Subepidermal calcified nodule presenting as a cutaneous horn: two cases and a review of the literature.

Subepidermal calcified nodules are an uncommon subtype of idiopathic calcinosis cutis. Morphologically, this entity typically present as a single, well-circumscribed, white-yellow nodule. Based on clinical appearance alone, subepidermal calcified nodules are frequently misdiagnosed and often requires histological confirmation. We describe two cases of subepidermal calcified nodules presenting atypically as cutaneous horns. Subepidermal calcified nodules presenting as a cutaneous horn has rarely been reported; on review, there are fewer than 10 such cases have been described within the past 30 years. The cases described here illustrate the clinical variety and should increase awareness of subepidermal calcified nodules presented.

Off-label use of dupilumab for pediatric patients with atopic dermatitis: A multicenter retrospective review.

BACKGROUND: Atopic dermatitis (AD) is a common, chronic type 2 inflammatory skin disease, typically starting in infancy, with increased risk for subsequent extracutaneous atopic morbidities. Dupilumab is the first biologic agent targeting type 2 inflammation approved by the U.S. Food and Drug Administration (USFDA); it was licensed in 2017 for adults with moderate to severe AD and 2 years later for adolescents. Systemic treatment for pediatric AD remains a significant unmet medical need. OBJECTIVE: To analyze off-label use of dupilumab in children with AD. METHODS: Multicenter retrospective review that evaluated children who were prescribed dupilumab for moderate to severe AD. RESULTS: One hundred eleven of 124 patients (89.5%) gained access to dupilumab after a mean of 9 weeks. The dosing range was 4 to 15.5 mg/kg for the loading dose and 2.0 to 15.3 mg/kg every other week for maintenance. The range was widest for 6- to 11-year-olds and was related to use of either full or half of adult dosing. Associated morbidities, treatment response, and adverse events were comparable to those in previous adolescent and adult trials. LIMITATIONS: The retrospective design of the study limited uniform data collection. CONCLUSION: Access to dupilumab was achievable for the majority of children after a mean 9-week delay because of insurance payment denial. This review supports dupilumab response and tolerability in children. Optimal dosing for patients younger than 12 years has not been defined. Availability of the drug in 2 different concentrations is an important safety issue.

Fibroblastic rheumatism: a report of 4 cases with potential therapeutic implications.

BACKGROUND: Fibroblastic rheumatism is a rare dermatoarthropathy characterized by the sudden onset of cutaneous nodules, flexion contractures, and polyarthritis. Histopathology in the correct clinical context confirms the diagnosis. Treatment is based on observational data from single case reports. OBJECTIVE: We describe 4 cases, review histologic findings, and discuss therapeutic responses. METHODS: Cases coded as fibroblastic rheumatism were retrieved from institutional and consultation files. Medical charts and biopsy specimens were reviewed. Elastic stains and immunostains for smooth muscle actin, S100, CD34, desmin, and epithelial membrane antigen were performed on selected cases. RESULTS: Four cases were identified. Patients displayed cutaneous nodules and arthralgias. Flexion contractures/decreased motion were present in two patients; one patient had associated Raynaud phenomenon and erosive joint disease. Biopsy specimens demonstrated a fibroblastic proliferation associated with a collagenous stroma. Growth patterns varied from cellular fascicles to paucicellular randomly arranged spindle cells. Elastic fibers were absent in all cases tested (3/3). Immunohistochemical stains demonstrated immunoreactivity for smooth muscle actin in one of 3 cases in a myofibroblastic pattern. Other stains were negative. One patient had complete resolution of disease with methotrexate. One patient partially responded to interferon-alfa and ribavirin and was subsequently treated with methotrexate with additional improvement. One patient had limited response to all therapies attempted. One patient was lost to follow-up. LIMITATIONS: Small sample size (n = 4) is a limitation. CONCLUSION: Our data expand the clinical, histologic, and therapeutic response data on fibroblastic rheumatism. Correlation with clinical history is critical to avoid misdiagnosis as other fibrosing lesions. Methotrexate and interferon-alfa are potential therapies.

Buschke-Ollendorff syndrome and bilateral cutaneous syndactyly.

A 3-year-old boy presented with asymptomatic elastomas on the posterior trunk. Radiographic studies revealed osteopoikilosis, confirming the diagnosis of Buschke-Ollendorff syndrome. The patient had a history of bilateral simple cutaneous syndactyly, which has not been previously reported with this condition. Buschke-Ollendorff syndrome is a rare autosomal-dominant disorder characterized by connective tissue nevi and osteopoikilosis. Several associated systemic abnormalities have been reported, but morbidity and mortality are generally not affected.

Pediatric dermatology emergencies.

PURPOSE OF REVIEW: The purpose of this review is to summarize recent advances in the diagnosis and treatment of several severe skin diseases seen in children. RECENT FINDINGS: Several articles published recently added significantly to our understanding of drug reaction with eosinophilia and systemic symptoms (DRESS), eczema herpeticum, staphylococcal scalded skin syndrome and inherited ichthyosis. A review of 60 patients with DRESS showed 80% with hepatic involvement, 40% with renal involvement, 33% with pulmonary involvement and 50% with hematologic abnormalities. A review on eczema herpeticum describes several clinical scenarios in which it is vital to keep that diagnosis in mind. Another review on severe staphylococcal skin infections reminds us of the importance of covering for methicillin-resistant Staphylococcus aureus in all such cases until cultures and sensitivities show otherwise. The results of an international consensus conference on ichthyosis published this year will help all of us to better characterize these complicated patients. Additionally, the first case of hypothyroidism associated with a collodion baby was reported, as was the finding of an increased incidence of atypical nevi in patients with inherited ichthyosis. SUMMARY: Although all of the conditions discussed in this article are relatively rare, it is important that they be kept on the differential diagnosis for sick infants and children with cutaneous findings, as early diagnosis and treatment of some of these conditions can be life saving.

Treatment of molluscum contagiosum and herpes simplex virus cutaneous infections.

Molluscum contagiosum (MC) is a human viral infection contracted by skin-to-skin inoculation, autoinoculation, and fomite inoculation. There are a variety of treatments for MC, including watchful waiting, curettage, cantharidin, cryotherapy, and topical imiquimod. Herpes simplex virus (HSV) infections also have been treated with imiquimod. This article discusses the treatment of MC and HSV infections and reviews the literature.

Atrophic dermatofibroma: a case report and review of the literature.

BACKGROUND: Atrophic dermatofibroma is an uncommon variant of dermatofibroma. It lacks the classic clinical features of a dermatofibroma and is often misdiagnosed. OBJECTIVE: To describe the clinical and histologic features of atrophic dermatofibroma. METHODS: Case report and review of the literature. RESULTS: A 45-year-old white woman was found to have a 7 mm x 5 mm atrophic, depressed lesion in the right axilla. A clinical diagnosis of anetoderma was made and the lesion was excised with minimal margins. Histopathologic examination revealed findings consistent with dermatofibroma. However, focal CD34 staining and involvement of superficial subcutaneous tissue raised concern regarding an early dermatofibrosarcoma protuberans developing in a dermatofibroma. The patient underwent Mohs micrographic surgery (MMS) for definitive treatment. The final diagnosis is thought to be atrophic dermatofibroma. Reported cases in the English language literature are reviewed and the clinical and histopathologic findings are described. CONCLUSION: Atrophic dermatofibroma is a well-described, yet uncommon, variant of dermatofibroma. It is often clinically misdiagnosed, and histopathologic evaluation can be misleading. The clinician and pathologist should consider this diagnosis in the evaluation of atrophic, depressed lesions.