A.W.P. Du Vivier

Psychiatric illness in patients referred to a dermatology-psychiatry clinic

There is a recognized psychiatric morbidity among those who attend dermatology clinics. We aimed to determine the pattern of psychological and social problems among patients referred to a liaison psychiatrist within a dermatology clinic. Notes from 149 patients were reviewed and more detailed assessments performed in a subgroup of 32 consecutive referrals. All but 5% merited a psychiatric diagnosis. Of these, depressive illness accounted for 44% and anxiety disorders, 35%. Less common general psychiatric disorders included social phobia, somatization disorder, alcohol dependence syndrome, obsessive-convulsive disorder, posttraumatic stress disorder, anorexia nervosa, and schizophrenia. Classical disorders such as dermatitis artefacta and delusional hypochondriasis were uncommon. Commonly, patients presented with longstanding psychological problems in the context of ongoing social difficulties rather than following discrete precipitants. Psychiatric intervention resulted in clinical improvement in most of those followed up. Of the dermatological categories 1) exacerbation of preexisting chronic skin disease; 2) symptoms out of proportion to the skin lesion; 3) dermatological nondisease; 4) scratching without physical signs, the commonest were dermatological nondisease and exacerbation of chronic skin disease. Anxiety was common in those from all dermatological categories. Patients with dermatological nondisease had the highest prevalence of depression. Skin patients with significant psychopathology may go untreated unless referred to a psychiatrist. The presence of dermatological nondisease or symptoms out of proportion to the skin disease should particularly alert the physician to the possibility of underlying psychological problems.

Serum S‐100 protein: a potentially useful prognostic marker in cutaneous melanoma

S‐100, an acidic calcium‐binding protein, is present within cells of neuroendocrine origin. Its value in the immunohistochemical diagnosis of tumours of melanocytic origin is well established. More recently, a potential role has been proposed for the serum concentration of this protein as a marker of metastatic melanoma disease activity. In the present study, the concentration of serum S‐100 protein was measured in 97 patients with histologically proven malignant melanoma who were attending a dermatology and/or oncology department for the follow‐up of their disease. Serum S‐100 was also measured in 48 control subjects without malignant melanoma. The clinical stage of the patients was classified according to the criteria of the American Joint Committee on Cancer into stages I‐IV. The median (range) serum S‐100 protein concentration was significantly higher in stage I (0·11 (0·1–0·21) μg/L, P<0·001), stage II (0·11 (0·05–0·22) μg/L, P<0·001), stage III (0·24 (0·07–0·41)μg/L, P<0·0001) and stage IV (0·39 (0·06–15·0)μg/L, P<0·0001) compared with the control group (0·1 (0·05–0·15)μg/L). At a threshold value of 0·2μg/L, the sensitivity and specificity for detection of advanced disease were 82% and 91%. respectively. Thus serum S‐100 protein may be a valuable prognostic marker for malignant melanoma and for monitoring therapy. Serum S‐100 protein concentration was also compared with the Breslow thickness of the tumours. There was a significant correlation between these variables (n=72. rs=0·32, P<0·01). Combining a serum S‐100 threshold value of >0·22μg/L and a Breslow thickness of >4mm improved the sensitivity and specificity for the presence of secondary spread to 91% and 95%, respectively. Therefore, a combination of both baseline serum S‐100 protein and Breslow thickness may provide a better indication of the prognosis at diagnosis.

The therapeutic use of topical contact sensitizers in benign dermatoses

Topical therapy using contact sensitizers has been practised since the 1960s to treat conditions associated with an altered immunological state. Dinitrochlorobenzene, squaric acid dibutyl ester and diphencyprone are most commonly employed in the therapy of alopecia areata and viral warts. Few dermatology departments in the U.K. provide such treatment. This systematic review discusses the various contact sensitizers used for topical immunotherapy, the methodology of treatment, factors influencing efficacy and likely adverse effects.

Cutaneous manifestations of granulocyte colony-stimulating factor.

Granulocyte colony‐stimulating factor (GCSF) is a recombinant human growth factor widely used in haematology. It is known to cause cutaneous vasculitis and neutrophilic dermatoses. We present three cases of Sweets syndrome (SS) associated with GCSF use. Raised GCSF levels have been demonstrated in patients with SS. GCSF is the best understood mechanism by which neutrophil accumulation occurs and shows a dose‐dependent effect in provoking SS.

Sclerodermatous graft-versus-host disease: clinical spectrum and therapeutic challenges

Summary Sclerodermatous graft‐versus‐host disease (GVHD) is a rare complication of bone marrow transplantation. While GVHD is often associated with the beneficial graft vs. tumour effect, it also contributes towards significant morbidity and mortality. No reliably effective treatment has yet been established. We present 10 patients with haematological malignancies who underwent an allogeneic stem cell transplant and developed sclerodermatous GVHD. Donor lymphocyte infusion administered for relapse or reducing donor T‐cell chimerism was a known trigger for sclerodermatous GVHD in four of the patients. Treatment with immunosuppressants, psoralen plus ultraviolet A (PUVA) and extracorporeal photopheresis has been largely unsuccessful in their management. Intensive immunosuppression including the use of anti‐CD20 monoclonal antibody may have contributed to relapse of leukaemia in one patient 10 years after her transplant. Sclerodermatous GVHD may occur without a preceding lichenoid stage. Clinical heterogeneity is common, although sclerodermatous GVHD has a predilection for the limbs. Treatment options are largely unsatisfactory if conventional immunosuppression fails. PUVA may give some symptomatic benefit and extracorporeal photopheresis seems to be less efficacious than previously published work suggests.

Congenital erythropoietic porphyria: dilemmas in present day management

Summary Congenital erythropoietic porphyria is a rare autosomal recessive disorder of haem biosynthesis caused by a deficiency of uroporphyrinogen III synthetase. There is resultant accumulation and hyperexcretion of porphyrinogens of the isomer I variety. These are converted by spontaneous oxidation into their corresponding photoactive porphyrins leading to photodamage. Accumulation of porphyrins results in haemolysis and extensive photosensitivity. The consequences of chronic haemolysis are splenomegaly, reactive erythroid hyperplasia, erythrodontia, bone fragility, extreme photosensitivity and photomutilation. We present a 35‐year‐old man who has the severe infantile form and illustrates the haematological and photodestructive complications despite attempts at treatment with hypertransfusion, oral charcoal therapy and beta‐carotene. Allogenic bone marrow transplantation has been considered but because of the high associated mortality this procedure has been discounted at present in the management of our patient.

Angina bullosa haemorrhagica--a possible relation to steroid inhalers.

Angina bullosa haemorrhagica (ABH) is a recently recognized condition, characterized by benign subepithelial blood‐filled blisters in the mouth. The history is characteristic and distinction from other causes of oral blistering can be made by simple clinical signs or on histological grounds. This condition does not appear in standard dermatology texts, yet patients with ABH usually present to a dermatology clinic. A case with typical history is reported in an asthmatic patient who very regularly used a steroid inhaler. The possible aetiological role of such steroid‐based inhalers is discussed.

How do malignant melanomas present and does this correlate with the seven-point check-list?

A pigmented‐lesion clinic was established in the skin department at Kings College Hospital to coincide with the British public‐education campaign on malignant melanoma. One‐hundred cases of malignant melanoma have subsequently been seen and evaluated prospectively with regard to the usefulness of the Glasgow seven‐point check‐list as a guide to diagnosis. The most‐frequently reported patient observations were growth of the lesion (74%) and change in its colour (53%) (especially darkening). However, 50% of the lesions were smaller in size than the 1 cm referred to in the check list and 15% were less than 0.5 cm. Itching was noted in 24% and bleeding and crusting were remarked upon in 18% of cases. Abnormal shape was noted in 20% of the patients. Inflammation was visible in only 11%. Seventy‐three per cent of our patients had early lesions (less than 1.5 mm Breslow thickness) and nearly half presented at the insistence of their relatives or friends or because of the family doctors incidental diagnosis. They stated they would not otherwise have attended the hospital and many had noticed nothing untoward even on direct questioning. Half of the patients scored less than three on the check list and 10 out of the 22 cases of nodular melanoma might not therefore have been referred to our clinic. The check list has subsequently been revised and now provides a more‐sensitive guide.

Neutrophilic eccrine hidradenitis in two neutropaenic patients.

We describe two patients, who presented with erythematous facial plaques, in keeping with neutrophilic eccrine hidradenitis, during chemotherapy for acute myeloid leukaemia. Both patients were neutropaenic and febrile. Histology showed a dermal neutrophilic infiltrate around the eccrine glands with gland destruction. The importance of recognizing this disorder is to prevent the inappropriate use of antibiotics as it is self limiting.

Etretinate and AIDS-related Reiter's disease

We report a case of a 34‐year‐old homosexual man who developed Reiters disease with severe cutaneous and joint involvement shortly after he was diagnosed as having acquired immunodeficiency syndrome (AIDS). Therapy with potent topical steroids and oral methotrexate was ineffective, but etretinate at a dose of 0.75 mg/kg resulted in rapid clearing of the skin lesions and a marked improvement of his arthralgia.