Maurizio Rotoli

Rapid Regression of Psoriasis in a Coeliac Patient after Gluten-Free Diet A Case Report and Review of the Literature

Background: Several skin disorders are present in patients affected by coeliac disease (CD) – among them, psoriasis has been described. However, at present the relationship between CD and psoriasis remains controversial since there are few and contrasting data on this topic. Method: Here we describe a case of psoriasis in a CD patient not responding to specific therapies for psoriasis. Result: The regression of skin lesions after gluten-free diet (GFD) was evident in a short time. Conclusion: The present case supports the association between CD and psoriasis and the concept that psoriasis in CD patients can be improved by GFD. Future studies are needed to clarify the possible mechanisms involved in this association.

Lichenoid reaction induced by adalimumab

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Aspergillus versicolor infection of the external auditory canal successfully treated with terbinafine.

We report the case of a 58-year-old Italian woman who came to our attention with a 4-month history of hypacusia and itching of the right external ear canal. The patient did not suffer from diabetes and was not under medication. Otoscopic examination revealed the presence of a white-greyish, dense material plugging the external auditory canal. Ear toilet was performed and the patient was free of symptoms for a few days. Then the occlusion reappeared and material from the ear toilet of the auditory canal was sent for cultural examination. Aspergillus versicolor was isolated and a course of antifungal therapy with itraconazole was started (200 mg a day for 21 days). After suspension of the treatment the clinical picture recurred. A complete blood count, blood chemistry and T cell subsets were normal. A review of the literature [1, 2] led us to consider terbinafine as an active agent on A. versicolor. Treatment with systemic terbinafine 250 mg a day for 30 days and topical terbinafine cream twice a day for 2 months were prescribed. The clinical picture rapidly improved and at the end of the treatment the patient did not show any sign or symptoms of fungal infection. The patient is free of symptoms after a 14-month follow-up, during which she spent some months in the tropics, and no fungal material could be seen on otoscopy, direct microscopy and cultural examination. Otomycosis is a clinical picture which is common in the tropics and in warm moist environments. It is generally caused by fungi belonging to the genus Aspergillus (niger, fumigatus, flavus), Candida sp., Rhizopus, Actinomyces and Penicillum [3]. A. versicolor is an opportunistic mould which is not reported as an aetiological agent of mycosis of the external ear. It is a ubiquitous fungus which may be isolated from buildings contaminated by moulds [4]. Also, it produces metabolites involved in tumour induction [5]. Recent studies [1, 2] have reported A. versicolor as an emergent pathogen in onychomycosis. In vitro, this fungus is resistant to griseofulvin, fluconazole and amphotericin B (which is the drug of choice for Aspergillus spp. infection) and it is sensitive to itraconazole and ketoconazole, but their minimal inhibiting concentrations vary from 0.50 to 4.0 Ìg/ml. Terbinafine has shown to have a very low minimal inhibitory concentration (!0.125 Ìg/ml) and seems to be an effective agent on this opportunistic fungus.

Anaplastic progression of classic Kaposi's sarcoma.

We describe a case of classic Kaposis sarcoma (KS) in an Italian HIV-negative patient, with bone involvement and progression to anaplastic histotype. At the age of 22, violaceous patches of KS appeared on his feet. At the age of 50, he noted the appearance of a violaceous firm nodule on his right wrist. The lesions grew rapidly and became ulcerated. Radiotherapy led to a complete remission of symptoms. At the age of 55, a subcutaneous nodule developed on the proximal third of the right forearm associated with a wide painful edema of the right forearm and the proximal third of the right arm. The nodule enlarged rapidly, and an X-ray of the right forearm revealed the presence of a large osteolytic area of the ulna. A biopsy specimen from the right ulna showed bone erosion by a mesenchymal neoplasia with a high degree of malignancy. The right arm was amputated, and histologic examination of the surgical material confirmed the diagnosis of undifferentiated spindle-cell malignant neoplasia strongly positive for factor-VII-related antigen and CD34 antigen. Three years after surgical treatment, no recurrences have been observed.

Lymphoepithelioma-like carcinoma of the skin.

The term lymphoepithelioma-like carcinoma identifies a group of nasopharingeal epithelial tumors characterized by aggregates of malignant undifferentiated cells surrounded by a dense reactive lymphoplasmacellular infiltrate. Primary cutaneous localization is rare, with approximately 30 cases reported in literature. We describe a case of primary lymphoepithelioma-like carcinoma of the skin in a 92-year-old woman. Immunohistochemical examination was positive for cytokeratine (KL1 and EMA) as regards epithelial cells, while the lymphocitic infiltrate was positive for LCA and CD3. In situ hybridization for Epstein Barr virus in tumor cells was negative. Electron microscopy showed rounded and occasionally spindle-shaped poorly-differentiated squamous epithelial cells, and a lymphoid stroma consisting mostly of normal-appearing small lymphocytes. Examination of the nasopharynx did not show any tumoral mass and after a 7 years follow-up the patient is free of local and distant recurrences. This tumor affects people aged over 50 years and is localized to the face, but scalp, shoulder and forearm may be involved. Research of Epstein-Barr virus is always negative in this tumor, unlike nasopharingeal epithelial carcinoma. The differential diagnosis of lymphoepithelioma-like carcinoma of the skin may present some difficulties and includes squamous cell carcinoma. Lymphoepithelioma-like carcinoma of the skin is a malignant neoplasm which tends to relapse locally and has a moderate tendency to metastatize.

Non-Herlitz Junctional Epidermolysis bullosa without Hair Involvement Associated with BP180 Deficiency

Junctional epidermolysis bullosa (JEB) is a clinically and genetically heterogeneous recessively inherited blistering disease of the skin and mucous membranes due to impaired epithelial adhesion. In particular, defective expression of the 180-kD bullous pemphigoid antigen (BP180) has been correlated to a non-lethal (non-Herlitz) form of JEB, generalized atrophic benign epidermolysis bullosa (GABEB), characterized by widespread skin blistering healing with atrophy and by atrophic alopecia with onset in childhood. We report the case of a 33-year-old man suffering from a generalized blistering skin disorder since birth. He also presented nail dystrophy and tooth abnormalities. Mucosal involvement was limited to gingival erosion. Alopecia was absent and body, axillary and pubic hair were normal. Immunofluorescence analysis showed a markedly reduced expression of BP180, electron microscopy studies evidenced hypoplastic hemidesmosomes and Northern blot analysis confirmed a striking decrease in the amount of BP180 mRNA. The clinical features of our patient confirm that BP180 deficiency usually results in a non-Herlitz JEB form. However, the degree of skin, mucous membranes and hair involvement appears more variable and less typical than originally described for GABEB.

INTESTINAL MALABSORPTION AND SKIN DISEASES

Several skin manifestations were described in patients affected by intestinal disorders. The development of skin diseases in these patients could be related to the impairment of intestinal absorption and motility, other than to immunological and hormonal changes. The growing evidence of the association between skin disorders and intestinal diseases suggests that the skin could be considered the ‘mirror of the gut’.

Apparent Isolated Skin Relapse in Acute Monocytic Leukemia. A Case Report

A case of acute monocytic leukemia with an apparent isolated skin relapse is reported. The cutaneous involvement was associated with a morphological bone marrow remission but a cytogenetic relapse was present. Regression of the skin lesions was obtained with a protocol including daunoblastine, aracytin and thioguanine, but the patient relapsed and died a few months later without achieving another remission. The relation between cutaneous and medullary disease is discussed.

Treatment of dermatomyositis with intravenous immunoglobulin

A patient affected by dermatomyositis, who did not respond to conventional therapies (steroids alone and in association with immunosuppressive drugs), developed necrotic lesions in the axillary and antecubital folds. The lesions did not improve after increasing the steroid dose. Treatment with intravenous immunoglobulin (IVIG) at a dose of 1 g/kg daily for 2 successive days every 4 weeks was started. Ten days after the first infusion of IVIG the cutaneous lesions disappeared and in the following months the muscle symptoms improved as well. The patient was treated with ten cycles of IVIG and then at a maintenance dose of 1 g/kg over one day every third month. This treatment has been demonstrated to be effective, but it is necessary to underline the need for an accurate selection of patients. The cost of nine months of therapy for a patient of 55 kg in body weight is about ±41 350 (L. 100 000 000).

Zygomycotic cellulitis in a renal transplant recipient

Abstract Zygomycosis is a rare complication in immunosuppressed patients and is caused predominantly by species of Rhizomucor , Absidia and Rhizopus . Lungs and central nervous system localization are the most commonly observed, while skin localization has been observed only in 15% of the patients. We describe a case of zygomycotic cellulitis, with severe edema, inflammatory lesions and a nonimmune bullous eruption, in a renal transplant recipient.