A rare presentation of squamous cell carcinoma in a patient with PIBIDS-type trichothiodystrophy.

The clinical presentation of trichothiodystrophy type F includes photosensitivity, ichthyosis, brittle hair, intellectual impairment, decreased fertility, and short stature, often referred to as the PIBIDS syndrome. While many of these patients demonstrate features also found in xeroderma pigmentosum patients, including similar nucleotide excision repair gene defects and photosensitivity, PIBIDS patients rarely demonstrate cutaneous malignancies. This case report demonstrates the rare presentation of squamous cell carcinoma developing in a PIBIDS patient.

Fever and rash in a child: when to worry?

The differential diagnosis of a fever and rash presenting in a pediatric patient is quite extensive. This article is not all-inclusive but is meant to aid in the diagnosis to differentiate serious, life-threatening eruptions from more benign common rashes.

Trends and antibiotic susceptibility patterns of methicillin-resistant and methicillin-sensitive Staphylococcus aureus in an outpatient dermatology facility.

OBJECTIVES: To determine whether the relative proportions of methicillin-resistant Staphylococcus aureus (MRSA) and methicillin-sensitive S aureus (MSSA) were changing or stable in an outpatient dermatology clinic and to examine the antibiotic susceptibility profiles of S aureus isolates. DESIGN: Retrospective observational data were collected from skin culture isolates annually between January 1, 2005, and December 31, 2010, and monthly during the 6-month period of January 1, 2011, to June 30, 2011. SETTING: The University of Miami Hospital outpatient dermatology clinic. PARTICIPANTS: A total of 387 S aureus isolates were analyzed between January 1, 2005, and June 30, 2011, from adult and pediatric patients. MAIN OUTCOME MEASURES: The relative proportions of MRSA and MSSA skin culture isolates were measured, along with antibiotic sensitivity profiles. RESULTS: The overall relative proportion of MRSA was 35.7%. The overall relative proportion of MSSA was 64.3%. During the last 6 months of the study, the relative proportion of MRSA was 33.3%, while the relative proportion of MSSA was 66.7%. The relative proportion of MRSA from January 1, 2008, through December 31, 2010, was significantly higher than the relative proportion from January 1, 2005, through December 31, 2007 (45.3% vs 28.3%, P = .001). MRSA became more sensitive to ciprofloxacin, while MSSA became more resistant to ciprofloxacin, clindamycin, gentamicin sulfate, and trimethoprim-sulfamethoxazole. CONCLUSIONS: The relative proportion of MRSA in the S aureus isolates increased by 17.0% during the last 3 years of our study. Despite this increase, MRSA became more sensitive to ciprofloxacin, while MSSA demonstrated increased antibiotic resistance to ciprofloxacin, clindamycin, gentamicin, and trimethoprim-sulfamethoxazole.

Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment.

Pemphigus foliaceus is an autoimmune disease that clinically manifests with cutaneous blisters of the superficial skin. The nonendemic or sporadic form of this entity is rare in children and typically presents with a milder, more localized rash that usually follows a benign course of short duration. We describe an affected patient atypical in both her young age and the severity of skin findings. Our patient presented with a full body exfoliative erythroderma at 21 months of age. After an extensive work-up to determine the etiology of her exfoliative erythroderma, direct and indirect immunofluorescence studies confirmed the diagnosis of pemphigus foliaceus. Rituximab therapy was initiated based on the patient's refractory disease course to multiple immunosuppressive agents. Rituximab is a therapeutic monoclonal antibody targeting CD20, an integral membrane protein highly expressed on the surface of pre-B lymphocytes and activated mature B lymphocytes. The patient's skin exhibited marked clinical improvement after the start of rituximab infusions over 12 weeks. Her initial desmoglein 1 antibody level was greater than 1:1280, which decreased to 1:16 after seven rituximab treatments. She has had no skin flares since initiating treatment with rituximab therapy. Based on this clinical and serologic response, the use of rituximab may be helpful in the treatment of pediatric pemphigus foliaceus refractory to mainstays of therapy.