Tollefson, Megha M.

AbstractBackgroundLeukocytoclastic vasculitis (LCV) in children is a complex group of conditions.ObjectivesThis study presents the demographics, clinical features, direct immunofluorescence (DIF) results and suspected aetiologies of 56 biopsy‐confirmed cases of leukocytoclastic vasculitis in children.MethodsRetrospective review of 56 children seen at Mayo Clinic in Rochester, Minnesota, from 1993 to 2013 with clinical features and cutaneous biopsy consistent with LCV.ResultsTwenty‐seven (48%) cases were found to be due to IgA vasculitis (Henoch‐Schonlein purpura). The remaining cases were found to be due to cutaneous small‐vessel vasculitis (n = 19, 34%), urticarial vasculitis (n = 5, 9%), ANCA‐associated vasculitis (n = 4, 7%) and acute haemorrhagic oedema of infancy (n = 1, 2%). IgA vasculitis was found to be associated with abdominal pain (P = 0.008), whereas the non‐IgA vasculitis group was associated with headache (P = 0.052). Children with IgA vasculitis had palpable purpura (P = <0.001), petechia (P = 0.057), vesicles (P = 0.009) and involvement of the buttock (P = 0.004) more frequently than the non‐IgA vasculitis group. On DIF, perivascular IgA was positive in IgA vasculitis compared to non‐IgA vasculitis cases (P = <0.001), the other conjugates were similar between the two groups.ConclusionThe most common subtype of biopsy‐confirmed LCV in children is IgA vasculitis. Clinical features, exam characteristics and DIF results can be helpful in determining the subtype of cutaneous vasculitis in children.

AbstractPemphigus 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.

Abstract Lichen planopilaris ( LPP ) is a rare form of cicatricial alopecia that has occasionally been reported in children. Because of the limited number of patients reported, little information is available about demographic characteristics, clinical presentation, or treatment options for these patients. A retrospective chart review of LPP cases in patients under 18 years of age from 1976 to 2013 was performed to further define clinicopathologic features of pediatric LPP . Four pediatric LPP patients ages 13 to 16 years were identified (three male, one female). One patient had scalp pruritus and one had other cutaneous findings of lichen planus ( LP ). Perifollicular scale and scarring were the most common physical examination findings, although changes mimicking those of alopecia areata were observed. Three patients were treated with topical or intralesional steroids. One patient was treated with minocycline. Histopathologic findings included perifollicular interface and perifollicular fibrosis in all cases. There was focal interfollicular interface in two cases and mild dermal mucin in one case. LPP is exceedingly rare in children. It may be misdiagnosed as alopecia areata in children because of the lack of symptoms and other features of LP . There should be a high index of suspicion for LPP in children with alopecia that is unresponsive to standard treatment or who have findings that are atypical for more common childhood alopecias.

BACKGROUND AND OBJECTIVES: Infantile hemangiomas (IH) are recognized as growing rapidly during the first months of life, but details of early growth before 3 months of age have not been well-characterized. Our goal was to study early IH growth by using parental photographs of infant children with facial IHs to better understand early hemangioma growth, with the aim of improving guidance for physicians and parents of infants with high-risk IH. METHODS: Serial images of 30 infants showing IH at intervals of 1 to 2 weeks up to 6 months were analyzed for characteristics of color, thickness, and distortion of anatomic landmarks. The presence or absence of an IH precursor at birth was noted. Mean scores per age interval were compiled. Results were analyzed by using signed rank test. An assessment of “optimal time for referral” was made. RESULTS: IH growth was nonlinear; most rapid growth occurred between 5.5 and 7.5 weeks of age. The mean “optimal age for referral” was 4 weeks of age. Hemangioma precursors were present at birth in 65% of patients. CONCLUSIONS: The most rapid hemangioma growth occurs before 8 weeks of age, much earlier than previously appreciated. Specialty evaluation and initiation of treatment, however, typically occur after the age of most rapid growth. Our findings suggest a need for a paradigm shift in the timing of referral and initiation of treatment of high-risk IH so that therapy can be initiated before or early in the course of most rapid growth, rather than after it is already completed.

Aplasia cutis congenita (ACC) is a rare disorder that is estimated to affect approximately three newborns in every 10,000 live births. It is usually detected at birth and most commonly affects the scalp as a solitary lesion. The type of lesion may be classified into one of nine groups. Membranous types and those with a “hair collar” are at highest risk to be associated with underlying neurodevelopmental abnormalities. In rare cases, associated abnormalities and syndromes may also be seen. Appropriate evaluation can be undertaken depending on clinical signs and symptoms. Treatment is often conservative, but more aggressive treatment may be indicated for some larger scalp lesions.