Jennifer J. Schoch

Pediatric Pyoderma Gangrenosum: A Retrospective Review of Clinical Features, Etiologic Associations, and Treatment

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis rarely seen in children. Its features have not been well characterized in children. We sought to characterize the clinical features, etiologic associations, and treatment of PG in children younger than 18 years.

Impact of childhood psoriasis on parents of affected children

Background: Childhood diseases, such as atopic dermatitis, have a negative impact on quality of life (QoL) of parents. How pediatric psoriasis affects a parents QoL is unknown. Objective: To explore the impact of childhood psoriasis on the lives of the parents. Methods: Semistructured interviews were conducted with 31 parents of children with psoriasis. Narrative data were analyzed and a conceptual framework of the effects of childhood psoriasis on parents was developed. Results: All parents reported that their childs psoriasis caused a substantial, negative impact on their own QoL. A conceptual framework showed a negative impact on health and self‐care, emotional well‐being, family and social function, personal well‐being, and life pursuits. Emotional well‐being was the most affected domain. Limitations: It was not possible to correlate psoriasis severity with parental QoL. Conclusion: Childhood psoriasis alters the QoL of parents in multiple ways. Information from this study can be used to develop a QoL instrument to explore treatment and support strategies for families affected by pediatric psoriasis.

Penile and Scrotal Swelling: An Underrecognized Presentation of Crohn's Disease

Background: Penile and scrotal swelling can occur as an extraintestinal manifestation of Crohns disease (CD) and is thought to be an uncommon form of metastatic CD (MCD). Because of the rarity of this manifestation, much is unknown concerning the presentation, treatment, and response to therapy in children with genital MCD. Methods: Boys ages 1 to 17 years presenting with genital edema and a confirmed diagnosis or strong suspicion of CD who were evaluated at the Mayo Clinic between 1996 and 2014 were included for review. We sought to characterize the clinical and pathologic features of genital MCD and response to treatment in our cohort of patients. Results: Eight patients with genital MCD were identified from our institution (mean age 11.4 yrs, range 7–16yrs). Seven (88%) patients experienced cutaneous symptoms before a formal diagnosis of CD was made, and two of the seven had no adverse gastrointestinal symptoms at that time. Patients were prescribed an average of 3.4 medications (topical and systemic) for management of their gastrointestinal CD and MCD. Conclusions: Penile and scrotal swelling can occur as an extraintestinal manifestation of CD and is thought to be an uncommon form of MCD. In boys, genital swelling typically precedes intestinal CD. Treatment of the underlying CD with systemic medications was most helpful in this series. An evaluation for CD is necessary in all patients presenting with unexplained genital swelling.

Pediatric subacute cutaneous lupus erythematosus: report of three cases

An 11-year-old boy presented to our dermatology department (Mayo Clinic, Rochester, MN, USA) with a 3-month history of a rapidly enlarging annular lesion on his left cheek (Fig. 1), followed by development of a lesion in the left postauricular area. The skin lesions had been unresponsive to treatment with topical antifungals and topical corticosteroids. He also received a trial of doxycycline for suspected Lyme disease, despite negative Borrelia serology. After treatment with doxycycline, the rash worsened, and headache, fatigue, and arthralgias developed. Serologic testing revealed: positive antinuclear antibodies (ANAs) (>12.0 U [reference range <1.0 U]); SS-A/RO antibodies (>8.0 U [reference range <1.0 U]); SS-B/LA antibodies (>8.0 U [reference range <1.0 U]); and antihistone antibodies (2.2 U [reference range <1.0 U]). Anti-dsDNA, RNP, SM, JO 1, and SCL70 antibodies were not detected. Mild lymphopenia was also noted (nadir at 1.02 9 10/l [reference range, 1.20–5.20 9 10/l]). Complement levels were not assessed. Histopathologic evaluation of a skin biopsy from the left cheek lesion showed vacuolar interface dermatitis with perivascular and periappendageal, predominately lymphocytic inflammation and dermal mucin deposition, consistent with lupus erythematosus (LE) (Fig. 2). Although he reported mild systemic symptoms, he did not meet the American College of Rheumatology criteria for a diagnosis of systemic lupus erythematosus (SLE); the diagnosis of SCLE was made based on characteristic clinical findings. Within six weeks after starting hydroxychloroquine and strict photoprotection, the skin lesions and systemic symptoms resolved. After more than one year of follow-up, he has continued to do well, without evidence of systemic symptoms indicative of SLE.

Successful Treatment of Keratosis Pilaris Rubra with Pulsed Dye Laser

Keratosis pilaris rubra is a common but rarely reported condition characterized by follicular‐based hyperkeratotic papules on a background of erythema. It can be embarrassing and symptomatic for patients, particularly adolescent boys. We sought to explore the efficacy of pulsed dye laser (PDL) in the treatment of keratosis pilaris rubra.

Chronic urticarial eruption associated with monoclonal gammopathy

Schnitzler syndrome is a rare acquired autoinflammatory disease characterized by monoclonal immunoglobulin M gammopathy and urticaria. Clinical features include fever, urticarial rash, myalgia, arthralgia, hepatosplenomegaly, and enlarged lymph nodes. Schnitzler syndrome is often underdiagnosed and not usually suspected until more common autoimmune, neoplastic, and infectious etiologies are excluded. Anakinra, an interleukin-1 receptor antagonist, is the first-line treatment and has been shown to provide fast and sustained symptom relief [1–3]. Image 1. Schnitzler Syndrome. A: Small, slightly edematous pink plaques on the back. B: Close-up of plaques on the arm. C: Axial computed tomography image of the pelvis from the whole-body FDG positron emission tomography/computed tomography. Patchy intramedullary osteosclerosis involving the left iliac wing (arrows). D: Skin histologic results showing predominant neutrophilic perivascular inflammation with scattered eosinophils (Hematoxylin–Eosin; original magnification, 203).

Scar Reactivation in a Postpartum Woman

A 33-year-old womanwas referred by her primary health care provider for increasing prominence of facial scars. The scars were sustained during a bicycle accident at age 5 years and were minimally appreciable until 10 weeks after the delivery of her third child. At that time, the patient noted the sudden development of linear pink, thin plaques within the scar sites. The lesions developed a deeper red to reddish yellow color over the next 2 months, and she was referred to the dermatology department (Figure 1). Dermoscopy revealed an “apple jelly” appearance to the lesions. Previous treatments included topical corticosteroids, with minimal improvement. Her medical and obstetric history, which included 2 prior pregnancies, was unremarkable. Two 3-mm punch biopsy specimens were obtained from the lesions for hematoxylin-eosin staining. What is your diagnosis?

Eccrine hamartoma with spectrum of histologic findings associated with limb deformity

A 12-year-old male with no significant past medical history presented for evaluation of a solitary painful plaque located on his right medial plantar foot. The lesion was first noticed approximately 3 years prior after mild trauma sustained while falling off his bicycle. Following this trauma, a brown hyperpigmented patch formed (Fig. 1), and the patient began to notice progressive pain and difficulty with ambulation. Subsequent compensatory supination of the right foot ensued and has since manifested in gross size discrepancy between the right and left foot. Initial treatment with casting, boots, and hydrocortisone resulted in no improvement of the patient’s symptoms.

Compound Heterozygosity of Dominant and Recessive COL7A Alleles in a Severely Affected Patient with a Family History of Dystrophic Epidermolysis Bullosa: Clinical Findings, Genetic Testing, and Treatment Implications

An 8‐year‐old girl born to a family with more than three generations of dominant dystrophic epidermolysis bullosa (DDEB) presented with life‐threatening confluent skin erosions, mitten hand deformity, and failure to thrive. Reassessment of her family history and genetic testing showed compound heterozygous COL7A mutations, one inherited from her DDEB‐affected mother and one from her unaffected, healthy father. This family illustrates the risk of unexpected, severe, autosomal recessive epidermolysis bullosa (EB) in a family with milder, multigenerational autosomal dominant EB. Clinicians should recognize the clinical spectrum of dystrophic EB and recommend genetic consultation when the phenotype conflicts with family history.

Transplacental Transmission of Pemphigus Herpetiformis in the Setting of Maternal Lymphoma

Pemphigus herpetiformis (PH) is characterized by grouped vesicular or papular pruritic lesions with histologic and immunopathologic features of pemphigus. PH can manifest at any age, and paraneoplastic cases have been reported. We describe a healthy boy born with acral crateriform erosions of the hands and feet whose 35‐year‐old mother had similar lesions. Biopsies from both patients were most consistent with PH. The mother was diagnosed with high‐grade B‐cell non‐Hodgkin lymphoma and began chemotherapy with dexamethasone, and her lesions quickly improved. The infant had no additional lesions after 3 weeks of age and his acral erosions healed. To our knowledge, this is the first report of a diagnosis of PH in an infant and the first case of transplacental transmission of PH. This is also the first report of paraneoplastic PH in lymphoma. This case adds to the growing differential diagnosis of skin disease in postpartum women and their neonates.