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.

A Single-Institution Cohort Study With Nevi of Special Site: Recurrence, Progression to Melanoma, and Patterns of Management.

ABSTRACT: Nevi of specialized sites (NOSS) occur on the scalp, ears, flexural, acral, and genital areas and display atypical clinical and histologic features. We assessed NOSS recurrence and progression to melanoma, management patterns, and associations between histologic features and treatment recommendations. We queried all histologic diagnoses of NOSS (n = 275) from 2012 to 2017 from a large U.S. academic medical center with reference dermatopathology laboratory and matched these to clinical records. A blinded panel of dermatopathologists re-evaluated lesions, catalogued histologic findings, and gave management recommendation. Associations with dermatopathologist decision and concordance between new and original recommendations were assessed. Of 117 cases with follow-up, 2 locally recurred (1.46%) and none eventuated in melanoma. Clinical features were not associated with original treatment recommendations. After histopathologic review, large melanocytes [odds ratio ratio (ORR) = 8.00, 95% CI, 1.35-47.4] and junctional mitotic figures (ORR = 65.0, 6.5-650) predicted excision recommendation. Likewise, accumulation of many (>9) high-risk features was associated with excision recommendation. Panel review changed treatment recommendation in 27% of cases. Fair concordance existed between original and panel recommendations (κ = 0.29, 0.15-0.44). The low rate of recurrence and lack of melanoma occurrence suggest that despite an atypical clinical and histopathologic appearance, these nevi have limited potential for malignant transformation. Histopathologic findings seem to be principal drivers behind the recommendation for excision in this analysis. Variability existed in treatment recommendations; the panel's consensus recommendation tended to downgrade treatment. This highlights the importance of further outcomes-based studies to identify true high-risk features and refine management guidelines.

Questions raised by a case of adult-onset linear nodular scleroderma.

Morphea presenting clinically with nodular or keloidal skin changes is extremely rare. Nodular scleroderma or keloidal morphea presenting in a linear distribution is even more uncommon. We present an otherwise healthy young woman with unilateral, linear, nodular scleroderma and review the somewhat confounding earlier literature in this area. To date, this young woman's skin changes have proven refractory to oral hydroxychloroquine and ultraviolet A1 phototherapy. Several aspects of this case including the patient's family history of Raynaud disease, her nodular sclerodermatous skin lesions, and the presence of U1RNP autoantibodies raised concern about her management with respect to future risk of developing systemic sclerosis.

Darier disease, radiation therapy, and herpesvirus -- an unfortunate triad.

Darier disease (DD) is a rare autosomal dominant keratinizing disorder often characterized by brown scaly pruritic papules over the face, neck, and trunk. Herein is reported a patient who developed secondary cutaneous herpes simplex virus (HSV) following exacerbation of his DD as a result of radiation therapy. In November 2020, a 78-year-old man presented to clinic for a pruritic rash on his back consistent with DD. He had developed the rash after the conclusion of chemoradiation therapy for recently diagnosed urothelial carcinoma of the bladder with squamous differentiation. However, he returned two weeks later complaining of a marked worsening of the rash associated with a pain and burning sensations. Histopathology was non-conclusive, but the lesions were found to be positive for HSV-1 by PCR. The patient recovered without complication over a period of two weeks following a course of valacyclovir. There is precedent in the literature for ionizing radiation inducing flares of DD lesions in overlying skin. In addition, DD has been shown to put a patient at increased risk for secondary infections such as HSV. This case report demonstrates that HSV could pose a significant risk to those with DD receiving radiation therapy and thus could warrant prophylactic treatment.

Eosinophils are surprisingly common in biopsy specimens of cutaneous herpes simplex virus and varicella zoster virus infections: Results of a comprehensive histopathologic and clinical appraisal.

BACKGROUND: While usually straightforward, diagnostic features of cutaneous herpes simplex virus and varicella zoster virus infection (HSV/VZV) are not always present in biopsy specimens. Although intuitively the presence of eosinophils may lead the pathologist away from the diagnosis of cutaneous HSV/VZV infection, in our practice we have noted that eosinophils are often encountered in diagnostic specimens. METHODS: To deduce the frequency with which the inflammatory response accompanying cutaneous HSV/VZV infection includes significant numbers of eosinophils, we performed a retrospective review. We included 159 specimens from our database, diagnosed between 2009 and 2017. We determined the number of eosinophils in 10 high-power fields and noted additional histologic factors including presence of follicular involvement, ulceration, and pseudolymphomatous change. RESULTS: Of all included cases, 63% had 0-1 eosinophils, 24% had 2-10 eosinophils, and 13% had more than 10 eosinophils. Statistical analysis did not reveal a significant association between any demographic or histologic features examined and the presence of increased eosinophils. CONCLUSIONS: In this study, more than one-third of biopsy specimens diagnostic of cutaneous HSV/VZV infection had a prominent number of eosinophils. The detection of eosinophils should not be unexpected and should not lessen diagnostic suspicion for cutaneous HSV/VZV infection.

Relapsing polychondritis with meningoencephalitis.

Relapsing polychondritis (RP) is a rare autoimmune disease of the cartilaginous structures of the body with many systemic manifestations including meningoencephalitis (ME). We present a case of a man with RP-associated ME that was responsive to steroid treatment. An updated literature review of 7 cases of RP-associated ME also is provided. Early diagnosis of this condition may be of benefit to this select population of patients, and further research regarding the prognosis, mechanisms, and treatment of RP may be necessary in the future.

Perforating lichen nitidus.

Transepidermal elimination and/or perforation of the epidermis is seen in a variety of conditions but has only rarely been reported in association with lichen nitidus. We describe a case of lichen nitidus with the unique finding of epidermal perforation, a feature that made the usual straightforward diagnosis of lichen nitidus much more difficult.

Melanocytic matricoma with melanocytic atypia: report of a unique case and review of the literature.

Melanocytic matricoma is a rare cutaneous neoplasm of presumed anagen hair follicle origin with approximately 10 reported cases in the literature. Melanocytic matricomas are clinically and histopathologically distinct cutaneous nodular proliferations of matrical and supramatrical cells admixed with dendritic melanocytes, which typically occur in the sun-exposed areas of elderly patients. We report a new case with additional histopathologic features not previously described. An 82-year-old white man presented with an exophytic papule of the ear clinically suspicious for basal cell carcinoma. Histopathologic examination demonstrated a polypoid neoplasm consisting of an admixture of matrical and shadow cells with numerous interspersed dendritic and epithelioid melanocytes arranged singly and in large expansile nests. An unusual feature in this case included prominent melanocytic proliferation with associated nuclear atypia and increased mitotic activity. Although atypical and malignant melanocytic colonization has been reported in basal cell carcinomas and squamoproliferative lesions, to our knowledge, it has not been previously described in melanocytic matricomas. The biologic significance of atypical melanocytic proliferations within melanocytic matricomas is uncertain and requires further study of additional cases and long-term follow-up.

Noninvasive in vivo imaging to evaluate immune responses and antimicrobial therapy against Staphylococcus aureus and USA300 MRSA skin infections.

Staphylococcus aureus skin infections represent a significant public health threat because of the emergence of antibiotic-resistant strains such as methicillin-resistant S. aureus (MRSA). As greater understanding of protective immune responses and more effective antimicrobial therapies are needed, a S. aureus skin wound infection model was developed in which full-thickness scalpel cuts on the backs of mice were infected with a bioluminescent S. aureus (methicillin sensitive) or USA300 community-acquired MRSA strain and in vivo imaging was used to noninvasively monitor the bacterial burden. In addition, the infection-induced inflammatory response was quantified using in vivo fluorescence imaging of LysEGFP mice. Using this model, we found that both IL-1α and IL-1ß contributed to host defense during a wound infection, whereas IL-1ß was more critical during an intradermal S. aureus infection. Furthermore, treatment of a USA300 MRSA skin infection with retapamulin ointment resulted in up to 85-fold reduction in bacterial burden and a 53% decrease in infection-induced inflammation. In contrast, mupirocin ointment had minimal clinical activity against this USA300 strain, resulting in only a 2-fold reduction in bacterial burden. Taken together, this S. aureus wound infection model provides a valuable preclinical screening method to investigate cutaneous immune responses and the efficacy of topical antimicrobial therapies.