HYPONYCHIUM ABNORMALITIES Congenital Aberrant Hyponychium vs. Acquired Pterygium Inversum Unguis vs. Acquired Reversible Extended Hyponychium: a proposed classification based on origin, pathology and outcome.

BACKGROUND: Pterygium Inversum Unguis (PIU) is a wing-like extended hyponychium associated with the absence of the distal groove. Although a rare condition, it has been described with different names, confusing both the investigator and the reader. OBJECTIVE: We propose a new nomenclature based on tissue origin and pathology, to account for these conditions. 1) Congenital Aberrant Hyponychium 2) Acquired Pterygium Inversum Unguis 3) Acquired Reversible Extended Hyponychium. MAIN OBSERVATIONS: We report a case of a 19-year-old male, with epidermal pigmentation abnormalities, who had painful fingertips of both index fingers and thumbs since he was 13. He therefore let his finger nails grow very long, minimizing painful contact with the hyponychium. Removal of the aberrant hyponychium revealed glomus bodies aggregates with increased nerve fibers. Subsequently after excision of the hyponychium, his pain was resolved. SUMMARY: Congenital, transient or permanent changes in the hyponychium should be named and classified according to tissue origin to avoid nomenclature confusion.

Diffuse dermal angiomatosis: a clinical mimicker of vasculitis associated with calciphylaxis and monoclonal gammopathy.

Diffuse dermal angiomatosis is a form of cutaneous reactive angiomatosis characterized clinically by painful erythematous or violaceous lesions with ulcers that may mimic cutaneous vasculitis/vasculopathy. Histologically it shows a benign, diffuse proliferation of endothelial cells with tiny blood vessels in the papillary and reticular dermis. Herein, we report four patients with diffuse dermal angiomatosis in the setting of calciphylaxis and monoclonal gammopathy and review the cases previously published in the literature. Comorbidities and management will also be discussed.

D2-40 highlights lymphatic vessel proliferation of angiolymphoid hyperplasia with eosinophilia.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign, uncommon idiopathic condition, characterized by cutaneous papules or nodules, whose etiopathogenesis is still unclear. It has been considered an angioproliferating lesion (epithelioid hemangioma) since histologically it is marked by a proliferation of blood vessels, accompanied by an inflammatory infiltrate, consisting mainly of lymphocytes and eosinophils. We present a case of ALHE assessed immunohistochemically for D2-40-a new marker for lymphatic endothelial cells. A biopsy specimen obtained from the same anatomical area of a healthy individual served as a normal control. The ALHE specimen showed increased number of lymphatic vessels when stained for D2-40, whereas the endothelial cells lining blood vessels were negative. The specificity of D2-40 for lymphatic vessels was further substantiated by studying Factor VIII-related antigen expression in consecutive sections of both ALHE and the control specimen. A reverse pattern was appreciated-blood vessels showed Factor VIII positive labeling, whereas lymphatic endothelial cells remained unlabeled. We therefore assume that apart from the lymphocytic infiltrate in the lesion, the recognized lymphoid component in ALHE is due to lymphatic vessel proliferation as well. Hence, this condition may be considered as possibly derived from lymphatic endothelium.

Immunohistochemical evaluation of toxic epidermal necrolysis treated with human intravenous immunoglobulin.

AIM: Toxic epidermal necrolysis (TEN) is a severe drug reaction characterized by massive epidermal cell death. The authors of the current study and others have noted improved outcomes in TEN patients treated with human intravenous immunoglobulin (IVIG), purportedly due to its ability to inhibit the fas/fas-ligand (Fas-L) apoptotic pathway, but published case series evaluating TEN through the use of immunohistochemical antibody stains for Fas and Fas-L before and after IVIG treatment are lacking. The authors hypothesized that due to IVIG's ability to arrest the evolution of TEN, expression of Fas/Fas-L on keratinocytes would be decreased or absent following IVIG treatment. METHODS: Ten patients diagnosed with TEN underwent biopsies of their lesions prior to and five days after treatment with IVIG. Seven post-treatment biopsies were of sufficient quality to undergo evaluation. RESULTS: All ten pretreatment biopsies had Fas and Fas-L expression by immunohistochemistry, while six out of seven (85.7%) post-treatment biopsies failed to demonstrate Fas or Fas-L expression. One of seven post-treatment biopsies stained positive for Fas and Fas-L. CONCLUSION: This reduced immunohistochemical expression of apoptotic markers may represent IVIG inhibition of the pathogenic mechanism of TEN. Alternatively reduced Fas and Fas-L may be a feature of reepithelialization in TEN, or characteristic of rapidly proliferating epidermis.

Colitis induced by nonsteroidal anti-inflammatory drugs. Report of four cases and review of the literature.

In four patients, ulcerative disease of the colon developed while they were taking nonsteroidal anti-inflammatory drugs (NSAIDs). Clinical presentation and laboratory and colonoscopic findings were similar to those of inflammatory bowel disease. Review of the literature disclosed 74 additional cases of NSAID-induced colitis in adults. This is a rare but serious, sometimes fatal, complication of NSAID therapy. The elderly and those taking long-term NSAID therapy appear to be at highest risk. The pathogenesis is thought to be related to inhibition of intestinal prostaglandin synthesis. Whether some NSAIDs are more likely to induce colitis than others is not known. Since NSAIDs are so widely prescribed and some are available without a prescription, heightened awareness by physicians and the lay public will be important in reducing injury from this disease, both by prevention and earlier recognition.

[2 cases of Kawasaki disease with increase of beta-2 thromboglobulin]. / Due casi di malattia di Kawasaki con aumento della beta-2 tromboglobina.

The authors report two cases of Kawasaki disease, in which both the classic symptomatology and an increase in the beta 2 thromboglobin were present. The antigens of histocompatibility do not appear similar either to those which are more frequent in the Japanese population or in the patients suffering from arterial occlusive disease and Takayasu arteritis. The prognosis for these patients appears to be favourable: vascular and heart damages have not been shown. The therapy by means of antiaggregating agents has given good results showing a regression of the symptoms and a normalization of the laboratory tests within a few months.