A case of mevalonate kinase deficiency, neonatal Sweet syndrome, and inflammatory bowel disease.

Mevalonate kinase deficiency is a group of rare metabolic autoinflammatory disorders that present with recurrent fevers, abdominal pain, arthralgias, adenopathy, and a variety of cutaneous manifestations. The skin findings may mimic cellulitis, erythema elevatum diutinum, IgA vasculitis, and Sweet syndrome, and there is often a morbilliform or urticarial rash and aphthous stomatitis. Mevalonate kinase deficiency is one of the identified monogenic variants that can cause very early onset inflammatory bowel disease (IBD).

Persistent, Pruritic, Reddish-Brown Papules and Plaques: An Atypical Presentation of Adult-Onset Still's Disease.

Adult-onset Still's disease (AOSD) is a systemic inflammatory condition characterized by recurrent fevers and a dermatologic eruption. The eruption is classically described as migratory and evanescent, composed of salmon-pink to erythematous macules, patches, and papules. However, a much rarer skin rash can also be seen in the setting of AOSD.

Wolf's isotopic response: A case of dermatomal granuloma annulare after COVID-19 vaccination.

The SARS-CoV-2 pandemic was announced in March 2020, with the first vaccines becoming available later that year. Although generally well tolerated, there have been reports of skin reactions occurring after receiving COVID-19 vaccines, with a rare occurrence of granuloma annulare. Granuloma annulare can occur secondarily to herpes zoster infection in a phenomenon known as Wolf's isotopic response (WIR).

Asymptomatic Plantar Nodules in a Toddler.

Precalcaneal congenital fibrolipomatous hamartoma (PCFH) is a rare benign skin lesion that typically presents at birth, or within the first several years of life, as single or multiple asymptomatic skin-colored papules or nodules on the plantar heels. We present a classic case of PCFH in a 3-year-old child. This uncommon entity has no reported malignant features or malignant transformations.

A Likely Case of Abemaciclib-Induced Hyperpigmentation in a Patient With Metastatic Breast Cancer.

We report a case of a 64-year-old female with a past medical history of invasive right breast adenocarcinoma presented with diffuse hyperpigmentation of her skin after admission to the hospital for an infected breast implant. She had no recollection of a similar cutaneous reaction in her past. The patient had been on a chronic regimen of anastrozole and abemaciclib for her metastatic breast cancer.

Congenital cutaneous candidiasis in a full-term neonate.

We present a case of a full-term neonate born with respiratory distress and a widespread erythematous rash, who was found to have congenital cutaneous candidiasis (CCC). The significance of this report is to contribute to the pre-existing literature on the rarity of CCC, but also to share a case of a patient who was successfully treated conservatively with topical antifungal agents only.

Float like a Butterfly, Sting like One Too.

Rash after contact with butterflies has not been previously reported in the medical literature to our knowledge. We describe potentially the first suspected case of a cutaneous reaction to the Compton tortoiseshell butterfly (Nymphalis vaualbum) in a young boy who developed urticaria after the modified hairs of the butterfly embedded within his finger. His urticaria improved through treatment with oral and topical steroids as well as systemic antihistamines.

Novel neonatal presentation of megalencephaly-polymicrogyria-pigmentary mosaicism syndrome (MPPM) related to MTOR mutation: Report of a case.

The PI3K/AKT/mTOR signaling pathway is a critical mediator of cell functions. Activating mutations of this pathway are known to disturb normal growth and development, leading to a range of overgrowth and neurologic syndromes. We report a case of megalencephaly-polymicrogyria-pigmentary mosaicism syndrome (MPPM) in novel association with MTOR pathogenic variant c.

Impact of skin biopsy on the management of acute graft-versus-host disease in a pediatric population.

Background/objectives: Acute graft-versus-host disease (GVHD) of the skin is a common complication of hematopoietic stem cell transplantation (HSCT) but often represents a diagnostic challenge. The adult literature suggests that histopathology rarely dictates management decisions, but the clinical utility of skin biopsies in pediatric patients with suspected acute GVHD is unknown. The objective of this study was to determine the frequency with which skin biopsy leads to a definitive diagnosis of acute GVHD and changes the management of acute GVHD in the pediatric population.

Linear morphea overlying site of previous lichen aureus in a pediatric patient.

Lichen aureus is a pigmented purpuric dermatosis that tends to occur on the lower extremities, and linear morphea is an inflammatory disease of the dermis and subcutaneous fat that leads to a scarlike induration. We present a pediatric case of linear morphea developing in the same anatomic distribution as prior lichen aureus. Due to the possible progression of lichen aureus to morphea described herein and the difficulty in diagnosing early morphea, close follow-up may be considered for pediatric patients who present with this pigmented purpuric dermatosis.

Herpes zoster at the vaccination site in immunized healthy children.

In this case series, we report seven immunized healthy children without underlying immunodeficiency who presented with herpes zoster that correlated with varicella-zoster vaccination site. The morphology of the lesions included erythematous papules, pseudovesicles, and plaques, with associated pain in two and pruritus in three patients; systemic symptoms ranged from none to low-grade fevers, upper respiratory symptoms, and joint pain. These cases highlight the clinical, diagnostic, and therapeutic implications of herpes zoster in vaccinated children.

Holmes heart and tetralogy of Fallot in association with PHACE.

PHACE is an association between large infantile hemangiomas and brain, arterial, cardiac, and/or ocular abnormalities. Aortic or subclavian aberrations are the most common cardiovascular anomalies in PHACE, whereas complex congenital heart disease is rare. We report a case of Holmes heart and three cases of tetralogy of Fallot in PHACE association.

Subcutaneous granuloma annulare involving the scalp.

Subcutaneous granuloma annulare (SGA) is an uncommon subtype of granuloma annulare. There are few reports of this entity solely affecting the scalp. We report a case of biopsy-proven SGA in a 21-month-old boy with six asymptomatic, rock-hard scalp nodules.

Self-limited neonatal periumbilical erythema.

We report three cases of neonatal, noninfectious, periumbilical erythema that resolved shortly after umbilical stump detachment. We hypothesize that these infants experienced an inflammatory and vasodilatory response during the normal umbilical cord separation process. We propose a new term: self-limited neonatal periumbilical erythema.

Vesiculobullous and hemorrhagic erythema migrans: uncommon variants of a common disease.

Background: The diagnosis of Lyme disease relies on the accurate diagnosis of erythema chronicum migrans (ECM) because serologic tests, culture, and polymerase chain reactions are often inaccurate. Although ECM is classically associated with a targetoid rash, there are many variants of this lesion. These variants of ECM are often initially diagnosed as cellulitis or spider bite reactions and treated with oral antibiotics.

Update on topical glucocorticoid use in children.

Purpose Of Review: This monograph reviews recent studies, examining topical glucocorticoid use in children. Emphasis is placed on mechanism of action, relative potency, cutaneous absorption, adverse affects, steroid phobia, and treatment compliance.

Recent Findings: Recent literature has shown that over 80% of patients prescribed topical glucocorticoids are fearful of side effects and fail to use them appropriately.