Neutrophilic urticarial dermatosis: a novel association with poststreptococcal rheumatic disease.

Neutrophilic urticarial dermatosis (NUD), a particular clinical and histological entity, can provide a strong pointer to underlying systemic disease, most frequently rheumatological diseases. We report the first case of NUD in association with a post-streptococcal rheumatic disease, with symptoms including recurrent sore throat, raised antistreptolysin O titre, persistent transient urticaria, polyarthralgia, rheumatic mitral valve disease and Jaccoud arthropathy. Histologically, NUD is characterized by an intense superficial and deep neutrophilic interstitial and perivascular infiltrate, without significant oedema or blood vessel damage. These neutrophils may have a tendency to concentrate along the basement membrane and extend into the epidermis, hair follicles, sebaceous glands and sweat glands (a feature termed 'neutrophilic epitheliotropism'). Clinicians should remain cognizant of NUD, and in particular its frequent association with an underlying inflammatory disorder.

Bullous subacute cutaneous lupus erythematosus.

We describe a 59-year-old woman, with a history of autoimmune disease and disseminated uterine leiomyosarcoma, who developed a photoaggravated, blistering skin eruption. An initial rash, at the outset of treatment with chemo- and radiotherapy, resembled erythema multiforme. Review of the original skin biopsy showed it to be subacute cutaneous lupus erythematosus. There were no systemic symptoms or signs to suggest systemic lupus erythematosus. The much later photoaggravated rash consisted mainly of bullae and eventual epidermal denuding which resembled toxic epidermal necrolysis. We propose that the clinical and histological diagnosis is one of bullous subacute cutaneous lupus erythematosus in a patient with no other features of systemic lupus erythematosus.

The management of psoriasis.

Psoriasis is a common condition affecting 1.5-2.0% of Western populations. It occurs equally commonly in men and women. Although it affects mainly the skin, an associated arthritis occurs in approximately 3-4% of those affected. It is rarely life-threatening, but is frequently the cause of significant morbidity, both physical and psychosocial. Management may involve topical treatments, phototherapy or photochemotherapy or systemic agents. The choice of treatment depends on a variety of factors, including the extent and type of the cutaneous lesions, the presence of an associated arthritis and whether the patient has any other intercurrent disease. Patients receiving treatment with systemic agents require careful supervision, as those currently used have potentially serious adverse effects.

Dermatitis herpetiformis and bullous pemphigoid: a developing association confirmed by immunoelectronmicroscopy.

We report a case of dermatitis herpetiformis associated with bullous pemphigoid in which the clinical, histopathological and immunopathological findings were corroborated by direct immunoelectronmicroscopy performed on normal and peri-lesional skin biopsies. Western immunoblotting detected the 180 kDa BPAG2 consistent with bullous pemphigoid. HLA-typing confirmed the DR3 DQ2 haplotype associated with dermatitis herpetiformis.

Anogenital lichen sclerosus in women.

A study of 350 women with lichen sclerosus, originally made to elucidate the relationship between lichen sclerosus and autoimmunity, led to the amassing of a considerable amount of clinical material. Our review is confined to those with anogenital lesions (342), supplemented by some new cases (15), giving a total of 357 women with biopsy proven lichen sclerosus. It demonstrates the wide age range of the condition, the association with morphoea and lichen planus and the occurrence of squamous cell carcinoma in some cases. It also shows that inappropriate surgery has continued to be carried out for benign disease.

Proximal cutaneous necrosis in association with chronic renal failure.

We report two patients with renal failure who developed painful symmetrical ulceration over the proximal thighs and abdominal wall. Histological examination of the ulcerated areas revealed intravascular calcification. This phenomenon has been termed calciphylaxis and is a rare complication of renal failure. The pathogenesis is poorly understood. However, abnormalities of calcium/phosphate metabolism and of coagulation are important.

Acrokeratosis paraneoplastica: Bazex syndrome.

Bazex syndrome, or acrokeratosis paraneoplastica, is a cutaneous paraneoplastic syndrome characterized by psoriasiform lesions associated with, usually, a squamous cell carcinoma of the upper aerodigestive tract. We present a case of Bazex syndrome associated with metastatic cervical squamous cell carcinoma with an unknown primary. The features of the condition are discussed in the light of current knowledge.

Dermatology in the intensive care unit.

We report our experience, and review the literature, concerning 'intensive care dermatology'. Over a period of 14 months, 27 patients who had significant cutaneous problems were seen in the intensive care unit. These included primary dermatological conditions, multisystem disorders with cutaneous signs, complications of dermatological therapy, and skin conditions developing as complications of intensive care. We discuss the diagnosis and management of dermatological problems in the intensive care unit.

Chronic verrucous varicella-zoster infection in a patient with AIDS.

The expression of herpes varicella zoster virus in patients already infected with the human immunodeficiency virus (HIV) provides the perfect illustration of an opportunist microbe at work. There is an increased incidence of herpes zoster which may be more severe, recurrent or disseminated. Likewise, varicella may be more severe and recurrent. In both patterns of infection atypical lesions in the form of persistent ulcerative or verrucous lesions have been seen. An HIV-positive patient is described who developed verrucous lesions de novo, 4 months after her child had varicella.

Ichthyosis hystrix and skin cancer.

Ichthyosis hystrix is a rare genodermatosis characterized by persistent spiny scales which cover a significant part of the skin surface. In many cases it is associated with palmoplantar keratoderma, and occasionally deafness and neurological defects coexist. The case of a man with ichthyosis hystrix is reported who developed skin malignancy and dysplastic keratoses, a complication which has not previously been recorded.

The anticonvulsant hypersensitivity syndrome.

Anticonvulsants can cause a characteristic hypersensitivity reaction. This multisystem reaction typically presents as fever, mucocutaneous eruptions, lymphadenopathy and hepatitis. There is cross-reactivity between different anticonvulsants, which complicates subsequent therapy. We report three cases to illustrate both the typical features, and less common complications, of this under-recognized and life-threatening syndrome.

Angioendotheliomatosis associated with Castleman's lymphoma and POEMS syndrome.

Castleman's lymphoma, a benign localized lymphoid hyperplasia, has been reported in association with POEMS syndrome, a multisystem reactive disorder that includes several skin manifestations. Benign reactive angioendotheliomatosis is a rare skin disorder that is associated with various systemic disorders. A patient with long-standing Castleman's lymphoma and POEMS syndrome also developed benign reactive angioendotheliomatosis.

HTLV-1-associated cutaneous disease: a clinicopathological and molecular study of patients from the U.K.

The clinicopathological features of eight patients with cutaneous disease associated with HTLV-1 infection are reviewed. All were U.K. residents of West Indian extraction, and two are currently alive. Disease remained confined to the skin in two patients. Five patients with a cutaneous prodromal phase developed leukaemia after a median duration of 124 months (3 months-21 years), and in one of these combination chemotherapy produced a sustained clinical remission for 20 months. Two patients developed cutaneous disease after remission of their leukaemia. Cutaneous lesions were heterogeneous and included localized papules, a generalized papulonodular eruption, diffuse and localized erythematous plaques, pompholyx-like lesions on the palms and soles, and tumours. The histology of the skin lesions was also variable, and consisted of a heavy dermal infiltrate with lymphocytes, histiocytes, plasma cells, eosinophils and cytologically atypical mononuclear cells. Epidermotropism was present in biopsies from five patients. Tumour cells with large, densely staining, pleomorphic nuclei, arranged in rows between collagen bundles, were present in the majority of cases. In one patient the infiltrate also consisted of epithelioid cells and multinucleated giant cells. Six cases were classified histologically as pleomorphic T-cell lymphoma, and two as cerebriform or mycosis fungoides type. Molecular studies revealed a clonal T-cell population associated with monoclonal integration of HTLV-1 provirus in tissue DNA from six patients. In two patients HTLV-1 integration was established retrospectively using enzymatic in vitro amplification of a specific HTLV-1 po1 gene sequence in DNA extracted from paraffin-embedded sections. This study indicates that the clinical and pathological features of HTLV-1-associated cutaneous disease are diverse. Patients may have disease confined to the skin for prolonged periods, either at presentation or following clinical relapse--cutaneous adult T-cell lymphoma. Molecular techniques allow distinction from other types of cutaneous T-cell lymphoma, and provide an opportunity for retrospective studies of archival material.

Acrokeratosis paraneoplastica of Bazex.

Bazex disease is one of the rarer cutaneous paraneoplastic syndromes. It is characterized by psoriasiform changes on the digits, and in some patients spread to the ears, nose and in later stages to the limbs and trunk. The associated malignancy is typically a squamous cell carcinoma of the upper aerodigestive tract. We review the literature regarding acrokeratosis paraneoplastica of Bazex and report three cases which illustrate both the typical and some of the less common changes that are seen in the condition.