonosis caused by a Gram negative bacillus of the Brucella gender. Skin manifestations have been reported in 1.5 to 11 p. 100 of the cases. Allergic vasculitis is rare. Recently a 24-year-old man was hospitalized for signs of infection. He had been treated with tetracycline. The clinical picture was suggestive of brucellosis and the Wright test was positive at 1/1,280. There were violet and purpuric papulae on the limbs, arthritis of the knee and ankle joints and renal involvement (haematuria, proteinuria). Histology revealed fibrinoid and leukocytoclastic vasculitis of the small veinules of the subpapillary plexus. Outcome was favourable with rifampicin, doxycycline and adjuvant dapsone, together with bed rest. Several types of skin manifestations have been reported in brucellosis although cases of allergic vasculitis are rare.

[Chromomycosis: 2 cases]. / Chromomycose: 2 observations.

We report two cases of chromomycosis due to Fonsecaea pedrosoi and Cladosporium herbarum, observed in two women from the Tlemcen region. Due to the wart-like or nodular lesions, these case were mistaken for other skin diseases with similar manifestations, particularly verrucous tuberculosis and cutaneous leishmaniasis. The histology revealed fumagoid bodies leading to the identification of the causative agents. Chromomycosis is rare in Algeria although 3 cases have been observed in our area since 1989. The physician should be aware of this diagnosis.

[KID syndrome, pachydermatoglyphy and Dandy-Walker syndrome]. / KID-syndrome, pachydermatoglyphie et syndrome du Dandy Walker.

We report a case of KID syndrome in a young girl born to non-consanguinous parents without any similar family history. The typical features of this dysplasia, erythrokeratodermia with dry rugous teguments, pachydermia folds of the knees, facial erythema, peribuccal grooves, leucokeratosic perleche, hypotrichosis of the eye lashes and eyebrows, early onset deafness and ophtalmological lesions were observed. The characteristic pachydermatoglyphia of the hands was present. A malformation of the posterior cerebral fossa--a dandy walker syndrome--was revealed at computed tomography.

[Costa acro-kerato-elastoidosis]. / Acrokérato-élastoïdose de Costa.

We report 2 cases of acrokeratoelastoidosis of Costa observed in two sisters born of unaffected first cousins. The lesions were lenticular papules, either white or of normal skin colour, which were isolated or arranged in groups of plaques crossed by the natural furrows and located on the thenar eminences, the lateral edges of the hands and fingers and the back of the interphalangeal joints. On the feet, the lesions, located on the lateral edges, the plantar arch and the heel, were much more keratotic and appeared to be translucent. Hyperhidrosis was noted in only one of the two patients. Histological examination showed, beneath an acanthotic and hyperkeratotic epidermis, a reduced elastic network with shorter and thinner fibres in the middle dermis. These are two typical but rough cases of acrokeratoelastoidosis of Costa. The mode of transmission of this disease (autosomal recessive?) seems to be unusual compared with the data found in the literature (dominant, or sporadic cases).

[The reappearance of chancroid in Algeria]. / La réapparition du chancre mou en Algérie.

After 35 observations of the chancroid observed in the department of dermato-venereology of the University Hospital of Tlemcen (West Algeria) from August 1988 to December 1991, we are led to analyze the flare of this sexual transmitted disease. The principal affected subjects are single male no older than 30 years, having had sexual intercourse with prostitutes (30/35). Less than 10 days (19/29) after the sexual contacts, the ulcer appears, and most often unique (25/35), mildly painful, accompanied frequently by adenopathies (31/35). The contamination took place mainly in Bel Abbès--city located at 90 km from Tlemcen--(12 cases), in Tlemcen (4 cases) and Morocco (5 cases). The treatment based on sulfonamides, erythromycin and tetracycline or doxycycline, has been constantly efficient. No concomitant HIV infection has been revealed. The chancroid is the first STD observed in our department in 1991, and also, the first cause for genital ulcer.

PIP: During August 1988 to December 1991 in western Algeria, the dermato-venereology department of the University Hospital in Tlemcen observed 35 cases of chancroid, all of whom were male. About 50% of the cases (18) occurred in 1991. Predominant characteristics of the chancroid cases included contact with prostitutes (30 cases), single (29), and age ranging from 20 to 30 years (70%). Leading sites where contamination occurred were Bel Abbes (90 km from Tlemcen) (12 cases), Tlemcen (4 cases), and Morocco (5 cases). The mean incubation period was 15 days (range, 3 days to 5 months). The genital ulcer appeared within 10 days in 19 men. More than 50% of the men made a visit to the clinic before 20 days of the chancroid's evolution. The ulcer appeared only once in 25 cases. It was accompanied by moderate pain in 13 cases. Gland enlargement occurred in all but 4 cases. None of the cases tested positive for syphilis or for HIV. 25 patients were successfully treated with 4 doses of co-trimoxazole per day for 10-15 days. Erythromycin was used to successfully treat five patients at a dose of 2 g/d for 10 days. The 10-day treatment for the last five cases consisted of 200 mg doxycycline per day, 2 g oxytetracycline per day, and 1 g intramuscular streptomycin. This combination treatment was also effective.

[The extension of cutaneous leishmaniasis in Algeria. Apropos of 25 cases observed in Wilaya de Tlemcen (West Algeria)]. / L'extension de la leishmaniose cutanée en Algérie. A propos de 25 cas observés dans la Wilaya de Tlemcen (Ouest-Algérie).

Following 25 observations, 19 of which are autochtonous, the authors bring to attention the problem of the cutaneous leishmaniasis in Algeria. Actually, this affection touches all the regions. The west part, supposed sofar untouched, has seen the hatching of a foyer in Tlemcen.