A confounding clinically aggressive case of necrotizing granulomatous and suppurative dermatitis.

Superficial granulomatous pyoderma gangrenosum is a rare, superficial, vegetating form of pyoderma gangrenosum that tends to occur as a single lesion, most commonly on the trunk. Herein, we report a clinically confounding case of disseminated superficial granulomatous pyoderma gangrenosum in a patient with a 5-year history of painful and chronic ulcerations of the bilateral upper extremities and face in a sun exposed distribution. This was a diagnostically challenging case due to the treatment-refractory nature of our patient's skin lesions and the atypical clinical and histologic presentations encountered. We review our clinical decision process and acknowledge other entities that were considered during the clinical course of this case. Additionally, we discuss the lack of responsiveness to various treatment options with eventual successful clearance of this patient's active skin disease with initiation of adalimumab.

Histopathologic vasculitis from the periulcer edge: A retrospective cohort study.

BACKGROUND: Histopathologic vasculitis is often reported in periulcer specimens, but the frequency and clinical significance of this finding have not been evaluated. OBJECTIVE: We evaluated the sensitivity, specificity, negative predictive value, and positive predictive value of histopathologic vasculitis from the periulcer edge for detecting ulcers due to cutaneous vasculitis. METHODS: We performed a retrospective chart review of patients with leg ulcers at a tertiary hospital between 2009 and 2016. Histopathologic slides were evaluated by 2 dermatopathologists who were blinded to the etiology of ulcer. Focal vasculitis was defined as involvement of fewer than 3 vessels. RESULTS: Vasculitis at the periulcer edge was seen in 51.6% of the specimens (32 of 62). Of the specimens with histopathologic vasculitis, focal vasculitis was seen in the majority of specimens (71.9% [23 of 32]), whereas diffuse vasculitis was observed in 28.1% (9 of 32). Periulcer vasculitis yielded a high sensitivity (100% [95% confidence interval, 29%-100%]). Furthermore, the specificity was low (50.9% [95% confidence interval, 38.1%-63.6%]) for detecting vasculitis-induced ulcers. LIMITATIONS: Small number of vasculitis-induced ulcers. CONCLUSION: Focal vasculitis from the periulcer edge is a nonspecific finding and provides little diagnostic value in determining the etiology of lower leg ulcers. Emphasis should be placed on the combination of clinical history and examination, histology, and laboratory findings when diagnosing ulcers.

Usefulness (or lack thereof) of immunophenotyping in atypical cutaneous T-cell infiltrates.

Our purpose was to evaluate the interobserver concordance for the diagnoses of mycosis fungoides (MF), atypical dermatoses (AD), and benign dermatoses (BD) and the impact of T-cell immunophenotyping on the diagnoses MF, AD, and BD. Specimens of MF (n = 57), AD (n = 27), BD and normal skin (n = 54) were reviewed by 2 hematopathologists and 1 dermatopathologist to establish diagnostic interobserver concordance by routine morphologic examination. Immunophenotyping was performed to evaluate expression of CD2, CD3, CD4, CD5, CD7, CD8, CD20, CD30, and MIB-1. The interobserver concordance was fair to moderate compared with the original diagnosis. Partial deletion of CD2 alone was associated significantly with MF. Epidermal deletions of 2 or 3 T-cell antigens or 2 T-cell antigens not including CD7 were associated significantly with MF. An elevated CD4/CD8 ratio correlated with MF. Morphologic features were most diagnostic of MF. Immunophenotyping generally resulted in downgrading of the reaction pattern but was helpful in distinguishing MF from benign dermatoses.

Erosive papulonodular dermatosis associated with topical benzocaine: a report of two cases and evidence that granuloma gluteale, pseudoverrucous papules, and Jacquet's erosive dermatitis are a disease spectrum.

Granuloma gluteale infantum/adultorum, pseudoverrucous papules, and Jacquet's erosive diaper dermatitis are fairly uncommon manifestations of irritant contact dermatitis historically thought to be distinct clinical entities. Here we report two informative cases of an erosive papulonodular dermatosis in the genital and suprapubic area associated with topical benzocaine. We review granuloma gluteale infantum, pseudoverrucous papules and nodules, and Jacquet's erosive diaper dermatitis, and propose that these disorders may all be variants of genitocrural irritant dermatitis.

An intertrigo-like eruption from pegylated liposomal doxorubicin.

Pegylated liposomal doxorubicin (PLD) is a chemotherapeutic agent used in the treatment of solid tumors. It has a considerably lower risk of cardiotoxicity than its parent compound, doxorubicin. PLD also has a different cutaneous side effect profile than doxorubicin, and its cutaneous toxicity can be dose limiting. We report the case of a 60-year-old woman who developed erythema and erosions in the axilla and groin while on PLD for breast cancer. Nystatin was ineffective. Biopsies revealed an interface dermatitis with epidermal dysmaturation. Bland emollients and reduction in the dose of PLD resulted in resolution of the eruption. An intertriginous eruption with histological features of epidermal dysmaturation and an interface dermatitis has been previously reported in the dermatopathology literature. This eruption appears to be a distinct cutaneous toxicity of PLD.

Case reports: treatment of nevirapine-associated dress syndrome with intravenous immune globulin (IVIG).

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is an adverse drug reaction most commonly associated with aromatic antiepileptic agents. It is characterized by the triad of skin eruption, fever, and systemic involvement, with the latter usually manifesting as hepatitis and lymphadenopathy. Mortality is primarily due to hepatic failure and can be as high as 10%. Formerly referred to by names such as Dilantin hypersensitivity syndrome and anticonvulsant hypersensitivity syndrome, DRESS syndrome is a more precise term since this reaction pattern can be seen with other agents. DRESS syndrome has also been reported in association with sulfonamides, allopurinol, terbinafine, minocycline, azathioprine, and dapsone as well as with several antiretroviral agents such as abacavir and nevirapine. We describe a patient with HIV who developed nevirapine hypersensitivity syndrome who was successfully treated with intravenous immune globulin (IVIG).

Relapsing polychondritis.

A 43-year-old Chilean man presented with a 5-month history of progressive hypertrophy of the ears bilaterally. He was seen initially by a dermatologist in Chile for complaints of erythema and swelling of the ears, and had been treated unsuccessfully with topical steroids and antimicrobial ointments. On presentation to our clinic, the hypertrophy had stabilized and the erythema had resolved, but he complained of decreased hearing due to narrowing of the external auditory canal. Associated symptoms included occasional pruritus, but he denied any pain. He also denied a history of sinus problems, respiratory symptoms, ocular pain, chest pain, and arthralgias. Physical examination revealed firm hypertrophy of the collagenous areas of both ears, sparing the ear lobes (Fig. 1). No pain was elicited on palpation. No conjunctivitis was noted and the nasal passages were clear. His chest was clear to auscultation. Histologic examination revealed a minimal perivascular infiltrate of lymphocytes and plasma cells in the dermis with fibrosis of the subcutis (Fig. 2). Blood tests showed a normal complete blood count, antinuclear antibody, and rheumatoid factor. Anti-collagen II antibodies were elevated at 29.2 Eu/ml (normal, 0-20 Eu/ml; borderline, 20-25 Eu/ml; elevated, > 25 Eu/ml).

Treatment of toxic epidermal necrolysis with intravenous immunoglobulin in children.

BACKGROUND: Toxic epidermal necrolysis (TEN) is an acute illness characterized by rapid onset of skin necrosis and high mortality. Standard treatment is primarily aimed at supportive care in a burn unit setting. OBJECTIVE: We evaluated the outcome of 8 pediatric patients treated for TEN with intravenous immunoglobulin (IVIg) over a 3-year period. METHODS: We performed a retrospective analysis of pediatric patients with a diagnosis of TEN between 1999 and 2001, obtained from a computerized database. RESULTS: Mean body surface involvement of 8 patients treated with IVIg was 67%. The average length of hospitalization was 13.6 days, with an average delay in treatment of 3.2 days. The average time to arrest in progression of lesions was 2.1 days and to complete re-epithelialization, 8.1 days. The mortality rate was 0%. The majority of complications were infectious. CONCLUSION: IVIg is a safe and effective treatment for TEN in the pediatric population. Randomized trials are needed to further evaluate the efficacy of IVIg compared with other modalities.