Clinical and Experimental Dermatology

Sarkany, R. P. E.

doi: 10.1046/j.1365-2230.2001.00825.xpmid: 11422163

Porphyria cutanea tarda (PCT), the commonest of all porphyrias, is usually characterized by blisters and fragility of skin in light‐exposed areas. It can be clinically indistinguishable from other disorders including variegate porphyria and the diagnosis can only be made by rigorous biochemical analysis. PCT does not cause acute attacks of porphyria. It is usually an acquired condition caused by inhibition of the uroporphyrinogen decarboxylase enzyme in the liver. Hereditary haemochromatosis, hepatitis C virus infection, alcohol, oestrogens and a family history of PCT are the major risk factors for the condition and should be searched for specifically in all patients. Liver disease, including hepatocellular carcinoma, is common in patients with PCT, and should be investigated for at presentation by means of a liver biopsy where possible. Patients with severe hepatic pathology or longstanding untreated PCT need to be monitored for the development of hepatocellular carcinoma in the long term. Low dose twice weekly chloroquine is the mainstay of treatment, but venesection should be used in patients with severe iron overload or hepatitis C‐related liver disease. Subsequently, long‐term follow‐up is needed in all patients to monitor for relapse.

Balagon, M. V.; Tan, P. L.; Prestidge, R.; Cellona, R. V.; Abalos, R. M.; Tan, E. V.; Walsh, G. P.; Watson, J. D.; Walsh, D. S.

doi: 10.1046/j.1365-2230.2001.00804.xpmid: 11422164

The aim of new treatments for psoriasis is to induce extended remissions with fewer side‐effects. Previous studies suggest that Mycobacterium vaccae, a harmless organism prepared as a heat‐killed suspension, may induce periods of remission in some psoriasis patients after intradermal administration. To assess a more potent derivative of M. vaccae, we conducted an open‐label study in which 20 patients with moderate to severe psoriasis (Psoriasis Area and Severity Index of 12–35) received two intradermal inoculations of heat‐killed, delipidated, deglycolipidated M. vaccae (DD‐MVAC or ‘PVAC’) in lesion‐free deltoid skin, separated by a period of 3 weeks. Twelve weeks after the injections, 13 out of 20 patients (65%) showed marked improvement in the PASI score (> 50% reduction), three were unchanged (< 25% reduction), three had worsened (> 5% increase), and one was withdrawn from the trial because of an exfoliative flare. At 24 weeks, 13 out of 19 patients continued to show > 50% improvement that, in some, lasted for 6 months or longer. Patients classified as good responders at 12 or 24 weeks were then offered additional PVAC injections after 24 weeks if the PASI reached 8 or higher. Intra‐dermal administration of PVAC was safe, well tolerated, and induced clinically significant improvement in many psoriasis patients. A randomized, double‐blind, controlled study is warranted.

Cho, Kwang‐Hyun; Kim, Chul‐Woo; Heo, Dae‐Seog; Lee, Dong‐Soon; Choi, Won‐Woo; Rim, Jong‐Hyun; Han, Won‐Suk

doi: 10.1046/j.1365-2230.2001.00805.xpmid: 11422165

We describe two Korean adult patients who had necrotizing papulovesicles mainly on their faces. Skin biopsy specimens showed perivascular and periadnexal infiltrate of atypical lymphoid cells with vasculitis in the dermis and subcutaneous tissue. In situ hybridization demonstrated a latent infection of Epstein–Barr virus in the majoriy of lymphoid cells in the dermis. These patients were diagnosed as having T‐cell lymphoma. Interestingly, large granular lymphocytosis was found in the peripheral blood of Case 2.

Varma, S.; Holt, P. J. A.

doi: 10.1046/j.1365-2230.2001.00806.xpmid: 11422166

Tear gas is used throughout the World for control of riots and civil disobedience. CS gas as used by the UK police force is issued as a ‘spray’ and is 5% CS in methylisobutylketone (MIBK), a potent irritant. Assaults on police officers in forces issued with CS spray have fallen significantly over the past 3 years, whilst having risen in areas without it. Thus, CS gas appears to be an effective deterrent. However, significant cutaneous reactions can occur as a result of exposure. We report a severe contact dermatitis to CS gas to highlight the clinical features. The nature of CS gas and potential cutaneous adverse reactions are discussed.

Buckley, D. A.; Munn, S. E.; Higgins, E. M.

doi: 10.1046/j.1365-2230.2001.00807.xpmid: 11422167

Eosinophilic pustular folliculitis (EPF) of infancy is a rare disorder which may begin in the neonatal period and cause considerable parental anxiety. It must be distinguished from other causes of a pustular eruption in neonates, including infection and erythema toxicum neonatorum, and rare disorders such as transient neonatal pustular melanosis, infantile acropustulosis and Langerhans' cell histiocytosis. Skin smears and occasionally skin biopsy may be necessary to reach a diagnosis. We report a case of a Caucasian child with an unusually early onset of EPF in the first day of life. We wish to emphasize the importance of recognizing this self‐limiting condition in order to prevent inappropriate antimicrobial treatment.

Hernández‐Núñez, A.; Daudén, E.; Moreno de Vega, M. J.; Fraga, J.; Aragüés, M.; García‐Díez, A.

doi: 10.1046/j.1365-2230.2001.00808.xpmid: 11422168

We report a patient who presented with an intensely pruritic rash, with hyperpigmentation of the whole back and multiple hyperpigmented and hyperkeratotic papules over his forearms and lower extremities. Histologically, he showed deposits of amyloid that reacted positively with monoclonal antibodies against cytokeratins. The lesions were resistant to antihistamines and corticosteroids. Treatment with oral acitretin was started and great improvement of the lesion was noted; the patient became asymptomatic. We review the literature on the effect of retinoids in cutaneous amyloidosis.

Patel, G. K.; Anstey, A. V.

doi: 10.1046/j.1365-2230.2001.00809.xpmid: 11422169

Rifampicin has been prescribed throughout the world for over 20 years, yet only four cases of rifampicin‐induced lupus erythematosus (LE) have been reported. Rifampicin‐induced LE is associated with combination therapy with clarithromycin or ciprofloxacin. These drugs are all metabolized through the cytochrome P450 liver enzyme system and combined usage may lead to higher rifampicin blood levels. Drug‐induced LE differs from systemic LE; cutaneous manifestations, although uncommon, are an important clue to the diagnosis. We report a case of rifampicin‐induced LE presenting with florid cutaneous features.

Helbling, I.; Tucker, S. C.; Chalmers, R. J. G.

doi: 10.1046/j.1365-2230.2001.00810.xpmid: 11422170

We report a man with a 3‐year‐history of a striking eruption of small, 2–3 mm diameter keratotic, crateriform papules distributed symmetrically over the lower limbs. Morphologically the individual papules closely resembled those described in acrokeratoelastoidosis (AK) of Costa and in focal acral hyperkeratosis of Dowd (FAH). The case is unusual, however, in that there was dramatic involvement of the legs but the hands and feet were largely spared. Current terms used for these entities are unsatisfactory.

Papadavid, E.; Yu, R. C.; Katsambas, A.; Koumantaki, E.; Chu, A. C.

doi: 10.1046/j.1365-2230.2001.00811.xpmid: 11422171

We present a case of the aggressive variant of African endemic Kaposi's Sarcoma (AKS) which presented as a large fungating and ulcerated plantar mass. Our patient responded extremely well to chemotherapy with no recurrence for 9 months after treatment completion. AKS is one of the most common cutaneous neoplasms in black Africans and although rare in Europe, it may be seen more frequently in the future because of the ease of international travel. The existing classification of KS into five different types (classic, African‐endemic, iatrogenically immunocompromised patients, epidemic HIV‐related and Mediterranean‐endemic) cannot address fully the many anomalies described in the disease. The detection of a new herpes simplex‐like viral DNA sequence (HHV‐8) in different types of KS helps to explain some of the enigma described in this disease.

Gruber, P. C.; Fuller, L. C.

doi: 10.1046/j.1365-2230.2001.00812.xpmid: 11422172

Lipoatrophy semicircularis is thought to be a rare condition characterized by band‐like horizontal depressions of the skin typically involving the lower limbs. Previous literature has suggested that repetitive trauma to the lower limbs could explain this condition; however, no direct causal link has ever been clearly established. There have also been several reports where no preceding history of trauma could be found. In our series we report seven cases of lipoatrophy semicircularis representing over one‐third of the staff in a particular office. We propose that the most logical explanation for the indentations present in these individuals is repetitive trauma to their thighs by the sharp edge of the desks. The consistent nature of the distance between the floor and the horizontal indentations on the lower limbs despite differences in height weight and body mass index support this theory. It is likely that this condition is more common than initially thought.