Keith Allen Knoell

Lipedematous alopecia : a clinicopathologic histologic and ultrastructural study

Lipedematous alopecia is a rare condition of unknown etiology characterized by a thick, boggy scalp with varying degrees of hair loss that occurs in adult black females, with no clearly associated medical or physiologic conditions. The fundamental pathologic finding consists of an approximate doubling in scalp thickness resulting from expansion of the subcutaneous fat layer in the absence of adipose tissue hypertrophy or hyperplasia. Observations by light and electron microscopy detailed in this report suggest that this alteration principally manifests by localized edema with disruption and degeneration of adipose tissue. Some diminution in the number of follicles as well as focal bulb atrophy is noted. Aberrant mucin deposition such as that seen in myxedema or other cutaneous mucinoses is not a feature. The histologic findings bear some resemblance to those seen in lipedema of the legs, a relatively common but infrequently diagnosed condition. We present a case of lipedematous alopecia with emphasis on histologic and ultrastructural features. The etiology is unknown.

Dermatomyositis associated with bronchiolitis obliterans organizing pneumonia (BOOP)

Bronchiolitis obliterans organizing pneumonia (BOOP) is rarely associated with dermatomyositis and may be resistant to conventional corticosteroid therapy under this circumstance. We present a case of BOOP associated with dermatomyositis that responded to a combination of cyclophosphamide and corticosteroid therapy after corticosteroid treatments, alone, had failed. We believe this case shows it is important to recognize that facial rash in the presence of respiratory distress may represent dermatomyositis with BOOP and aggressive treatment may be necessary for resolution of pulmonary symptoms.

Sudden onset of disseminated porokeratosis of Mibelli in a renal transplant patient

Porokeratosis is a disorder of epidermal keratinization of uncertain cause. Five clinical variants of porokeratosis have been described. These include porokeratosis of Mibelli, punctate porokeratosis, linear porokeratosis, porokeratosis palmaris plantaris et disseminata, and disseminated superficial porokeratosis. Disseminated superficial porokeratosis and single plaque porokeratosis of Mibelli have each been documented to occur in association with immunosuppression. To our knowledge, only 5 cases of disseminated porokeratosis of Mibelli in transplant recipients have been reported. We present a patient who developed explosive onset of disseminated porokeratosis of Mibelli shortly after renal transplantation. It is important to differentiate this unusual variety of porokeratosis from other cutaneous manifestations in transplant patients so that appropriate therapy can be instituted.

Familial multiple blue nevi

The blue nevus most commonly presents as a solitary blue nodule. Four histologic variants of blue nevus have been described. Rarely, multiple blue nevi blue nevi may occur in a single person. Only 1 case of familial multiple blue nevi has been described.

Efficacy of adalimumab in the treatment of generalized granuloma annulare in monozygotic twins carrying the 8.1 ancestral haplotype.

plied Biosystems), and 3.0 μL each of 25mM magnesium chloride and Taq polymerase (AmpliTaq Gold; Applied Biosystems). The forward keratin 9 primer sequence used was 5 -TTG GCT ACA GCT ACG GCG GAG GAT-3 ; reverse, 5 -TGA GAT CAT CAA TAG TGT TAT AAT-3 . The PCR program involved 1 cycle at a temperature of 94°C for 5.5 minutes; 1 cycle at 62°C for 1.0 minute; 1 cycle at 72°C for 1.0 minute; 1 cycle at 94°C for 0.5 minute; 1 cycle at 62°C for 1.0 minute; 40 cycles at 72°C for 1 minute each; and 1 cycle at 72°C for 10 minutes in a PTC-100 Programmable Thermal Controller (MJ Research Inc, Watertown, Massachusetts). Polymerase chain reaction products were purified using QIA Quick PCR purification Kit (Qiagen, Hilden, Germany). The genomic DNA from the patient’s peripheral blood showed an R162W mutation in the keratin 9 gene. We therefore diagnosed the patient with Vörner type EPPK.

Structure and Quantitative Efficacy of the Basic Lattice Stitch

BACKGROUND The basic lattice stitch has been proposed to be a highly effective component in the repair of atrophic skin and wounds under high tension. OBJECTIVE To compare the efficacy of the basic lattice stitch in dispersing tissue tension from the perpendicular plane of closure with that of a simple interrupted stitch. A dispersion of closure force could lead to fewer tissue tears at closure, lower wound dehiscence, less necrosis, and better wound healing. METHODS Closure forces on structural components of the basic lattice stitch in 21 repairs with closing tensions ranging from 0.3 to 4.0 N were studied and compared with the closing force of a simple interrupted stitch. RESULTS In 21 closures, the basic lattice stitch resulted in 54.4 ± 3.2% less tissue tension in the perpendicular plane of closure than a simple interrupted stitch (p<.005). CONCLUSION The basic lattice stitch distributes tissue tension away from the perpendicular plane of wound closure significantly more than a simple interrupted stitch. The basic lattice stitch should be considered in any closure under tension or for repair of atrophic skin as a means of effectively dispersing closure forces from the tissue in the perpendicular plane of wound closure. The author has indicated no significant interest with commercial supporters.

Rapidly Enlarging Mass on the Leg Develops After Local Trauma—Quiz Case

A 58-year-old man was seen with an 8-month history of an asymptomatic ulcerated lesion of the oral mucosa. Physical examination revealed a 4 6-mm necrotic ulceration on the lower left gingiva (Figure 1). A whitish indurated material was exposed through the mucosa, and it was easily removed with forceps (Figure 2). No other mucous or cutaneous lesions were observed. His medical history was remarkable for a prostate adenocarcinoma treated surgically in 2004 and multiple myeloma diagnosed in 2006 that had been treated with bortezomib, intravenous zoledronate, and syngenic hematopoietic stem cell transplantation. Two years later, the patient was free of disease, and his drug regimen was reduced to zoledronate only. He denied previous radiotherapy on the left jaw. Histologic examination of the exposed material was performed (Figure 3). What is your diagnosis?

Flutter valve purpura

intravascular coagulation, liver disease, anticoagulant use), thrombocytopenia, endocrinopathies (diabetes mellitus, Cushing’s disease), infections (eg, subacute bacterial endocarditis, Rocky Mountain spotted fever, cytomegalovirus), stasis purpura, toxic venoms, and capillaritis.1 Nonpalpable purpura localized primarily to the head and neck have occurred after strenuous coughing or vomiting (Valsalva purpura).2-5 It is likely that prolonged use of oral corticosteroids sufficiently weakened supporting connecNonpalpable purpura may occur as a result of many diseases, some of which are serious. We describe a case of benign truncal nonpalpable purpura in a man who had been treated with a new pulmonary mucus clearing device called a “Flutter valve.”