Bullous Systemic Lupus Erythematosus

Two patients with bullous systemic lupus erythematosus are reported. In one, the disease appears to have been caused by hydralazine. The cutaneous lesions of bullous SLE have not been previously reported in drug-induced lupus.     

SPOROTHRIX SCHENCKII—INCIDENCE IN THE SYDNEY REGION

Sporothrix schenckii is a fungus which occurs in soil and on plant material. It has not often been reported in Australia from clinical sources. Most reports have been from Queensland, and it has rarely been reported from the Sydney and Newcastle Metropolitan areas. Twelve isolations from the Sydney area are reported.     

Familial multiple blue naevi

Blue naevi are benign melanocytic neoplasms. They are usually solitary blue or blue-black nodules or plaques. Multiple blue naevi have been reported rarely and there is one report of familial solitary blue naevus. However, familial multiple blue naevi have not been reported previously. We report the case of a family with multiple blue naevi occurring in three generations with an autosomal-dominant inheritance pattern.     

Palmoplantar keratoderma with amyotrophy

Four members of a South African Black family who have palmoplantar keratoderma with amyotrophy are reported. No neuromuscular defects have been found and the muscle thinning appears to result from disuse atrophy. Volar hyperhidrosis, nail abnormalities and in 2 cases knuckle pads, were additional features. The condition appears to be inherited as an autosomal dominant trait. We are not aware of a similar syndrome having been previously reported.     

Acanthosis nigricans in a plaque of scleredema on the back of a diabetic patient: a case report

Acanthosis nigricans (AN) and scleredema are two skin conditions that have been reported in association with diabetes mellitus. Few cases associating scleredema and AN have been reported. A literature search did not reveal any reports of diabetic patients developing AN on top of a scleredema plaque. Here we report a patient with diabetes mellitus who developed AN within the same indurated scleredema plaque.     

Cutaneous manifestations of Costello syndrome

Cutaneous findings are common in Costello syndrome, but have not been extensively reviewed in the dermatology literature. We present the cutaneous and histopathologic findings from two cases of Costello syndrome and review previously described cutaneous features of this syndrome. Both patients had manifestations of Costello syndrome with thick, lax skin on the dorsal aspects of hands and feet, deep palmar and plantar creases, curly hair, hyperkeratoses, acanthosis nigricans, papillomas, and multiple pigmented lesions. One patient had multiple syringomas on the forearms, a finding not previously reported. Pigmented lesions have previously been reported as nevi in the literature though no biopsies have been reported. We conclude that thick, loose skin on the dorsal aspects of hands and feet and deep palmar and plantar creases are cardinal manifestations of Costello syndrome and benign tumors of ectodermal origin such as papillomas, calcified epitheliomas, dermoid cysts, mammary fibroadenosis, and syringomas are important features of this syndrome.     

Colletotrichum species as emerging opportunistic fungal pathogens: a report of 3 cases of phaeohyphomycosis and review.

BACKGROUND: Numerous etiologic agents of subcutaneous phaeohyphomycosis have been reported. Colletotrichum spp, common plant pathogens, have been reported as a cause of ocular keratomycosis, but only one previous case of cutaneous disease (hyalohyphomycosis) has been attributed to this genus. OBJECTIVE: Our purpose was to describe 3 cases of subcutaneous phaeohyphomycosis due to Colletotrichum spp occurring in patients undergoing chemotherapy for hematologic malignancies. METHODS: Three cases of Colletotrichum-induced phaeohyphomycosis are reviewed. The clinical and histologic features of this infection are presented, the antifungal susceptibilities are reported, and treatment options are discussed. RESULTS: We describe the first report in which C coccodes and C gloeosporioides are implicated as etiologic agents of subcutaneous phaeohyphomycosis. Despite treatment, one patient died after the onset of visceral fungal disease. CONCLUSION: Colletotrichum spp may cause life-threatening phaeohyphomycosis in immunosuppressed patients. Prompt recognition and intervention with surgical and antifungal treatment may result in decreased morbidity and mortality associated with these infections.     

Multiple eruptive dermatofibromas and immunosuppression: report of two cases and review of the literature

Dermatofibromas are common benign fibrohistiocytic tumors that are most often solitary. The occurrence of multiple eruptive dermatofibromas (MEDF), on the contrary, is a rare event. MEDF have been reported in the setting of autoimmune diseases, treated with immunosuppressive drugs, in the course of HIV infection and in neoplastic diseases. An association with immunosuppression has led to the speculation that they are the result of an abortive immunoreactive process. Here, we describe a patient with Sézary syndrome and a patient with multiple IgA myeloma who developed MEDF. These associations have not been reported previously.     

Delayed systemic allergic reactions to corticosteroids

From the limited number of reports in the literature, it would appear that various types of delayed reactions from systemically, intralesionally, and intra-articularly administered corticosteroids are rare, particularly given their widespread use. The purpose of this literature review is to summarize in table form the reported cases of generalized delayed systemic corticosteroid reactions with respect to patient presentation, methods of evaluation, and conclusions reached. In total, 24 cases of generalized delayed systemic corticosteroid reactions have been reported in the literature. Clinical presentation (timing and cutaneous manifestations) as well as evaluation of these patients has been variable; reactions reported include eczematous or exanthematous eruptions, with or without bullae or purpura. In 16 cases, the diagnoses have been supported by positive patch or intradermal testing. Overall, it appears that generalized delayed systemic reactions to corticosteroids show considerable variability and are infrequently reported. Patch and intradermal tests with standardized allergens appear to be the most useful tests currently available to support this clinical diagnosis.     

Oesophageal webs preceding carcinoma and rupture of the oesophagus in cicatricial pemphigoid

A case in which oesophageal webs preceded the development of carcinoma and rupture of the oesophagus in a 77-year-old woman with cicatricial pemphigoid is reported. Oesophageal webs in cicatricial pemphigoid have been reported but are rare. Clinical, histological, radiological and post-mortem features are described. Western immunoblotting of serum demonstrated a 180-kDa antigen which comprises one of the antigens reported in cicatricial pemphigoid.     

Mal de Meleda: a review of Turkish reports

Keratoderma Palmoplantare Transgrediens or mal de Meleda (MDM) is a very rare, inherited disorder. Its name derives from the island of Meleda. Although, MDM has been reported from many different countries, only two Turkish patients with MDM have been reported in the international literature. Our six cases of MDM are reported, and retrospective analysis of published Turkish patients with MDM is included. We review the clinical characteristics, associated findings, consanguinity, and family history of the cases. In total, 26 Turkish cases of MDM including ours have been documented. Ten cases showed various nail abnormalities, the most common associated feature. We add 24 new cases from Turkey to the list in the literature. Our findings suggest that the MDM gene may also have originated elsewhere than in Mljet.     

Atypical cutaneous lymphoproliferative disorder resembling mycosis fungoides in AIDS. Report of a case with concurrent Kaposi's sarcoma

A variety of neoplastic disorders have been described in patients with AIDS. Kaposi's sarcoma is the most common neoplasm and is recognized as one of the diagnostic criteria for AIDS. Unusual mucocutaneous carcinomas have also been reported in these patients, as have a variety of lymphoid neoplasms, including Hodgkin's and non-Hodgkin's lymphomas (NHLs). The NHLs that occur in AIDS patients are usually of B-cell or non-B, non-T-cell phenotype. In contrast, T-cell lymphomas have only rarely been reported in this patient population. We present the clinical, morphologic, and immunologic features of an atypical, cutaneous lymphoproliferative disorder resembling mycosis fungoides that developed in a 28-year-old homosexual man with AIDS and disseminated mucocutaneous Kaposi's sarcoma.     

Human keratin diseases: the increasing spectrum of disease and subtlety of the phenotype–genotype correlation

Keratins are obligate heterodimer proteins that form the intermediate filament cytoskeleton of all epithelial cells. Keratins are tissue and differentiation specific and are expressed in pairs of types I and II proteins. The spectrum of inherited human keratin diseases has steadily increased since the causative role of mutations in the basal keratinocyte keratins 5 and 14 in epidermolysis bullosa simplex (EBS) was first reported in 1991. At the time of writing, mutations in 15 epithelial keratins and two trichocyte keratins have been associated with human diseases which include EBS, bullous congenital ichthyosiform erythroderma, epidermolytic palmoplantar keratoderma, ichthyosis bullosa of Siemens, diffuse and focal non-epidermolytic palmoplantar keratoderma, pachyonychia congenita and monilethrix. Mutations in extracutaneous keratins have been reported in oral white sponge naevus and Meesmann's corneal dystrophy. New subtleties of phenotype-genotype correlation are emerging within the keratin diseases with widely varying clinical presentations attributable to similar mutations within the same keratin. Mutations in keratin-associated proteins have recently been reported for the first time. This article reviews clinical, ultrastructural and molecular aspects of all the keratin diseases described to date and delineates potential future areas of research in this field.     

Antioxidants in cosmetics

Antioxidants are commonly added to cosmetics. Allergy to these substances will be missed unless specifically looked for. During a 2-year period, patients with facial dermatitis have been routinely tested with butylhydroxyanisole and tertiary butylhydroquinone. 7 patients are reported who have been shown to have an allergic contact dermatitis to an antioxidant in their cosmetics or toiletries.     

Erythema with features of seborrheic dermatitis and lupus erythematosus associated with systemic 5-fluorouracil

Multiple cutaneous reactions have been reported in association with topical and systemic 5-fluorouracil therapy. Three patients are reported who noted facial erythema with features of both seborrheic dermatitis and lupus erythematosus following the administration of systemic 5-fluorouracil.     

Cutaneous apocrine carcinoma of the scrotum: A case with widespread subcutaneous induration

Cutaneous apocrine carcinoma (CAC) is a rare malignancy. It develops predominantly in the regions where apocrine glands are distributed. Some cases of CAC have been reported in the axilla and the inguinal regions, but only a few in the scrotum. We herein report a case of CAC which widely spread over both sides of the whole scrotum with plate‐like hard induration, and such a manifestation has never been reported before. CAC is known to have high rates of local recurrence or metastasis, and the efficacy of radiotherapy or chemotherapy has not been established. As therapeutic options for CAC are limited, it is critical to reach the diagnosis and treat at an early stage.     

Poromatosis in pregnancy: a case of 8 eruptive poromas in the third trimester

The poroid family of neoplasms includes hidroacanthoma simplex, eccrine poroma, dermal duct tumor, and poroid hidradenoma. These benign adnexal neoplasms are derived from the eccrine or apocrine sweat ducts or glands. Poroid neoplasms, including poromas, have been reported during pregnancy and have been hypothesized to be hormonally influenced. Poromatosis, the occurrence of multiple poromas, rarely has been reported in association with hidrotic ectodermal dysplasia, prior radiation therapy, and non-Hodgkin lymphoma occurring after chemotherapy. We report a case of eruptive poromatosis in pregnancy with 8 poromas occurring in the third trimester, further supporting the hypothesis of a hormonal association in the etiology of this neoplasm.     

Non-bullous congenital ichthyosiform erythroderma, with ocular albinism and Noonan syndrome

A 21-year-old woman presented with non-bullous congenital ichthyosiform erythroderma; she was born a collodion baby. Associated features were ocular albinism, anterior segment dysgenesis of both eyes and Noonan syndrome. X-linked ichthyosis (steroid sulphatase deficiency) and X-linked ocular albinism have been mapped to the Xp22.3 region and cases have been reported with both conditions due to a partial short-arm deletion of the X chromosome. The ichthyosis and ocular albinism in the present case, however, are likely to be of the autosomal recessive type - a very rare association - and the combination with Noonan syndrome has not been reported previously.