Assessing comfort level with pediatric skin specimens among dermatopathologists and pediatric pathologists: A national cross-sectional survey.

BACKGROUND: Cutaneous histopathologic diagnoses in children often differ from those in adults. Depending on practice setting, these specimens may be evaluated by dermatopathologists or pediatric pathologists. We sought to determine whether comfort level with pediatric dermatopathology is associated with prior training, pediatric dermatopathology exposure during fellowship, career duration, or specimen subtype. METHODS: We surveyed dermatopathologists and pediatric pathologists practicing in the United States. Training and practice variables were evaluated by multivariable regression for association with comfort level. RESULTS: Of the 156 respondents, 72% were dermatopathologists (response rate 11.6%) and 28% were pediatric pathologists (response rate 9.3%). Dermatopathologists reported higher comfort overall (P < .001); this was also true for inflammatory dermatoses and melanocytic neoplasms (P < .001). Thirty-four percent and 75% of dermatopathologists and pediatric pathologists, respectively, reported lower comfort with pediatric skin specimens than their usual cases. Pediatric pathologists were 28% more likely to refer these cases to colleagues. Among dermatopathologists, dermatology-trained were more comfortable than pathology-trained colleagues interpreting inflammatory dermatoses (P < .001). CONCLUSIONS: Pathologists' comfort with pediatric dermatopathology varied significantly based upon prior training, career duration, and specimen subtype. These results suggest opportunities for improving education in this domain.

Verrucous pilar cysts infected with beta human papillomavirus.

Epidermoid cysts with histopathologic features of human papillomavirus (HPV) infection have been previously reported and are commonly termed verrucous cysts. We report a series of eight histopathologically distinct verrucous pilar cysts, distinguished from traditional verrucous epidermoid cysts by trichilemmal keratinization, as well as two verrucous hybrid pilar-epidermoid cysts. These lesions contain characteristic stratified epithelial linings with abrupt transitions to compact eosinophilic keratin, as well as areas of papillomatosis, coarse intracytoplasmic keratohyalin granules, and vacuolar structures suggestive of HPV-induced cytopathic change. HPV-24, a ß genus HPV species, was identified by degenerate polymerase chain reaction in DNA extracted from two of the lesions, and the presence of ß-HPV E4 protein was confirmed by immunohistochemistry. HPV-60, the HPV species most commonly reported in verrucous epidermoid cysts, was not detected. Verrucous pilar cysts represent histopathologically and potentially etiologically distinct lesions which may be underrecognized.

Therapy for anogenital verrucae in preadolescent children with topical and systemic treatment.

BACKGROUND/OBJECTIVES: Anogenital verrucae (AV) are benign, human papillomavirus (HPV)-induced tumors of the anogenital skin and mucosa. Medical therapy for AV in preadolescents has not been well studied. We explore the efficacy and safety profile of sinecatechins 15% ointment and imiquimod 5% cream in the treatment of AV, alone and in combination therapy with other commonly used medications. METHODS: A single-institution, retrospective review of children under 12 years of age with AV treated with imiquimod 5% cream and sinecatechins 15% ointment was performed. Demographic data, side effects, and outcomes of therapy were recorded for each patient, and overall efficacy was determined. RESULTS: A total of 37 patients met inclusion criteria. Responses were seen in 8 out of 9 patients treated with sinecatechins 15% ointment (5 full, 3 partial, and 1 no response) and 9 out of 17 patients treated with imiquimod 5% cream (4 full, 5 partial, and 8 no response). Combination therapy with one or more of the following treatments (podophyllin, cimetidine, candida antigen injection, and HPV vaccine) were evaluated, but no combination was objectively superior to the others. No significant difference was found in overall efficacy between sinecatechins and imiquimod. Side effects were mild and limited to irritation and erythema. CONCLUSIONS: Both imiquimod 5% cream and sinecatechins 15% ointment are moderately effective in the treatment of AV in preadolescent children, with a trend toward greater effectiveness of sinecatechins. Combination therapy with other treatments did not significantly increase the effectiveness of topical therapies. Each modality has a tolerable side effect profile with a low risk of serious complications.

High-risk human papillomavirus in a child with digital pigmented Bowen's disease: Case report and dermoscopic findings.

Squamous cell carcinoma in situ, also known as Bowen's disease (BD), is a skin malignancy most commonly seen in middle-aged and elderly adults. Pediatric BD is rare and can be a diagnostic challenge for physicians. Digital BD has largely been associated with human papilloma virus. We report an immunocompetent 11-year-old girl with periungual pigmented BD induced by high-risk human papilloma virus.

Pernio as the presenting sign of blast crisis in acute lymphoblastic leukemia.

A previously healthy 5-year-old girl presented with acute onset of blue toes and red spots on the nose and fingers. The striking nature of these lesions, along with the finding of submandibular lymphadenopathy, prompted further evaluation. Laboratory findings were remarkable for anemia, high transaminase levels, and high blast count. Histopathologic findings were consistent with early pernio. Further examination revealed acute B-cell lymphoblastic leukemia. Treatment of the leukemia led to resolution of the pernio.

Congenital infantile fibrosarcoma mimicking a cutaneous vascular lesion: a case report and review of the literature.

Congenital infantile fibrosarcoma (CIFS) is a rare neoplasm of infancy that occurs most frequently in the extremities, and when presenting in the skin, may sometimes resemble infantile hemangiomas or other vascular lesions. Clinically, these tumors differ from hemangiomas in the time of onset, morphology, and growth pattern and must be evaluated histologically for definitive diagnosis. We describe an infant with a neoplasm involving the distal left forearm initially presumed to be a vascular lesion after evaluation by two separate ultrasound studies. He presented at seven weeks of life with a multinodular lesion that had enlarged significantly since birth, and the skin biopsy revealed a fibrosarcoma. This case highlights an unusual cutaneous presentation of CIFS, which varies in appearance from the previous 12 cases reported in the literature. We review the clinical manifestations of these congenital masses and emphasize early diagnosis for conservative therapy and improved prognosis.

Acral Plexiform Palisaded Encapsulated Neuromas as the Initial Cutaneous Manifestation of Cowden Syndrome.

Cowden syndrome (CS) is an autosomal dominant genodermatosis associated with characteristic mucocutaneous findings of facial trichilemmomas, palmoplantar keratoses, sclerotic fibromas, and oral papillomas. Mucocutaneous neuromas have also been reported in association with CS. We describe a patient with CS whose sole cutaneous finding was palisaded encapsulated neuromas (PENs) with a plexiform growth pattern in an acral location. Along with previous reports, this case suggests that acral plexiform PENs may be an early, highly specific finding in CS and highlights the importance of screening these patients for PTEN mutation.

Segmental stiff skin syndrome (SSS): A distinct clinical entity.

BACKGROUND: Stiff skin syndrome (SSS) is a noninflammatory, fibrosing condition of the skin, often affecting the limb girdles. OBJECTIVE: We present 4 new patients with SSS with largely unilateral, segmental distribution. To date, reported cases of SSS have been grouped based on generally accepted clinical and histopathologic findings. The purpose of this study was to analyze differences in clinical and histopathologic findings between previously reported SSS cases. METHODS: This is a retrospective review of 4 new cases and 48 previously published cases of SSS obtained from PubMed search. RESULTS: Of 52 total cases, 18 (35%) were segmentally distributed and 34 (65%) were widespread. The average age of onset was 4.1 years versus 1.6 years for segmental versus widespread SSS, respectively. Limitation in joint mobility affected 44% of patients with segmental SSS and 97% of patients with widespread SSS. Histopathologic findings were common between the 2 groups. LIMITATIONS: This was a retrospective study of previously published cases limited by the completeness and accuracy of the reviewed cases. CONCLUSIONS: We propose a distinct clinical entity, segmental SSS, characterized by a segmental distribution, later age of onset, and less severe functional limitation. Both segmental SSS and widespread SSS share common diagnostic histopathologic features.

Acrokeratoelastoidosis.

Acrokeratoelastoidosis is a rare palmoplantar keratoderma. We report the case of a 32-year old man with multiple keratotic papules and plaques on the peripheral margins of his hands and feet. Histopathologic evaluation showed compact orthokeratosis; Verhoeff van Gieson stain showed thin and fragmented elastic fibers. The differential diagnosis includes focal acral hyperkeratosis, which has a similar presentation but with normal elastic fibers. Treatment of acrokeratoelastoidosis is often disappointing, with minimal response to topical glucocorticoids, keratolytics, retinoids, cryotherapy, and lasers.

Generalized granuloma annulare in a folliculocentric distribution.

We present a 69-year-old man with type 2 diabetes mellitus and a five-year history of an eruption of follicular pustules, papules, and nodules, which was identified histopathologically as folliculocentric granuloma annulare (GA). Folliculocentric generalized GA is a rarely reported variant of GA, in which the the palisading histiocytes form focal granulomas in a follicular pattern. In this case, the GA may represent an isotopic phenomenon, with lesions developing in hair follicles that were previously affected by a suppurative folliculitis.

Eosinophilic dermatitis of hematologic malignancy.

Eosinophilic dermatosis of hematologic malignancy is a rare, paraneoplastic phenomenon that presents as a pruritic papular or vesicular eruption that is clinically and histopathologically similar to insect bites. We present a 56-year-old man with multiple relapses of diffuse large B cell lymphoma with a typical presentation of pruritic papules and vesicles on the extremities that correlate with a recent relapse of his lymphoma.

Photodistributed granuloma annulare.

Annular elastolytic giant cell granuloma (AEGCG) is a controversial entity that is considered by many to be a variant of granuloma annulare (GA). The majority of cases of AEGCG occur in Caucasian women (3:2) between the ages of 40 and 70, with the distribution of the mostly annular lesions favoring exposed areas of skin and rarely involving covered skin. The most common systemic association has been with diabetes mellitus. We present a 52-year-old woman with an asymptomatic, annular, erythematous, photodistributed eruption of two-years duration. As part of her evaluation, it was detected that she had a hemoglobin A1C of 10.3% and a diagnosis of diabetes mellitus was made. We review the literature on the clinical and histopathologic features of GA and AEGCG and the overlap between these entities.

Squamous-cell carcinoma in situ.

We present a 30-year-old woman with atopic dermatitis and ichthyosis vulgaris and a one-year history of an erythematous, scaly plaque on the dorsal surface of her right hand, which developed three years after an accidental exposure to prolonged ultraviolet C (UVC) radiation in a laboratory accident. The plaque, which was initially treated as eczematous dermatitis, was eventually identified histopathologically as squamous-cell carcinoma in situ. Although causation is not definitive, this case is the first to describe development of non-melanoma skin cancer (NMSC) in an area of skin known to be acutely exposed to UVC radiation. As UVC radiation becomes a more frequently used anti-microbial technology, UVC radiation may become a more commonly identified risk factor in the development of NMSC.

Paraneoplastic erythema annulare centrifugum eruption (PEACE).

Erythema annulare centrifugum (EAC) is a reactive erythema with distinct, annular, erythematous plaques with trailing scale. This condition has been associated with various etiologies, which include an associated malignant condition. EAC with cancers or paraneoplastic erythema annulare centrifugum eruptions (PEACE), is more likely to be associated with lymphoproliferative malignancies such as lymphomas and leukemias. Histopathologic features include a superficial and deep, lymphohistiocytic perivascular infiltrate. We present a patient with a history of diffuse large B cell lymphoma in remission for two years, who presented with a one-year history of EAC.

Vesicular erythema migrans: an atypical and easily misdiagnosed form of Lyme disease.

Erythema migrans is the initial sign in the majority of patients infected with Borrelia, the genus of spirochetes that causes Lyme disease. Early identification and treatment decrease the risk of progression to later stages of disease. Although a "bull's eye" appearance owing to lesional clearing is considered classic for erythema migrans, this feature is surprisingly often lacking among patients in the United States. Furthermore, cutaneous Lyme disease can exhibit a wide range of morphologic variability in a minority of patients. Herein, we describe the case of a patient with Lyme disease in which the presence of atypical vesicular features, in conjunction with the initial absence of clearing, resulted in multiple misdiagnoses and delayed treatment. We also review the literature on the epidemiology and management of erythema migrans for cases in which the diagnosis may pose a challenge.

Hydroxycholoroquine-induced hyperpigmentation.

A 57-year-old woman with systemic lupus erythematosus and Sjögren syndrome presented with blue-grey hyperpigmentation of the face, upper back, and dorsal aspects of the feet after seven years of therapy with hydroxychloroquine. We present an unusual case of drug-induced hyperpigmentation.

Perifollicular Langerhans cell histiocytosis.

A 25-year-old man presented with a 13-year history of an erythematous, papular eruption of his face and trunk, which was treated in the past as acne and psoriasis with isotretinoin and methotrexate, respectively. Histopathologic examination demonstrated an infiltrate of Langerhans cells, which was consistent with Langerhans cell histiocytosis. The epidemiology, clinical presentation, and treatment options of this disease are reviewed.

Erythema annulare centrifugum.

Erythema annulare centrifugum (EAC) is a gyrate erythema, which is typically characterized by annular, erythematous plaques with trailing scale. It is considered to be a reactive condition with a wide variety of inciting causes but unclear pathophysiology. The mean duration of the eruption is 11 months. We present a patient with a 50-year history of recurrent EAC with no known cause. Although it does follow a previously reported pattern of seasonal recurrence, this case represents the longest reported duration of EAC.