Cutis verticis gyrata associated with congenital heart disease.

Cutis verticis gyrata (CVG) is a very rare benign disorder characterised by convoluted folds and deep furrows of the scalp that mimic cerebral sulci and gyri. Associations with other pathologies as neuropsychiatric and/or ophthalmologic disorders, secondary cases to inflammatory or neoplastic processes, as well as cases associated to genetic disorders as Turner's syndrome have been reported, but there is no literature describing an association with a congenital structural heart defect and no other underlying condition. We report a case of primary CVG in a 3-week-old female infant associated with an echocardiographic diagnosis of cor triatriatum. Other systemic examination findings and investigations were unremarkable, and the patient has normal neurodevelopment at 1 year old. Aside from the neuropsychiatric and ophthalmologic pathologies commonly associated with primary non-essential CVG, it should be noted that isolated congenital cardiac lesions are also possible, so as to increase our index of suspicion in patients with the disorder.

Dynamic evolution of a scalp congenital melanocytic nevus with poliosis and cutis verticis gyrata.

Cutis verticis gyrata (CVG), characterized by cerebriform overgrowth of the scalp, is rarely observed in congenital melanocytic nevi (CMN). We describe a 13-year-old male with autism and a large CMN of the scalp with numerous satellite nevi whose scalp nevus exhibited evolution with poliosis and CVG. Given the potential association of CVG (independent of CMN) with seizures, neuropsychiatric, and ophthalmologic disorders, and nevus-associated CVG (cerebriform intradermal nevus) with melanoma, multidisciplinary evaluation of CMN patients with CVG is important to guide management and treatment.

Localized alopecia with itching on the scalp.

We describe the use of trichoscopy to arrive at a diagnosis, and discuss the possible differential diagnoses in a patient presenting with localized itching and alopecia of the scalp.

Cutis verticis gyrata: a cutaneous finding in acromegaly.

Acromegaly is a rare disease characterized by changes in the bone and soft tissue systems, induced by excess growth hormone and insulin-like growth factor type 1. Among the skin lesions associated with acromegaly is cutis verticis gyrata, an hypertrophic, and coarse folding of the skin of the scalp, an association of uncommon incidence and unknown prevalence. This case report describes the case of a patient diagnosed with acromegaly at age 60 with previously unidentified cutis verticis gyrata. This report aims to review the literature on cutis verticis gyrata and its unusual association with acromegaly.

Regression of lipoedematous scalp following cryolipolysis using an icepack, with a novel dermoscopic feature of yellow background.

The novel use of cryolipolysis via icepacks, a readily available and inexpensive device, is described for lipoedematous scalp with subjective and objective relief. This is a simple solution to a troublesome, intractable condition and may readily be utilized for patient benefit. Juxtaposing pre and post clinical images.

Primary nonessential cutis verticis gyrata with acne keloidalis nuchae.

Cutis verticis gyrata is a rare disorder characterized by redundant skin forming deep furrows and convolutions. It has been associated with several systemic and cutaneous disorders. We report a case of primary non-essential cutis verticis gyrata in association with acne keloidalis nuchae in a schizophrenic patient.

[Alopecia and cancers: From basics to clinical practice]. / Alopécie et cancers : de la physiopathologie à la pratique clinique.

Alopecia, although long considered an unavoidable consequence of cancer therapy, currently presents a multifaceted challenge. The knowledge of the physiology of the hair and consequently of the pathophysiology of alopecia has led to show that there is not one but several types of alopecia. Transposed to the world of oncology, different types of alopecia and subsequently molecular pathways have been characterized, allowing a better understanding of the underlying mechanisms. Thus, in patients with cancer, alopecia can be iatrogenic (chemotherapies, endocrine therapies, targeted therapies, immunotherapies, radiotherapy, surgery) or directly the consequence of the disease itself (malnutrition, scalp metastases, paraneoplastic syndromes). Knowledge of the incriminated mechanism(s) could thus make it possible to deploy an appropriate care component, whether on the preventive or curative sides or in terms of supportive care. These are particularly essential regarding the psychological repercussions caused by alopecia, with significant consequences on the quality of life of patients and with a potential impact on treatment compliance. On the preventive side, the last few years have seen the advent of the automated scalp cooling therapy, supported by several randomized clinical trials. On the curative side, several therapeutic proposals are currently deployed or under development in order to provide relevant treatments.

Lichen planopilaris with significant post-inflammatory pigmentary alteration.

Lichen planopilaris is an uncommon dermatological manifestation of lichen planus of the scalp and results in cicatricial alopecia. We present a patient with lichen planopilaris and significant post-inflammatory pigmentary alteration, confirmed by histopathology. The patient's case represents a clinically important variation from an expected typical pattern of dyschromia at periphery of alopecic zones in lichen planopilaris.

Erosive pustular dermatosis in a patient with lichen planopilaris treated with aminolevulinic acid photodynamic therapy.

Erosive pustular dermatosis of the scalp (EPDS) is a chronic inflammatory condition with an unclear etiology that typically occurs on sun-damaged skin of older individuals. Clinically it is characterized by sterile pustules, erosions, and crusting that can lead to scarring alopecia and skin atrophy (Yang et al., 2016; Broussard et al., 2012; Cunha et al., 2019). EPDS is a therapeutic challenge and often resistant to numerous treatments. Herein, we describe a case of erosive pustular dermatosis of the scalp in a patient with lichen planopilaris successfully treated with curettage followed by aminolevulinic acid photodynamic therapy.

Beare-Stevenson Syndrome With Blepharoptosis as a Complication of Front-Orbital Advancement and Remodeling.

Beare-Stevenson syndrome (BSS) is an extremely rare genetic disorder characterized by a broad range of congenital malformations including craniosynostosis, cutis gyrata, facial deformities, and abnormal genitalia. The authors report a case of a 7 month old female who developed a mechanical ptosis secondary to dermatochalasis as a complication of fronto-orbital advancement and remodeling (FOAR) surgery which subsequently required multiple lid surgeries to reverse ptosis. This is the first report of blepharoptosis correction in a child with BSS as a complication of FOAR.

Diffuse variants of scalp lichen planopilaris: Clinical, trichoscopic, and histopathologic features of 40 patients.

BACKGROUND: Fibrosing alopecia in a pattern distribution and cicatricial pattern hair loss are poorly recognized diffuse variants of lichen planopilaris (LPP). OBJECTIVES: The medical features of 40 patients affected by a diffuse hair thinning associated with a long-lasting history of pruritus and erythema of the scalp and a histopathologic diagnosis of LPP were reviewed. METHODS: Clinical data, results of trichoscopy and histopathology, response to treatment, and follow-up were analyzed. RESULTS: There were 18 patients diagnosed with fibrosing alopecia in pattern distribution and 2 patients with cicatricial pattern hair loss. A new variant of diffuse LPP, named "lichen planopilaris diffuse pattern," was described in 20 individuals. LIMITATIONS: Low number of cases due to rarity of the diseases. CONCLUSION: In patients complaining of a long-lasting history of scalp erythema, itching/dysesthesia, and diffuse hair thinning, it is advisable to consider diffuse variants of LPP.

Seborrhea herpeticum: cutaneous herpes simplex virus infection within infantile seborrheic dermatitis.

Eczema herpeticum has been well described in the setting of atopic dermatitis (AD) and other dermatoses. We present the case of a 2-month-old infant boy with cutaneous herpes simplex virus (HSV) infection within existing diffuse infantile seborrheic dermatitis. Providers should be aware that cutaneous HSV can be confined to a seborrheic distribution and may represent underlying epidermal dysfunction secondary to seborrheic dermatitis.

Dilation of Multiple Eccrine Ducts as a Highly Specific Marker for Cicatricial Alopecia.

BACKGROUND: Eccrine duct dilation (EDD) was recently described to occur more frequently in cicatricial alopecias than noncicatricial alopecias. Because single EDD can be useful in the evaluation of alopecias, we aimed to determine whether dilation of multiple eccrine duct units, or "multiple eccrine duct dilation (MEDD)," could more specifically discriminate between cicatricial and noncicatricial alopecias. METHODS: We retrospectively evaluated 611 scalp biopsies (342 cicatricial alopecias and 269 noncicatricial alopecias). RESULTS: Among cicatricial alopecias, MEDD was found in 21% (25/118) of central centrifugal cicatricial alopecia, 26% (29/109) of lichen planopilaris, 13% (10/73) of discoid lupus erythematosus, 31% (5/16) of acne keloidalis nuchae, and 26% (7/26) of folliculitis decalvans. In noncicatricial alopecias, MEDD was found in 1% (1/102) of androgenetic alopecia, 0.7% (1/150) of alopecia areata, and 0% (0/17) of telogen effluvium. In cicatricial alopecias, MEDD occurred in a significantly higher frequency (22%; 76/342) compared with noncicatricial alopecias (0.7%; 2/269) (P-value <0.0001). The presence of MEDD correlated with a diagnosis of cicatricial alopecia with 22% sensitivity and 99% specificity. MEDD also occurred more frequently in cases with moderate to severe inflammation and fibroplasia, suggesting that EDD is a reactive change secondary to the scarring processes. CONCLUSION: The presence of MEDD on scalp biopsies may be a highly specific marker of cicatricial alopecia and can aid in rendering a more accurate diagnosis. MEDD without other definitive histopathologic features of cicatricial alopecia may compel pathologists to pursue additional workup and/or raise the possibility that a cicatricial alopecia cannot be entirely excluded.

Nasal continuous positive airway pressure with head cap fixation as a contributing factor to extensive scalp necrosis in a preterm neonate with early-onset sepsis and scalp hematoma.

BACKGROUND: Nasal continuous positive airway pressure (CPAP) is widely used in the treatment and prevention of respiratory distress in preterm neonates, with only few severe adverse skin effects reported. CASE PRESENTATION: A preterm neonate was born at 34 + 1 weeks of gestation, birth weight 1860 g, and presented with early-onset sepsis (EOS) and scalp hematoma. He developed respiratory distress day 2 after birth. Antibiotics, nasal CPAP and other supportive treatment were initiated. A scalp hematoma in the occipital region was complicated by nasal CPAP cap pressure leading to an extensive scalp necrosis equaling 6% of the total body surface. Debridement and skin grafting were performed day 11, and 51, respectively. The boy survived with good healing of the skin graft. CONCLUSION: The nasal CPAP head cap contributed to the development of severe, but potentially preventable, scalp necrosis in a preterm with birth-related scalp skin injury and EOS.