Pure Bullous Pyoderma Gangrenosum, a Challenging Clinico-Pathological Diagnosis: Critical Literature Review with Emphasis on Diagnostic Criteria.

Pyoderma gangrenosum (PG) is an uncommon, idiopathic, neutrophilic dermatosis characterised by large necrotic ulcers. Occasionally, patients develop atypical presentations, including pustular, bullous, and vegetative lesions. Bullous pyoderma gangrenosum (BPG) is considered an extremely rare form. We describe a case of BPG in a 76-year-old man, with active oncological history, including a recent diagnosis of hairy cell leukemia. Diagnosis of PG was delayed because of atypical clinical presentation that mimicked necrotising fasciitis. The patient was treated with diverse intravenous antibiotics and several surgical procedures. The suspicion of neutrophilic dermatosis arose from the histopathological studies. In the setting of mandatory clinico-pathological correlation, the aim of this report is to point out the morphological characteristics that allow recognition of this uncommon variant of pyoderma gangrenosum.

Letter: Cerebriform intradermal nevus presenting as secondary cutis verticis gyrata.

Cutis verticis gyrata is a rare skin condition characterized by ridges and furrows resembling the surface of the brain. It can be considered as a manifestation of a variety of diverse causes such as cerebriform intradermal nevus. We report a 48-year-old man with cerebriform and soft folds on the left parietal and temporal areas. Histology showed solitary or clusters of nevus cells in the dermis. The diagnosis of cerebriform intradermal nevus was confirmed.

Paraneoplastic polyarthritis as the first manifestation of lingual carcinoma.

Paraneoplastic polyarthritis is an inflammatory arthritis, is usually seronegative, and has a temporal and pathophysiological relationship with an underlying malignancy. Although head and neck tumors may be a cause of paraneoplastic polyarthritis, its association with tongue carcinoma has not been previously reported. We present the case of a 69-year-old man who was a former smoker and presented with polyarthritis since 2 months in the wrists, proximal interphalangeal joints, knees, and elbows, with increased levels of acute-phase reactants; negativity for rheumatoid factor, anticitrullinated cyclic peptide antibody, and antinuclear antibody; and negative results for crystals and microorganisms in the synovial fluid. Cervical computed tomography and posterior rhinoscopy were performed, which detected an asymptomatic lesion on the base of the tongue, whose biopsy was compatible with nonkeratinizing squamous cell carcinoma. Polyarthritis did not respond to glucocorticoids at medium doses (oral prednisone 20 mg/day) but progressively resolved after the initiation of antineoplastic therapy.

An unusual case of lymphomatoid papulosis type E with extensive necrosis.

Lymphomatoid papulosis type E (LyP) is a recently described subtype of LyP characterized by an angioinvasive infiltrate of atypical lymphocytes expressing CD30. We present a case of type E LyP with extensive cutaneous necrosis in the histopathological evaluation which was misdiagnosed as an ulcerative form of bacterial skin infection. The remarkable cutaneous necrosis showed in our case might be related to the angiodestructive infiltrate that was present in this circumstance.

Prognostic significance of DNA ploidy and proliferative index (MIB-1 index) in childhood rhabdomyosarcoma.

The prognostic value of DNA ploidy and proliferative index (PI) are well established in many cancers, but their significance in childhood rhabdomyosarcoma (RMS) is unclear. We studied the DNA content and PI of 45 cases of childhood RMS obtained retrospectively. DNA histograms were hyperdiploid in 30 cases (67%), diploid in 6 (13%), tetraploid in 5 (11%), polyploid in 3 (7%), and nonclassifiable in 1 (2%). The 5-year overall survival rate by ploidy was 60% (3/5) in tetraploid, 57% (17/30) in hyperdiploid, and 0% in diploid and polyploid cases (P = .000002). The 5-year overall survival by a PI less than or greater than 19% was 62% (13/21) and 21% (5/24), respectively (P = .006). In multivariate analysis, DNA ploidy (P = .001) was an important independent prognostic factor. DNA content in childhood RMS is an important variable in predicting prognosis. DNA hyperdiploid and tetraploid rhabdomyosarcomas had a favorable prognosis, while DNA diploid and polyploid tumors had a poor prognosis.

DNA content and proliferative activity in pediatric genitourinary rhabdomyosarcoma.

Most of the studies have reported that cases of pediatric rhabdomyosarcoma (RMS) with hyperdiploid DNA and low cellular proliferative activity have better outcomes. The aim of our study was to evaluate the possible clinical relevance of DNA ploidy and proliferative activity in childhood genitourinary RMS. Twelve childhood genitourinary RMS cases were reexamined histologically and correlated with clinical features and patient survival. DNA analysis was performed on cytospin single-cell preparations obtained from the paraffin-embedded tissue blocks. MIB-1 was the proliferative marker used on paraffin sections. All patients were male with a mean age at diagnosis of 65 months. There were 5 tumors on the bladder, 5 on the pàratesticular region, and 2 on the prostate. All cases were treated with multimodality therapy using the protocols proposed by the International Society of Pediatric Oncology. The following subtypes of RMS were recognized: embryonal (n=6), spindle cell (n=4), and botryoid (n=2). DNA hyperdiploid was detected in 11 tumors (92%) and high MIB-1 index (>19%) in 4 cases (33%). Now, 10 patients are alive without evidence of disease and 2 patients are alive with evidence of disease. Our data suggested that childhood RMS of the genitourinary tract are preponderantly DNA hyperdiploid and have low cellular proliferative activity. It is also interesting that 83% of our genitourinary pediatric RMS patients are alive. As consequence of this study, we propose that DNA content and proliferative activity is a useful method to supplement findings in children with RMS.