Atopy as a risk factor for thyroid autoimmunity in children affected with atopic dermatitis.

BACKGROUND: As a result of several clinical reports addressing coincidence or coprevalence of atopy and autoimmune disease such as multiple sclerosis and type I diabetes mellitus, there has been considerable interest in defining the relationship between the expression of allergic and autoimmune disease in populations of patients. Although thyroid autoimmunity has been regularly associated with chronic urticaria in children, the cofrequency of thyroid autoimmunity and atopic dermatitis has not yet been investigated. The aim of the study was to describe our experience with children affected by atopic dermatitis and associated thyroid autoimmunity. METHODS: From January 2010 to December 2012, 147 children affected by atopic dermatitis were consecutively referred to the Pediatric Clinic of the Pediatric Department at the Second University of Naples. Seventy healthy children of comparable ages, unaffected by atopic dermatitis, atopy or thyroid disease, served as a control group. RESULTS: On the basis of skin prick test results we selected 54 IgE-mediated (36.7%) and 93 non-IgE-mediated AD (63.3%) children. Fourteen of 147 patients (9.52%) showed increased levels of antithyroid antibodies. CONCLUSIONS: Our results therefore suggest that atopy, especially food allergy, and autoimmunity are two potential outcomes of dysregulated immunity.

Opportunistic metastatic porocarcinoma after saphenous venectomy for coronary bypass surgery.

Immunocompromised areas of the skin, caused by chronic lymphoedema, paraplegia, infections or traumas, represent a site of regional neuroimmunocutaneous destabilization, termed the immunocompromised cutaneous district (ICD), in which malignancies and other opportunistic disorders are more likely to occur. We report the case of a metastatic porocarcinoma (PC) occurring on a lymphoedematous limb in a 72-year-old man. We reviewed the literature to better understand the potential pathogenetic mechanisms behind this condition. It has been reported that removal of the leg vein destroys the medial group of the superficial lymphatic vessels and alters the normal lymph drainage of the leg, predisposing to recurrent cellulitis. Our observations suggest that saphenous venectomy can induce development of an ICD. We suggest that PC, a rare cutaneous tumour, should be included in the growing list of tumours arising in the ICD.

The practical use of cytology for diagnosis in dermatology.

Exfoliative cytology for diagnostic purposes is rarely used in Dermatology despite the rapid and reliable results which this procedure can offer in many clinical conditions. This simple procedure may prove advantageous in a wide range of skin diseases, including genodermatoses (Hailey-Hailey disease), infections (mainly herpetic infections, molluscum contagiosum, leishmaniasis), immune disorders (early oral pemphigus) and tumours (basal and squamous cell carcinomas, Paget disease, erythroplasia of Queyrat, and others). The specific circumstances where cytological examination provides a very helpful and practical aid to confirmation or exclusion of a clinically suspected diagnosis are briefly reviewed. Cytological patterns, along with some technical hints on how to take and stain Tzanck smears correctly, are described in connection with the diseases considered.

Cutaneous leishmaniasis treated with itraconazole.

Leishmaniasis is a human disease produced by a parasite of the Leishmania genus transmitted by prick of an infected female sandfly. The disease occurs clinically with either cutaneous, mucocutaneous or visceral form, depending on the infective species and the immune status of the patient. Antimonial drugs are the current treatment of choice for all clinical forms. We report a case of cutaneous Leishmaniasis in a young girl successfully treated with itraconazole.

Tinea capitis mimicking tufted hair folliculitis.

We report a case of tinea capitis mimicking tufted hair folliculitis in a 56-year-old European man, who presented with a 4-year history of pain and erythema in an area of scarring alopecia of the occipital scalp, with scales and tufts of hair emerging from individual follicles. Histological examination showed hair plugging, and a dense perifollicular infiltrate of plasma cells, lymphocytes, and neutrophils. There was widespread scarring and fibrosis. Bacterial cultures were negative for Staphylococcus aureus, but fungal cultures and periodic-acid-Schiff stain were positive for Trichophyton tonsurans. Videodermatoscopy of the lesion showed a pattern consistent with folliculitis decalvans. Diagnosis was made on the basis of the clinical, histological, microbiological and videodermatoscopy data. After 30 days of systemic antifungal treatment, there were a substantial clinical improvement and disappearance of pain. After 5 months, a residual cicatricial area was seen with some hair tufts emerging from a single orifice.

Cefuroxime-induced pemphigus erythematosus in a young boy.

Pemphigus erythematosus (Senear-Usher syndrome) is a variant of superficial pemphigus with features of both lupus erythematosus and pemphigus. It affects mainly middle-aged adults, and is rarely observed before the age of 20 years. The case of a 14-year-old boy who showed cutaneous lesions suggestive for pemphigus erythematosus is described. Not all laboratory and histopathological investigations confirmed the hypothesis, so a diagnosis of clinical pemphigus erythematosus was made. Systemic steroid therapy was effective in controlling the disease. This case is interesting because of the rare occurrence of pemphigus erythematosus in adolescence and the possibility of another drug being added to the list of pemphigus inducers.

The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites.

Systemic immunodeficiency is known to facilitate the onset of opportunistic infections, tumours and immune disorders in any district of the body. There are clinical events, such as chronic lymphoedema, herpetic infections, vaccinations and heterogeneous physical injuries which can selectively damage and immunologically mark the cutaneous district they act upon. After the causing event has disappeared, the affected district may appear clinically normal, but its immune behaviour is often compromised forever. An immunocompromised district becomes a site which is particularly susceptible to subsequent outbreaks of opportunistic infections, tumours and immune disorders confined to the district itself. In this review, there is an ample case-report collection of opportunistic disorders (infectious, neoplastic, immune) which appeared in immunocompromised districts. The cases have been grouped according to the clinical settings responsible for the local immune imbalance: regional chronic lymphoedema; herpes-infected sites, which feature the well-known Wolf's isotopic response; and otherwise damaged areas, comprising sites of vaccination, ionizing or UV radiation, thermal burns and traumas. Whatever the immunocompromising factor, a common denominator which facilitates the occurrence of tumours, infections and dysimmune reactions in an immunocompromised district may reside in locally hampered lymph drainage and/or locally altered neuromediator signalling. In fact, any obstacle to the normal trafficking of immunocompetent cells through lymphatic channels or any interference with the signals that the neuropeptides and neurotransmitters released by peripheral nerves send to cell membrane receptors of immunocompetent cells, can significantly alter the local immune response, thus paving the way for heterogeneous opportunistic disorders in the immunocompromised district.

Pyoderma gangrenosum: an updated review.

Pyoderma gangrenosum is a rare, ulcerative, cutaneous condition. First described in 1930, the pathogenesis of pyoderma gangrenosum remains unknown, but it is probably related to a hyperergic reaction. There are various clinical and histological variants of this disorder. Pyoderma gangrenosum often occurs in association with a systemic disease such as inflammatory bowel disease, rheumatologic disease, paraproteinaemia, or haematological malignancy. The diagnosis, mainly based on the clinical presentation and course, is confirmed through a process of elimination of other causes of cutaneous ulcers. Local treatment may be sufficient for mild disease, while for severe cases, systemic immunosuppressants are the mainstay. Long-term treatment with these agents is often required, but this can expose patients to adverse side-effects.

Pemphigus vulgaris after coxsackievirus infection and cephalosporin treatment: a paraviral eruption?

Pemphigus is an autoimmune disease that results from the interaction between predisposing genetic factors and exogenous agents, mainly drugs and viruses. Herein we report the case of a 66-year-old woman referred to our department for the onset of painful oral erosions and bullous lesions on the torso. Clinical, laboratory and histopathological investigations led to the diagnosis of pemphigus vulgaris. Two weeks before the outbreak of the lesions, the patient had suffered from a viral pharyngitis, subsequently diagnosed as herpangina, and had been taking an oral cephalosporin (cefixime) for 1 week to prevent possible bacterial complications. A relationship between the onset of pemphigus and coxsackievirus infection or cefixime administration or both was supposed. The case may represent a peculiar paraviral eruption, where a predisposing pemphigus-prone genetic background paved the way for the acantholytic autoimmune disorder as a consequence of the combined effect of the coxsackievirus infection and the cephalosporin treatment.

Tannic acid induces in vitro acantholysis of keratinocytes via IL-1alpha and TNF-alpha.

The mechanism of acantholysis in pemphigus vulgaris (PV) is an intriguing argument since several chemical mediators are implicated. We previously reported a central role for IL-1alpha and TNF- alpha, both able to regulate complement activation and plasminogen activators. Very little is known about what triggers the disease (drugs, viruses or food). In this study, we evaluate the molecular role of tannins in acantholysis. By HPLC chromatography we measured tannic acid (TA) and gallic acid (GA) in blister fluid of 4 groups of patients divided according to their dietary habits, including a regular diet, a diet rich in tannins, a diet free of tannins, and a group of pemphigus patients. Blister fluid was obtained from patients using a suction blister apparatus. We show that people with a diet rich in tannins have increased tannin metabolites (TA and GA) in the skin in respect to controls (tannin-rich diet: GA = 194.52+/-2.39 nmol/ml; TA = 348.28+/-1.4 nmol/ml versus tannin-Mediterranean diet: GA = 15.28+/-1.63 nmol/ml; TA = 22.81+/-1.68 nmol/ml). PV patients showed similar values to the Mediterranean diet population (PV patients: GA = 95.8+/-1.97 nmol/ml; TA = 199.09+/-4.15 nmol/ml versus Mediterranean diet: GA = 83.53+/-2.35 nmol/ml; TA = 195.1+/-2.50 nmol/ml). In an in vitro acantholysis system using TA and PV-IgG we show that TA 0.1 mM in NHEK culture is able to induce acantholysis. This effect was able to amplify the acantholytic action of PV-IgG in vitro. A blocking study using anti IL-1 alpha and anti TNF-alpha antibodies showed a reduction in TA-induced acantholysis. Taken together, these results suggest that a diet rich in tannins could be a trigger in genetically predisposed patients. If these data are confirmed, a complementary diet poor in tannins may be useful in patients affected by PV.

Internalization of non-clustered desmoglein 1 without depletion of desmoglein 1 from adhesion complexes in an experimental model of the autoimmune disease pemphigus foliaceus.

Serum antibodies against desmoglein 1 (Dsg1) are known to induce the clinical and histological manifestations of pemphigus foliaceus (PF), autoimmune bullous disease targeting skin. The basic pathophysiological phenomenon of PF blistering is the disruption of epithelial integrity in the granular layer of the epidermis due to separation of keratinocytes from one another, or acantholysis. In this report we investigate the changes in subcellular distribution of Dsg1 in response to serum of patients with PF by using an in vitro model of PF. Immunofluorescence analysis on HaCaT cells indicates that non-clustered Dsg1 is markedly internalized after exposure to serum. However, binding of PF IgG to Dsg1-rich adhesion complexes (desmosomes) does not cause disruption of such structures nor depletion of clustered Dsg1, as revealed by colocalization of PF IgG and Dsg1 in a punctate staining on cell membrane 24 hours after treatment. Furthermore, morphological studies demonstrate that the dramatic alterations induced by PF sera are not the result of apoptotic programs. Taken together, our data strongly suggest that anti-Dsg1 antibodies from PF serum could cause the internalization of non-clustered Dsg1 and perturb the formation of new desmosomes but not directly disrupt Dsg1-containing junctions when stable contacts are already formed.

Crohn's disease and skin.

Crohn's disease is a chronic inflammatory bowel disease potentially involving any segment of the gastrointestinal tract. Extra-intestinal manifestations may occur in 6%-40% of patients, and disorders of the skin are among the most common. This manuscript will review skin manifestations associated to Crohn's disease, with a particular focus on lesions associated to anti-tumour necrosis factor therapy.