Sheila S. Galbraith

Serum 25-hydroxyvitamin D concentration does not correlate with atopic dermatitis severity

BACKGROUND An inverse correlation between serum 25-hydroxyvitamin D concentration and atopic dermatitis (AD) severity has been suggested. OBJECTIVE To determine if a statistically significant relationship exists between serum 25-hydroxyvitamin D concentration and AD severity. METHODS A cross-sectional study was conducted of patients with AD who were 1 to 18 years of age. An objective Severity Scoring of Atopic Dermatitis (SCORAD) and a serum 25-hydroxyvitamin D concentration were measured for each subject. Statistical analysis was performed using appropriate univariate tests and multivariable models. RESULTS Ninety-four of 97 enrolled subjects were included in the analysis. Vitamin D deficiency (25-hydroxyvitamin D <20 ng/mL) was present in 37 subjects (39%), insufficiency (25-hydroxyvitamin D 21-29 ng/mL) in 33 (35%), and sufficiency (25-hydroxyvitamin D ≥30 ng/mL) in 24 (26%). The correlation between 25-hydroxyvitamin D concentration and SCORAD was not significant (r = -0.001; P = .99). A multivariate model showed that a lower serum 25-hydroxyvitamin D concentration was significantly associated with age 3 years or older (P < .0001), black race (P < .0001), and winter season (P = .0084). LIMITATIONS Limitations of this study include the inability to control for natural sunlight exposure, vitamin D intake, and AD treatment; in addition, only a single time point was captured. CONCLUSIONS Serum 25-hydroxyvitamin D concentration is not significantly correlated with AD severity in our pediatric population.

Asymptomatic Inflammatory Bowel Disease Presenting With Mucocutaneous Findings

Although inflammatory bowel disease (IBD) typically presents with gastrointestinal complaints, mucocutaneous lesions are commonly associated and can precede gastrointestinal symptoms, thereby alerting the clinician to the diagnosis of IBD before the onset of gastrointestinal symptoms. Nine children are reported who had no gastrointestinal symptoms suggestive of IBD but presented with mucocutaneous findings of IBD and were subsequently diagnosed with Crohns disease or ulcerative colitis based on characteristic features on gastrointestinal endoscopy and/or biopsies. The majority of the patients had oral and perianal lesions. We believe that IBD is a common etiology for persistent oral lesions in the pediatric population. In addition to a good history, children with unexplained oral mucous membrane lesions should have an examination of the rectal and genital mucosa as well as tests for complete blood count, iron levels, sedimentation rate, albumin, and occult blood in the stool with endoscopy and biopsies to rule out IBD if indicated.

Risk Factors for Morphoea Disease Severity: A Retrospective Review of 114 Paediatric Patients

Morphoea is a rare fibrosing disease of the skin and subcutaneous tissue with an unpredictable disease course, running the spectrum from mild skin involvement to severe disfigurement or extracutaneous complications.

Demographic and clinical characteristics of cutaneous lupus erythematosus at a paediatric dermatology referral centre.

Paediatric cutaneous lupus erythematosus (CLE) is uncommon and inadequately described in the literature. Similar to adults, children with CLE develop LE‐specific and/or LE‐nonspecific skin findings. Similarities and differences in demographics and clinical course between paediatric and adult CLE have not been sufficiently described.

Generalized congenital smooth muscle hamartoma presenting with hypertrichosis, excess skin folds, and follicular dimpling.

Abstract:  We describe an interesting patient with a diffuse smooth muscle hamartoma who presented with hypertrichosis, increased skin folds, and follicular dimpling. While smooth muscle hamartoma classically presents as a small hairy, skin‐colored to hyperpigmented patch or plaque, rare presentations with diffuse involvement or follicular papules have been described. We present our patient and review the literature to highlight the diverse and under recognized manifestations of this benign neoplasm.

The natural history of skin-limited Langerhans cell histiocytosis: a single-institution experience.

Introduction: Prior reports of Langerhans cell histiocytosis (LCH) suggest that isolated skin involvement is rare and often progresses to systemic disease. More rapid access to pediatric subspecialty care has likely led to more frequent representation of this condition. The purpose of this study is to characterize the natural history of skin-limited LCH in an era of increased access to pediatric subspecialty care. Materials and Methods: A retrospective chart review was performed on all patients newly diagnosed with LCH between 2001 and 2012 at the Children’s Hospital of Wisconsin. Extensive review of laboratory, physical examination, and imaging reports was performed and data collected for patients with biopsy-proven skin LCH. Results: Sixteen individuals with skin-limited LCH were identified. The median age at onset of skin eruption was birth (range, birth to 6 mo), and median duration of follow-up was 19.5 months (range, 2 wk to 10 y) from diagnosis. One patient (6%) developed pituitary disease and 1 patient (6%) had refractory skin involvement. All others experienced complete resolution. For patients without progressive or refractory disease, resolution of skin findings occurred within 7 months from onset. Discussion: Progression of skin-limited to multisystem LCH likely may be less frequent than previously described.

Congenital lupus erythematosus presenting at birth with widespread erosions, pancytopenia, and subsequent hepatobiliary disease.

Abstract:  Neonatal lupus erythematosus is an uncommon disease caused by transplacental passage of maternal anti‐Ro (SS‐A), anti‐LA (SS‐B), or anti‐U1RNP antibodies. Cutaneous findings of neonatal lupus are variable, but annular, erythematous plaques occurring within a few weeks of birth are most typical. Cutaneous lesions of congenital onset lupus erythematosus can differ from that of neonatal lupus erythematosus, presenting with atrophy or scarring, and less commonly, erosions. We report an unusual case of congenital lupus erythematosus presenting at birth with widespread erosions, pancytopenia, and subsequent hepatobiliary disease.

Cutaneous and Disseminated Blastomycosis: A Pediatric Case Series

Abstract:  Blastomycosis is a rare fungal infection that most often initially infects the lungs and can progress to disseminated involvement of the skin, bones, and central nervous system (CNS). Pediatric blastomycosis constitutes a small portion of total cases, but delay in diagnosis may result in significant morbidity. Seventeen pediatric cases of blastomycosis were identified at Children’s Hospital of Wisconsin from 1999 to 2009 through retrospective chart review; 53% had evidence of dissemination (bone, skin, or CNS) confirmed by culture. Six cases presented with cutaneous lesions, and five of these were found to have other systemic involvement. These five nonimmunosuppressed cases of primary pulmonary disease with cutaneous involvement plus dissemination to bone or the CNS are reported in detail. The diagnosis of blastomycosis in children is often delayed, and dissemination by the time of diagnosis may be more common than in adults. Cutaneous dissemination may occur in immunocompetent children, may indicate underlying systemic involvement, and can be more readily identified than symptoms of bony or neurologic involvement. These reported cases indicate the importance of dermatologists recognizing and investigating all potentially involved organ systems when a patient presents with characteristic skin lesions with or without a history of respiratory illness.

Large Plantar Mass in a Newborn

A 1-day-old white boy was seen by the dermatology service for a congenital mass on the plantar surface of his left foot. The mass appeared to be slightly tender. There had been no episodes of bleeding. The infant was born at term, and there were no complications during pregnancy or delivery. He was otherwise healthy and taking no medications. On physical examination, he had a 3.5 × 5 cm, slightly firm, violaceous tumor on the medial plantar surface of his left foot extending between the first and second toes. A 0.5-cm crusted erosion over the first metatarsal head was also seen. The periphery of the tumor had a grayish hue and was softer with a lax, convoluted appearance (Fig. 1). The infant withdrew his foot upon palpation of the mass. No other lesions were noted. Ultrasound showed a solid, superficial mass with no appreciable blood flow. Plain radiographs of the left foot revealed a soft tissue mass with small areas of mineralization. Forefoot adductus and a shortened second metatarsal were also noted on radiograph. Magnetic resonance imaging (MRI) showed a soft tissue mass on the plantar surface of the left foot extending into the deeper tissues between the first and second digits. There was diffuse enhancement of the lesion following gadolinium administration. A 4-mm punch biopsy specimen was obtained. The hematoxylin–eosin-stained specimen showed a spindle cell proliferation with abundant small, narrow, mildly branching vascular channels extending into the deep dermis (Fig. 2A,B). Mitotic figures were present (0–2 per 10 high power fields), but no cytologic atypia was identified. Immunohistochemistry stains were positive for vimentin and smooth muscle actin, but negative for desmin and S-100. CD31 was positive in endothelium lining the vasculature.

White Papules in a Child with Down Syndrome

An 11-year-old white girl with Down syndrome was referred for evaluation of numerous white cutaneous papules. The lesions were first noticed on the dorsal aspects of the hands at 4 years of age. New lesions developed on her palms, the tops of her feet, and periorbital/perioral areas over the next several years. There was no history of trauma to the involved areas. The lesions were asymptomatic and many spontaneously resolved without scarring. Past medical history was significant for an atrial-septal defect repaired at 15 months of age, tonsillectomy and adenoidectomy in 1993, cardiac valve surgery in 1996, and sinus surgery in June 2000. The patient had no history of renal failure/ stones, chronic abdominal pain, or skeletal fractures. She was taking no medications or supplements. On examinations, the patient had multiple 1–2 mm, firm, white papules on the dorsal surfaces of her hands and feet, with a few similar lesions on her palms and periorally (Figs. 1 and 2). There was mild erythema surrounding a few lesions, but no livedo reticularis, tissue necrosis, or ulceration. Skin examination was otherwise unremarkable. Serum calcium, phosphorus, parathyroid hormone, 25-hydroxyvitamin D, and 24hour urinary calcium levels were all within normal limits. A shave biopsy of one of the papules was obtained prior to referral to our clinic. WHAT IS YOUR DIAGNOSIS?