Fibrous papule of the face with granular cells.

Fibrous papule of the face is a common benign lesion located most often on the nose. It presents usually as a single small, firm, skin-coloured papule and is often misdiagnosed as melanocytic naevus, wart or small nodular basal cell carcinoma. Histopathologically, the lesions are characterized by involvement of the upper dermis by a fibrovascular proliferation and scattered triangular or stellate, often multinucleated cells. Uncommon histopathologic variants of fibrous papule of the face include hypercellular, clear-cell, pleomorphic, pigmented, inflammatory and granular-cell types. We present here a patient with the syndrome of familial cancer and fibrous papule of the face with granular cells. The granules stained strongly with PAS stain, as well as with CD68 and NKI/C3 immunostains, whereas S-100 protein resulted negative. In our patient the mutations in the 2 most often affected DNA mismatch repair genes of Muir-Torre syndrome were not found, therefore the origin of the familial cancer syndrome remains unknown. Probably the occurrence of the granular-cell fibrous papule of the face was coincidental.

Lupus erythematosus profundus in black South Africans.

BACKGROUND: Lupus erythematosus profundus (lupus panniculitis) is a rare variant of lupus erythematosus with predominant involvement of the subcutaneous tissue. There are only a few reported series of patients with this condition; none in individuals of African ancestry. The aim of the study was to evaluate clinical, histopathological and laboratory findings in black South African patients with lupus profundus. PATIENTS AND METHODS: Ten prospectively observed patients were studied. Skin biopsies were performed, and several laboratory tests routinely employed in patients with lupus erythematosus were carried out. All patients were followed up for at least a year and their response to treatment was assessed. RESULTS: All patients were females. Mean age at diagnosis was 28.5 years. Face was the most common site of involvement. In five patients, the periorbital edema was the initial manifestation. Only one patient had systemic lupus, and three patients also showed lesions of discoid lupus. In only 30% of the patients were the ANA titers greater than 1 : 80, and positivity in other lupus tests was rare and inconsistent. In general, patients responded to antimalarials and/or systemic corticosteroids. CONCLUSION: African patients with lupus profundus when compared with other series comprising Asian and white patients showed greater frequency of periorbital edema as the initial manifestation. Review of the literature disclosed that this subset of lupus profundus appears to have a more benign course.

Squamous cell carcinoma arising in CHILD syndrome.

Congenital hemidysplasia with ichthyosiform naevus and limb defects (CHILD) syndrome is a rare X-linked dominant disorder. The first case of squamous cell carcinoma arising within the affected ichthyosiform skin in a 33-year-old woman is reported.

Highly aggressive extraocular sebaceous carcinoma.

Extraocular sebaceous carcinoma is an uncommon neoplasm usually localized on the head and neck. We report a case of sebaceous carcinoma of the axillary skin with a highly aggressive behavior. The patient was a 43-year-old black man who developed multiple cutaneous and lymph node metastases shortly after the excision of primary sebaceous carcinoma of the axillary skin. Many neoplastic aggregations were identified within the lumina of the dermal lymphatic vessels in the excised specimen of the primary neoplasm. Although extraocular sebaceous carcinoma has been traditionally considered a less aggressive neoplasm than its ocular counterpart, a review of the literature and this case demonstrate that extraocular sebaceous carcinoma may also lead to disseminated metastatic disease.

Adapalene in the treatment of African patients.

AIM: To assess the efficacy and safety of topical adapalene gel 0.1% as a treatment for acne vulgaris in black South African patients. BACKGROUND: African and other darker skin types represent a particular clinical challenge for dermatologists treating acne. In many cases, this is due to the higher risk of postinflammatory hyperpigmentation in patients with dark skin. Acne vulgaris is an extremely common dermatological problem among Africans and people of African descent worldwide. Few studies of any of the major acne therapies have been carried out in exclusively black populations, and relatively little is known about the specific responsiveness of black skin to these agents. The ideal acne treatment for black people would specifically target the inflammatory process, which so often results in hyperpigmentation. Topical retinoids do this to some degree, but they can be highly irritating and this in itself can provoke post-treatment hyperpigmentation. METHODS: An open-label study of adapalene 0.1% gel in 65 black South Africans, aged 12-30, for 12 weeks. Patients all had mild to moderate facial acne as defined by the Leeds scoring system; they were instructed to apply the medication once daily. Lesion counts and severity scores were assessed at 4, 8 and 12 weeks. RESULTS: A total of 44 subjects completed the trial and all three follow-up visits. Adapalene gel 0.1% showed clear efficacy against both inflammatory and non-inflammatory lesions. The drop in mean total facial-lesion count ranged from 46 to 72% between the first and last visit, and in most cases, there was clear improvement in cosmesis. In two-thirds of cases, patients experienced reductions in both number of hyperpigmented macules and density of hyperpigmentation. CONCLUSION: Adapalene gel 0.1% is an effective, well-tolerated topical therapy for black patients. It is able to reduce both inflammatory and non-inflammatory lesions, as well as prevent and alleviate acne-associated hyperpigmentation.

Adapalene gel 0.1% for topical treatment of acne vulgaris in African patients.

Acne vulgaris is a common dermatologic disease in African patients, as well as in Caucasians. Our report evaluates the safety and efficacy of adapalene gel 0.1% in African patients with acne vulgaris. We used a 12-week, 2-center, open-label, noncomparative study of adapalene gel 0.1% in 65 African patients with acne vulgaris to assess the drug's effect on hyperpigmented lesions in people with dark skin. The study demonstrated that the progressive and significant improvements in lesion counts and global acne grades produced by adapalene in African patients were paralleled by significant improvements in the degree of hyperpigmentation of acne lesions. During treatment with adapalene, less than 5% of patients reported moderate or severe skin irritation at any time during the study, and the incidence of skin oiliness decreased markedly. We conclude that adapalene gel 0.1% was well tolerated and highly effective in African patients with acne vulgaris and was found to reduce hyperpigmentation.

Cutaneous sarcoidosis in black South Africans.

BACKGROUND: It has been observed that, in the USA, sarcoidosis is more common in African-Americans than in other races. It has also been noted that sarcoidosis in African-Americans is characterized by more severe extrapulmonary involvement and more exuberant skin lesions. There is little information on sarcoidosis in black Africans. METHODS: Fifty-four black South African patients with cutaneous lesions of sarcoidosis proven by biopsy were prospectively studied. Dermatologic and ophthalmologic examinations and chest X-rays were performed in all patients. Other investigations relevant in the diagnosis of extracutaneous sarcoidosis were also performed in a variable number of patients. RESULTS: In 40 patients (71%), systemic sarcoidosis was found with lung, eye, and acral bone involvement being most common. Great variations in the morphology of skin lesions were observed. In one-quarter of patients, atypical cutaneous lesions (hypopigmented, ichthyosiform, lymphedematous, mutilating, ulcerative, verrucous) were found. Lupus pernio, once thought to be confined to Northern Europe, was observed in five patients in the subtropical milieu of South African Transvaal. Sarcoidal dactylitis with nail changes was seen in eight patients. Fibrinoid necrosis was found in 12% of the biopsies. CONCLUSIONS: Sarcoidosis in black South Africans is characterized by extensive cutaneous involvement. The lesions are morphologically extremely variable, frequently atypical, and often demonstrate fibrinoid necrosis on histology.

Xeroderma pigmentosum in black South Africans.

BACKGROUND: Xeroderma pigmentosum occurs in all races. There is little information on its clinical picture, frequency, and types of malignant lesions in individuals of African negroid extraction. METHODS: Fifteen black South African patients, aged from 10 months to 21 years, with xeroderma pigmentosum were prospectively studied. Detailed dermatologic, ophthalmologic, and neurologic examinations were carried out in all patients. Cutaneous, conjunctival, and lingual malignant lesions were histologically assessed. Complementation studies were not performed. RESULTS: Twelve patients had cutaneous malignancies, predominantly squamous carcinomas, usually several at one time. Carcinomas of the tip of the tongue occurred in five patients and conjunctival carcinomas in four. Cutaneous and mucosal carcinomas developed at an earlier age than in the series from other parts of the world. Three patients had extensive skin involvement characteristic of xeroderma pigmentosum, but did not develop malignancies. They most likely belong to a complementation group(s) with higher rates of unscheduled deoxyribonucleic acid (DNA) synthesis. CONCLUSIONS: Xeroderma pigmentosum in South African black people is characterized by the very early development of cutaneous, ocular, and tongue squamous cell carcinomas and usually has a rapid and devastating course.

Pityriasis rubra pilaris in black South Africans.

Eighteen black South African patients with pityriasis rubra pilaris were prospectively studied, nine adults and nine children; patients with the classical types (namely I and III) constituted 75% of the group. Follicular involvement was very prominent, especially in the children with type III, but otherwise the clinical and histological findings did not differ from those reported in white patients. This is the first series of black African patients with this condition to be reported.

Tubular apocrine carcinoma arising in a nevus sebaceus of Jadassohn.

We present an example of tubular carcinoma that developed within a nevus sebaceus of Jadassohn on the scalp of a 53-year-old woman. The neoplasm showed features of sebaceous and apocrine differentiation. We review the literature about the malignant neoplasms arising in the nevus sebaceus of Jadassohn.

Cutaneous infection due to Cladophialophora bantiana in a patient receiving immunosuppressive therapy.

Cladophialophora bantiana (Xylohypha bantiana, Cladosporium trichoides), a dematiaceous fungus, causes mainly infections of the central nervous system. Systemic antifungal therapy is apparently unsuccessful. Extracerebral involvement is uncommon and only few cases have been reported. We describe a 63-year-old patient, a kidney transplant recipient on prolonged prednisone and cyclosporin, who developed a large single lesion on the thigh from which Cladophialophora bantiana was cultured. A 3-month course of itraconazole 200 mg daily resulted in complete healing of the lesion. To the best of our knowledge this is the first report on cutaneous infection with Cladophialophora bantiana in an organ transplant recipient and the first in whom treatment with itraconazole led to resolution.

Viral warts in a patient with Darier's disease show acantholytic dyskeratosis.

A 15-year-old boy with classic clinical and histopathological features of Darier's disease developed viral warts that were confirmed by DNA hybridization studies. Histologically, there were features of acantholytic dyskeratosis in otherwise typical common warts.

Systemic lupus erythematosus in South African blacks: prospective study.

BACKGROUND: The aim of the study was to evaluate clinical and laboratory findings in black South African patients with systemic lupus erythematosus (SLE) and to compare these with findings in a group of white patients. MATERIALS AND METHODS: Forty black patients were studied. The group of exclusively white patients reported by Beutner et al. was used for comparison. RESULTS: A decreased frequency of malar rash, photosensitivity, and hematologic involvement was found in black patients. They had anti-Sm and anti-nRNP antibodies and low complement more often than white patients with SLE in Beutner et al. group. CONCLUSIONS: More differences have been noted in laboratory than in clinical parameters and findings reported in African-Americans with SLE have been confirmed in black Africans. Ethnic factors may play a role in the symptomatology of SLE.

Pachydermatous eosinophilic dermatitis.

We report three South African black teenage girls with extensive pruritic papular lesions arising on a pachydermatous base, resembling severe atopic dermatitis or onchodermatitis. All three had peculiar hypertrophic genital lesions and peripheral blood eosinophilia. Histological studies showed an eosinophil-rich lymphohistiocytic infiltrate and variable fibrosis. Extensive fibrillar extracellular deposition of eosinophil granule major basic protein was demonstrated by an indirect immunofluorescence technique. A beneficial therapeutic effect was obtained using dapsone, prednisolone and cetirizine. The term pachydermatous eosinophilic dermatitis is proposed and its position among other conditions characterized by peripheral blood and skin tissue eosinophilia, is discussed.