Pyoderma gangrenosum, acne and suppurative hidradenitis syndrome following bowel bypass surgery.

The clinical triad of pyoderma gangrenosum (PG), acne and suppurative hidradenitis (PASH) has recently been described as a new disease entity within the spectrum of autoinflammatory syndromes, which are an emerging group of inflammatory diseases distinct from autoimmune, allergic and infectious disorders. PASH syndrome is similar to PAPA (pyogenic arthritis, acne and PG), but it differs in lacking the associated arthritis and on a genetic basis. PAPA syndrome is caused by mutations in a gene involved in the regulation of innate immune responses, the PSTPIP1, while no mutations have been detected to date in patients with PASH syndrome. We report a young male patient who developed coexisting disseminated PG, typical suppurative hidradenitis and acneiform eruption on the face, after he had undergone bowel bypass surgery for obesity. The cutaneous manifestations associated with bowel bypass syndrome often mimic PG or other neutrophilic dermatoses, suggesting a pathogenesis related to neutrophil-mediated inflammation for this condition. This is the first report describing PASH syndrome after bariatric surgery, and we propose to include such neutrophilic dermatoses in the list of complications occurring after bowel bypass surgery. Extensive genetic studies may help to clarify the etiopathogenesis of PASH as well as of autoinflammatory diseases in general.

Tomesa balneophototherapy in mild to severe psoriasis: a retrospective clinical trial in 174 patients.

Psoriasis is a chronic inflammatory skin disease with a high social and psychological impact on the quality of life of patients. Tomesa balneophototherapy is based on bathing in a magnesium-rich salt solution combined with exposure to narrowband ultraviolet B phototherapy. We conducted a retrospective clinical trial on 174 patients affected by mild to severe psoriasis undergoing Tomesa balneophototherapy. The basal course consisted of three to five sessions per week for a total of 30 sessions. Subsequently, patients could continue with a maintenance course of one session per week for a total of 30 sessions. We recorded a significant reduction of the mean Psoriasis Area and Severity Index (PASI) index with an achievement of at least PASI 75 in 52.1% of the 119 patients who completed the basal course and an improvement of the 'quality of life' of patients. The good efficacy obtained by this treatment, and the psychological impact on the quality of life of patients, demonstrated that Tomesa balneophototherapy could be a good option for the treatment of a chronic disease associated with psychological distress, like psoriasis.

Cutaneous T-cell/histiocyte-rich B-cell lymphoma: a case report and review of the literature.

BACKGROUND: T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) primarily presenting on the skin is an extremely rare entity with only sporadic cases reported in the literature. METHODS: We here report an extraordinary case of primary cutaneous THRLBCL with self-healing and 24 months of follow-up. RESULTS: The lesion was a dermohypodermal/subcutaneous circumscribed ulcerated nodosity. Histological examination with immunohistochemical, molecular analysis and comparative genomic hybridization were performed. A complete staging was negative for secondary involvement. CONCLUSION: Our case is remarkable because it is the second well-documented primary cutaneous THRLBCL in which we observed a complete self-regression of skin lesions without recurrences or dissemination of the disease. According to the literature, we highlight that the tumoral microenvironment, in our case, could play a relevant role in stopping lymphoma growth. Furthermore, this case supports the observation that THRLBCL primarily presenting on the skin shows an overall good prognosis.

Anatomic location and histopathologic subtype of basal cell carcinomas in adults younger than 40 or 90 and older: any difference?

BACKGROUND: Differences in age, site, and histopathologic subtype exist in basal cell carcinoma (BCC). OBJECTIVE: To compare the distribution of BCCs in patients younger than 40 with that of those aged 90 and older according to sex, site, and subtype. METHODS & MATERIALS: One hundred seventy-five BCCs were examined. The site was classified as head and neck, trunk, or limbs and the subtype as nodular, superficial, or morpheic-infiltrative. RESULTS: Younger exhibited a lower prevalence of BCCs on the head and neck (36.0% vs 57.3%, p<.01) and a higher prevalence on the trunk (59.3% vs 31.5%, p<.01) and of superficial BCCs (43.0% vs 31.5%, p<.05) than older patients. Site was associated with subtype in younger (p<.001) and older (p=.004) patients. Superficial BCCs were mostly on the trunk (p<.001), with a higher prevalence in younger patients (86.5% vs 62.5%, p<.05). Morpheic BCCs were mostly on the head and neck (p<.001), and prevalence did not differ between age groups. Nodular BCCs were mostly on the head and neck in older patients (p=.011). Subtype was independently associated with site (p=.005) but not with age or sex. CONCLUSION: A different distribution of site and subtype occurs in younger and older patients. Subtype is associated with site independent of age and sex. These findings suggest that, at least in some patients, the anatomic location of BCC may favor the development of a particular subtype.

Nodular melanomas: analysis of the casistic and relationship with thick melanomas and diagnostic delay.

The present study aimed to: (i) define thick melanomas related to nodular melanomas and other melanoma subgroups; and (ii) establish diagnostic delay in relation to the biological behavior of these melanomas and prevention programs. Cutaneous primary melanomas were studied. Nodular melanoma (NM), lentigo maligna melanoma (LMM) and superficial spreading melanoma (SSM) were selected. A further category named vertical growth melanoma (VGM) was also utilized. Analysis for sex, age, different values of thickness (1-2 mm, > 2 mm; 1-3 mm, > 3 mm; > 4 mm), delay to diagnosis and patterns of detection were performed in all of the different subtypes. Eighty-seven patients with melanomas more than 1 mm of Breslow's thickness out of 506 melanoma were collected. Twenty-six were nodular cases, 39 SSM, five LMM and 17 VGM. Of those patients with NM, 42% had a thickness of more than 1-2 mm, 34% of 2-4 mm, 23% of more than 4 mm; and 54% with 1-3, 46% with more than 3 mm; and 58% with more than 2 mm. Even considering different values of thickness of more than 1 mm, a delay to diagnosis was significantly lower in NM (4.79 months) than in other subgroups. The value of more than 1 mm of Breslow's thickness may be sufficient to consider a melanoma to be thick. The lower diagnostic delay of NM suggests that they represent faster growing lesions probably with a different biological behavior than other melanoma subtypes. VGM should not be confused with NM, having a longer delay and different clinical features compared with the latter. They represent an area of diagnostic carelessness than potentially be improved.

Melanoma of the glans penis.

The authors describe the case of a 64-year old man who presented with an asymptomatic brown macula on his glans penis that had appeared about 18 months earlier. Dermoscopy analysis demonstrated a prominent, wide and irregular pigment network, which stopped abruptly at the periphery of the lesion. A diagnostic biopsy showed the characteristics of a melanoma in situ . The patient was referred for partial surgical excision of the glans. No recurrence or metastasis occurred during the two years after the operation. Melanoma in situ of the penis is very rare in dermatologic literature. Early diagnosis is of paramount importance because its prognosis is very poor. Early systematic use of dermoscopy may be useful for the differential diagnosis of pigmented mucosal lesions, which include mucosal melanosis and other benign melanoses of genitalia.

Basal cell carcinoma of the sole.

We report a case of a 72-year-old woman with a two year history of a plantar lesion. The clinical appearance, a nodular reddish irregular mass about 2 centimeters in diameter, suggested a tumoral lesion. Histologic examination revealed a basal cell carcinoma. The lesion was excised with no recurrence after two years of follow up. The clinical events, and the histological findings are briefly discussed.

[Neonatal toxic erythema : clinico-epidemiologic characteristics and recent pathogenic hypothesis]. / Eritema tossico del neonato: caratteristiche clinico- epidemiologiche e recenti ipotesi patogenetiche.

Erythema toxic neonatorum (ETN) is a common, self-limiting condition of term infants, appearing particularly within the first 48 hours of life. ETN has a worldwide distribution, with a frequency variable from 19% to 72%. Erythematous macules, wheals, papules and pustules in varying combination occur, with crops of lesions waxing and waning and spontaneous resolution within hours to days. It's important the differential diagnosis from other pustular neonatal conditions, some of which are severe. The etiology of ETN remains unknown. Recent studies reveal an activation of immune cells in the lesions of ETN and a high presence of peptide antibiotics, suggesting that ETN is an inflammatory skin reaction to the microbial colonization at birth.

Vitamin D and skeletal health in autoimmune bullous skin diseases: a case control study.

BACKGROUND: The presence of hypovitaminosis D in patients with autoimmune bullous skin diseases, such as pemphigus vulgaris (PV) and bullous pemphigoid (BP), is debated. In a previous study we found an increased prevalence of vertebral fractures (VFx) and hypovitaminosis D in PV and BP patients. The present study extends the sample size of the previous one, for investigating the 25-hydroxyvitamin D (25OHVitD) levels in relation with the skeletal health and disease intensity in these patients. METHODS: The previous study was performed in 13 PV and 15 BP patients and 28 controls. Data from 39 additional patients (22 PV and 17 BP) were now added. Eventually, we studied 67 patients (35 PV, 32 BP, 51 females), aged 64.7 ± 16.9 years and 67 age- gender- and body mass index-matched controls. In all subjects, serum 25OHVitD, calcium and alkaline phosphatase (ALP) levels were measured, bone mineral density (BMD) was evaluated by Dual-energy X-ray. Absorptiometry at lumbar spine (LS) and femoral neck (FN) and the presence of VFx were ascertained by visual assessment from spinal radiographs. In patients, the disease intensity was evaluated by the autoimmune bullous skin disorder intensity score (ABSIS). RESULTS: As compared with controls, both PV and BP patients showed lower 25OHVitD (22.2 ± 11.1 vs 13.9 ± 8.3 ng/mL, p < 0.001 and 22.4 ± 14.9 vs 9.5 ± 7.7 ng/mL, p < 0.0001, respectively) and higher prevalence of severe hypovitaminosis D (22.9 vs 48.6%, p < 0.02 and 31.1 vs 75.0%, p < 0.0001, respectively) and VFx (28.6 vs 57.1%, p = 0.03 and 34.4 vs 62.5%, P = 0.02, respectively). In both PV and BP patients, LS and FN BMD did not differ from controls. In the whole patients' group, ABSIS score was inversely associated with 25OHVitD levels (R = -0.36, p < 0.005), regardless of age (ß = -3.2, P = 0.009). CONCLUSIONS: PV and BP patients have an increased prevalence of hypovitaminosis D and VFx. The extended study shows, for the first time, that the 25OHVitD levels are inversely associated with disease intensity and that VFx occur in spite of a not reduced BMD.

Factors of delay in the diagnosis of melanoma.

Delay in the diagnosis of melanoma was defined as the interval in months from the patient's report of first noticing a suspected lesion to the date of the removal of a histologically confirmed melanoma. 216 patients were included in our study. Total delay was subdivided in three components: patient, medical and referral delay. The major component of delay is due to the patient and the most important cause of it was lack of concern. Lesions in men were detected more frequently by family members, while in women melanomas tended to be self-detected. Nodular melanoma had lower delay but higher thickness. Amelanotic melanomas had a higher delay principally due to the physicians. A significative positive correlation between Breslow thickness and patient delay was observed. The visibility of the tumor and the educational or socio-economic status did not seem to improve early diagnosis. The observation about the shorter delays in thicker tumors lead us to think that educational campaigns should be more focused on recognition of changing or growing lesions than other signs of the ABCD rule and that nodular melanomas are quite different from superficial spreading melanomas.

Basal cell carcinomas of the areola-nipple complex: case reports and review of the literature.

Two white men 57 and 39 years old, and a 47-year-old white woman were seen with slowly developing papulo-nodular lesions of the areola-nipple complex. None of the patients presented with regional lymphadenopathy, history of trauma, or relevant sun-exposure. After excison of the mass, the histologic diagnosis of basal cell carcinoma was made. At two years of follow-up, no recurrence was evident. The low incidence of basal cell carcinoma in this particular site allows us to consider the areola-nipple complex location as unusual. Moreover, literature reports do not suggest that these BCCs have an increased potential for malignancy. The treatment options depend on the extension of the tumor and on the possible involvement of the areola-nipple complex and mammary tissue.

Cutaneous manifestations in patients with inflammatory bowel diseases: pathophysiology, clinical features, and therapy.

The skin is one of the most common extraintestinal organ system affected in patients with inflammatory bowel disease (IBD), including both Crohn's disease and ulcerative colitis. The skin manifestations associated with IBD are polymorphic and can be classified into 4 categories according to their pathophysiology: (1) specific, (2) reactive, (3) associated, and (4) induced by IBD treatment. Cutaneous manifestations are regarded as specific if they share with IBD the same granulomatous histopathological pattern: perianal or metastatic Crohn's disease (commonly presenting with abscesses, fistulas or hidradenitis suppurativa-like features) is the prototype of this setting. Reactive cutaneous manifestations are different from IBD in the histopathology but have close physiopathological links: pyoderma gangrenosum, a neutrophil-mediated autoinflammatory skin disease typically manifesting as painful ulcers, is the paradigm of this group. Among the cutaneous diseases associated with IBD, the most commonly seen are erythema nodosum, a form of panniculitis most commonly involving bilateral pretibial areas, and psoriasis, a T helper 1/T helper 17-mediated erythematous squamous inflammatory disease. Finally, the number of cutaneous adverse reactions because of IBD therapies is progressively increasing. The most frequent drug-induced cutaneous manifestations are psoriasis-like, eczema-like, and lichenoid eruptions, as well as cutaneous lupus erythematosus for biologics, and nonmelanoma skin cancer, mainly basal cell and squamous cell carcinomas for thiopurines.