Caterina Ferreli

Cutaneous adverse drug reactions to allopurinol: 10 year observational survey of the dermatology department – Cagliari University (Italy)

Background  Allopurinol is extensively prescribed for conditions associated with urate excess, despite being responsible for severe cutaneous adverse drug reactions (ADR).

Cutaneous Crohn Disease in a Child

Abstract:  Cutaneous Crohn disease refers to granulomatous skin manifestations not contiguous with gastrointestinal affected areas. It is a very rare condition, especially in children, and is easily misdiagnosed when characteristic gastrointestinal symptoms are absent. We report a 10‐year‐old Caucasian girl with a 6‐month history of erythematous, firm tumescence of the left labium majus pudendi and moist vegetations circumscribing the anal ostium. Histologic analysis of skin biopsy specimens from both types of lesions showed a characteristic granulomatous noncaseating infiltrate throughout the dermis. Endoscopic examination and a colon biopsy specimen showed chronic granulomatous inflammation consistent with Crohn disease. Treatment with prednisolone 20 mg daily, metronidazole 250 mg three times daily, topical corticosteroids, and mupirocin ointment produced marked improvement of the vulvar edema, whereas the perianal lesion had a relapsing course. Early recognition of extra‐intestinal manifestations of Crohn disease, which are extremely rare in children, may be difficult. Coexistence of contiguous and noncontiguous lesions, representing the very first signs of the disease, are further peculiarities in our patient.

Local immune response in the skin of the external auditory meatus: An immunohistochemical study

Cerumen plays an important role in the protection of the external auditory meatus against several kinds of damage. Its hydrophobic properties, due to the high concentration of lipids, shelter the canal from physical damages, while other components probably protect against certain microbial strains. Nevertheless there has been considerable dispute in the literature with regard to the antibacterial activity of cerumen. Because of the importance of the role of immunoglobulins (Ig) in local defense mechanisms, we attempted to study, by immunohistochemical methods, the presence and localization of the cells necessary to activate an Ig‐mediated immune response and the epithelial expression of immunoglobulin A (IgA), immunoglobulin M (IgM), and immunoglobulin G (IgG) in order to obtain information about a local immune response in those areas of the skin that take part in cerumen production. Our findings indicate that in the human skin of the external auditory canal the cells necessary to activate an antibody‐mediated immune response were localized in the different layers of the epidermis and/or in the dermis surrounding the sebaceous and ceruminous glands and the piliary follicle, while an intense immunoreactivity for IgA and IgG was observed in the epithelial layers of the skin. The results suggest that the external auditory canal is protected from the insults of pathogens by an antibody‐mediated local immune response, because all the effector components of an active local immune system are present. Microsc. Res. Tech. 38:329–334, 1997.

Leishmaniasis of the lip in a patient with Down's syndrome

Cutaneous leishmaniasis is an endemic protozoan infection in Sardinia, one of the major islands of the Mediterranean Basin. The main causative strain in this country is Leishmania infantum, which rarely involves mucocutaneous areas, but has the potential to cause visceral leishmaniasis. An atypical leishmaniasis involving the inferior lip of a 57‐year‐old female with Downs syndrome was observed at the Dermatology Department of Cagliari (italy). The diagnosis was mainly based upon histopathological examination, revealing intra‐ and extra‐cellular leishmania amastigotes. The leishmania infantum zymodeme MON‐111 was identified by isoenzymatic characterization. Laboratory investigations revealed a normal complete blood count and biochemistry profile, except for an inverted CD4/CD8 ratio. Treatment with meglumine antimoniate 60 mg/kg/day (Glucantime®) intramuscularly for 15 days, followed by intralesional administration 1 ml weekly for 4 weeks led to complete recovery. No relapses were observed at 6‐month follow‐up. The unusual localization is likely to be a reflection of the uncommon site of inoculation of the protozoa, transmitted by bites from flying vectors. Nevertheless, the presence of Downs syndrome in our patient may have contributed to the atypical presentation by traumatic exacerbation of the lesion, due to repeated auto‐induced microtraumas of the inferior lip accompanied by subclinical immunodeficiency. In fact, the specific immune response to Leishmania infection depends on a host‐cell‐mediated immune response, reported as defective in Downs syndrome patients. Differential diagnosis and early detection of the infection are necessary in order to start effective treatment and prevent more serious complications.

Lichen planus in a child requiring circumcision.

Abstract: Lichen planus is a rare disorder in infants and children where it usually has the classical cutaneous pattern and only exceptionally involves the mucosa and skin appendages. A 9‐year‐old boy was referred to our department with a 4‐month history of erythematous keratotic papules on the trunk and the upper and lower limbs. There were no signs of mucosal involvement. Subsequently white papules and striae, occasionally forming a lace like pattern, appeared on the penis and prepuce which quickly led to phimosis necessitating circumcision. Histologic examination of skin biopsy specimens and genital mucosa fragments confirmed the clinical diagnosis of lichen planus.

Drug‐induced sweat gland necrosis in a non‐comatose patient: a case presentation

Background Coma‐induced bullae and sweat gland necrosis is a rare clinicopathological entity often associated with drug‐induced coma.

Classic Kaposi's sarcoma in southern Sardinia, Italy

The first examination of classical Kaposis sarcoma incidence in southern Sardinia (Italy) in 1998–2002 found the highest rate recorded in the island of 2.49 per 100 000 per year (standardised).

Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review

Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. This article reviews the clinical presentation with emphasis on cutaneous disease, etiopathogenesis, diagnosis, and treatment options available for the different forms of scleroderma firstly and for scleroderma-like disorders, including scleromyxedema, scleredema, nephrogenic systemic fibrosis, eosinophilic fasciitis, chronic graft-versus-host disease, porphyria cutanea tarda, diabetic stiff-hand syndrome (diabetic cheiroartropathy), and other minor forms. This latter group of conditions, termed also scleroderma mimics, sclerodermiform diseases, or pseudosclerodermas, shares the common thread of skin thickening but presents with distinct cutaneous manifestations, skin histology, and systemic implications or disease associations, differentiating each entity from the others and from scleroderma. The lack of Raynaud’s phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies is also important diagnostic clues. As cutaneous involvement is the earliest, most frequent and characteristic manifestation of scleroderma and sclerodermoid disorders, dermatologists are often the first-line doctors who must be able to promptly recognize skin symptoms to provide the affected patient a correct diagnosis and appropriate management.

Amniotic membrane transplantation in the surgical management of symblepharon following toxic epidermal necrolysis

Toxic epidermal necrolysis (TEN) is a rare, potentially life-threatening acute condition, whose pathophysiology is not completely understood, but there is a certain agreement that it consists of a cell-mediated cytotoxic reaction against keratinocytes, altered by drugs or their metabolites. 1–3 Early recognition and withdrawal of the suspected drug are the main recommended procedures. 3 Therapeutic goals in the acute phase are directed toward promotion of wound healing, prevention of sepsis and management of multisystem failure. 4 In those patients who survive, ocular complications represent the most significant long-term sequelae and occurs in about 50% of cases. 5

Adverse cutaneous reactions to selective cyclooxygenase 2 inhibitors: experience of an Italian drug-surveillance center.

Background: Selective cyclooxygenase (COX) 2 nonsteroidal anti-inflammatory drugs (NSAIDs) have been associated with a general lower incidence of side effects compared with nonselective NSAIDs. Postmarketing information has highlighted the need to reassess the risk evaluation for specific organs, including the skin. Objective: A prospective databank to record all cases of adverse cutaneous reactions associated with the use of COX inhibitors was conducted at the Centre for Drug Surveillance of the Dermatology Department of Cagliari University. Material and Methods: An intensive surveillance program from November 2000 to October 2004, adopting the World Health Organization Collaborating Centre for Drug Monitoring causality assessment criteria and algorithm. Results: Seventeen cases, 4 male and 13 female, were studied. None had previously presented any drug intolerance or allergy. Clinical manifestations were mainly maculopapular exanthema followed by urticaria-angioedema. A severe case of leukocytoclastic vasculitis was also observed. Responsible drugs were celecoxib (13 cases; 76%), rofecoxib (3 cases; 18%), and etoricoxib (1 case; 6%). All cases recovered with drug withdrawal. Causality was probable for all eruptions, except for the fixed drug eruption, for which causality was certain. Discussion: Although most cases were associated with celecoxib, the observation of severe eruptions owing to rofecoxib and etoricoxib in this prospective study is consistent with a class effect of COX inhibitors on the skin, which merits further studies to explain the fine underlying mechanisms.