Ultrasound Pattern of Cutis Verticis Gyrata.

Cutis verticis gyrata (CVG) is a cutaneous benign condition that usually presents symmetric or asymmetric convoluted folds and deep furrows in the scalp, which mimic the disposition of the cerebral sulci and gyri. This scalp deformation may be a worrying situation for the patients and their families. Clinically, its diagnosis may be challenging because it can mimic other cutaneous conditions. So far, the ultrasonographic pattern of CVG has not been reported. Thus, we aim to review the ultrasound findings of this entity. All patients (n = 14) presented zones with dermal and hypodermal thickening that corresponded with the elevated clinical zones, followed by folds with normal cutaneous thicknesses. There was undulation of the cutaneous layers in all cases. The scalp areas involved the frontal, parietal, and occipital regions bilaterally. The mean maximum dermal and hypodermal thicknesses and echogenicities and the color Doppler characteristics are provided. No significant dilation of the hair follicles was detected. In conclusion, CVG presents an ultrasonographic pattern that can support its diagnosis and follow-up. This can help its differential diagnosis with other scalp dermatologic conditions.

Pyogenic granuloma of the scalp arising from a retained bullet fragment after a contralateral skull-penetrating gunshot wound.

Pyogenic granulomas are benign lesions commonly found on the skin and mucosal surfaces. Although the etiology of pyogenic granuloma is not well understood, some reports have suggested that PG may be caused by impaired wound healing following tissue injury. Rare cases of pyogenic granulomas in the setting of local foreign body have been reported in the literature. Although foreign body reactions have not been identified as a cause of pyogenic granuloma, these reports evidence the need to further investigate this association. We present a 33-year-old man who presented with a pyogenic granuloma on the scalp six years after a contralateral skull-penetrating gunshot wound that resulted in retained bullet fragments.

Cutis verticis gyrata in a patient with acromegaly: an unusual case and review of literature.

Acromegaly is a rare systemic syndrome induced by the overproduction of growth hormone (GH) and insulin-like growth factor type one (IGF1). It is responsible for changes in the skeletal and soft tissue systems and it almost always occurs because of a pituitary adenoma. Amongst the skin complications related to acromegaly, cutis verticis gyrate (CVG) is occasionally found. It is a skin condition characterized by excessive growth of the skin of the scalp, resulting in furrows and folds. Only a few cases of this uncommon association have been reported in the literature. The present clinical case illustrates typical CVG associated with acromegaly. Imaging revealed a pituitary macroadenoma lesion and hormonal evaluation revealed elevated IGF1 and hypopituitarism. The patient underwent a transsphenoidal resection of the pituitary adenoma and the histopathological examination confirmed the diagnosis. The diagnosis of CVG is clinical, so radiologic assessments are generally not necessary. The management of acromegaly associated with CVG depends on controlling the serum levels of GH and IGF1. In some cases, specific injections or surgery can be used to minimize CVG.

Nonmelanoma skin cancer in the setting of erosive pustular dermatosis of the scalp: A case series and comment on management implications.

BACKGROUND: Erosive pustular dermatosis of the scalp (EPDS) is an inflammatory cutaneous disorder typically affecting sun-damaged skin of mature individuals. Clinical features of EPDS include sterile pustules and chronic crusted erosions that can be hyperkeratotic and lead to scarring alopecia, atrophy, and telangiectasia. While the condition occurs on sun-damaged skin, a relationship with non-melanoma skin cancer (NMSC) has not been investigated. OBJECTIVES: Here we attempted to identify cases of NMSC developing in the setting of EPDS. METHODS: Retrospective review of EPDS cases in a dermatology practice. RESULTS: Six patients with mean (range) age 82 (65-92) years that developed NMSC in the setting of EPDS are reported. Five patients had skin phototype I or II associated with substantial solar elastosis. Four patients had history of NMSC. Four patients developed squamous cell carcinoma and two patients basal cell carcinoma on the scalp in the setting of EPDS. A morphologic change in an EPDS lesion, such as a crusted plaque becoming nodular and/or growing significantly within a relatively short period of time, prompted a biopsy that revealed NMSC. CONCLUSIONS: NMSC may develop in the setting of EPDS. Possible mechanisms underlying this association include the chronic inflammation associated with EPDS and ultraviolet light exposure. It is crucial to promptly obtain a biopsy in EPDS cases showing signs suspicious for NMSC. Further studies are required to confirm whether NMSC shows a higher prevalence in the setting of EPDS.

Scalp involvement in patients referred for patch testing: Retrospective cross-sectional analysis of North American Contact Dermatitis Group data, 1996 to 2016.

BACKGROUND: Scalp conditions are often multifactorial. OBJECTIVE: To characterize patients with scalp involvement and patch-testing outcomes. METHODS: Retrospective cross-sectional analysis of North American Contact Dermatitis Group data (1996-2016). Study groups included patients with scalp involvement (≤3 anatomic sites coded) with or without additional sites. RESULTS: A total of 4.8% of patients (2331/48,753) had scalp identified as 1 of up to 3 affected anatomic sites. Approximately one-third of "scalp-only" individuals had a specific primary diagnosis of allergic contact dermatitis (38.6%), followed by seborrheic dermatitis (17.2%) and irritant contact dermatitis (9.3%). When adjacent anatomic sites were affected, allergic contact dermatitis was more frequently identified as the primary diagnosis (>50%). The top 5 currently clinically relevant allergens in scalp-only patients were p-phenylenediamine, fragrance mix I, nickel sulfate, balsam of Peru, and cinnamic aldehyde. Methylisothiazolinone sensitivity was notable when adjacent anatomic sites were involved. The top 3 specifically identified sources for scalp-only allergens were hair dyes, shampoo/conditioners, and consumer items (eg, hair appliances, glasses). LIMITATIONS: Tertiary referral population. CONCLUSION: Isolated scalp involvement was less likely to be associated with allergic contact dermatitis than when adjacent anatomic sites were involved. Overlap with multiple diagnoses was frequent, including seborrheic dermatitis, irritant dermatitis, other dermatoses, or all 3. p-Phenylenediamine was the most common allergen.

Expanding the phenotype in Adams-Oliver syndrome correlating with the genotype.

RATIONALE: Adams-Oliver syndrome (AOS) is a genetic disorder characterized by the association of aplasia cutis congenita (ACC), terminal transverse limb defect (TTLD), congenital cardiac malformation (CCM), and minor features, such as cutaneous, neurological, and hepatic abnormalities (HAs). The aim of the study is to emphasize phenotype-genotype correlations in AOS. METHODS: We studied 29 AOS patients. We recorded retrospectively detailed phenotype data, including clinical examination, biological analyses, and imaging. The molecular analysis was performed through whole exome sequencing (WES). RESULTS: Twenty-nine patients (100%) presented with ACC, the principal inclusion criteria in the study. Seventeen of twenty-one (81%) had cutis marmorata telangiectasia congenita, 16/26 (62%) had TTLD, 14/23 (61%) had CCM, 7/20 (35%) had HAs, and 9/27 (33%) had neurological findings. WES was performed in 25 patients. Fourteen of twenty-five (56%) had alterations in the genes already described in AOS. CCM and HAs are particularly associated with the NOTCH1 genotype. TTLD is present in patients with DOCK6 and EOGT alterations. Neurological findings of variable degree were associated sometimes with DOCK6 and NOTCH1 rarely with EOGT. CONCLUSION: AOS is characterized by a clinical and molecular variability. It appears that degrees of genotype-phenotype correlations exist for patients with identified pathogenic mutations, underlining the need to undertake a systematic but adjusted multidisciplinary assessment.

Scalp eschar and neck lymphadenopathy after tick bite (SENLAT) caused by Bartonella henselae in Korea: a case report.

BACKGROUND: Tick-borne lymphadenopathy (TIBOLA) is an infectious disease, mainly caused by species from the spotted fever group rickettsiae and is characterized by enlarged lymph nodes following a tick bite. Among cases of TIBOLA, a case of scalp eschar and neck lymphadenopathy after tick bite (SENLAT) is diagnosed when an eschar is present on the scalp, accompanied by peripheral lymphadenopathy (LAP). Only a few cases of SENLAT caused by Bartonella henselae have been reported. CASE PRESENTATION: A 58-year-old male sought medical advice while suffering from high fever and diarrhea. Three weeks before the visit, he had been hunting a water deer, and upon bringing the deer home discovered a tick on his scalp area. Symptoms occurred one week after hunting, and a lump was palpated on the right neck area 6 days after the onset of symptoms. Physical examination upon presentation confirmed an eschar-like lesion on the right scalp area, and cervical palpation revealed that the lymph nodes on the right side were non-painful and enlarged at 2.5 × 1.5 cm. Fine needle aspiration of the enlarged lymph nodes was performed, and results of nested PCR for the Bartonella internal transcribed spacer (ITS) confirmed B. henselae as the causative agent. CONCLUSION: With an isolated case of SENLAT and a confirmation of B. henselae in Korea, it is pertinent to raise awareness to physicians in other Asian countries that B. henselae could be a causative agent for SENLAT.

Erosive pustular dermatosis of the scalp following surgical procedures: a systematic review.

Erosive pustular dermatosis of the scalp (EPDS) occurs in elderly individuals with significant actinic damage. EPDS also occurs in association with surgery; however, significant studies determining an association of EPDS with type of surgical closure is absent. This review examines whether the closure method following cutaneous surgery performed on the scalp is associated with development of EPDS. Databases were reviewed and studies describing EPDS after cutaneous surgery met inclusion criteria. Articles were excluded if EPDS developed after trauma or non-surgical procedures. Descriptive analyses were performed on the data. Thirteen case reports and 6 case series involving 32 patients met inclusion criteria. Fourteen articles (73.7%) stated that EPDS developed in the same location as, or near to, the closure site. Thirteen patients (40.6%) developed EPDS following skin grafting. Three patients (9.4%) developed EPDS following secondary intention healing, two patients (6.3%) following repair by primary intention, and one patient (3.1%) following repair with a local skin flap. Thirteen cases (40.6%) did not specify closure type. This review revealed that surgical procedures performed on the scalp utilizing skin grafts for closure may be increasingly associated with the development of EPDS compared to other closure types.

Itching for an answer: A review of potential mechanisms of scalp itch in psoriasis.

Scalp psoriatic itch is a common complaint and often poses a therapeutic challenge. The pathophysiology of this phenomenon is unclear. The unique anatomy of the scalp contains richly innervated hair follicles, abundant vasculature and perifollicular inflammatory cytokines which may all contribute to this common sensory complaint. The mast cell, in particular, is portrayed as one of the main itch conductors for its ability to trigger neurogenic inflammation, activate the peripheral hypothalamic-pituitary-adrenal-axis, process and integrate itch signalling through its interactions with the scalp hair follicles. Herein, we explain and speculate upon potential mechanisms underlying itchy scalp psoriasis, involving interconnections between the neuroimmune, neurovascular and neuroendocrine systems. Many factors may play roles in itchy scalp psoriasis including the scalp hair structure, immune system, endocrine system, nervous system and vascular system. These may warrant further exploration as therapeutic targets that go beyond the application of mere anti-inflammatory agents.

Erosive pustular dermatosis of the scalp: A neutrophilic folliculitis within the spectrum of neutrophilic dermatoses: A clinicopathologic study of 30 cases.

BACKGROUND: It is general opinion that histopathology is nonspecific and of little value in diagnosing erosive pustular dermatosis of the scalp (EPDS). OBJECTIVES: Clinicopathologic correlation of erosive pustular dermatosis of the scalp. METHODS: We reviewed the clinical and pathologic records of patients with a clinicopathologic diagnosis of EPDS between 2011 and 2016 at the Dermatopathology Unit of Turin University. RESULTS: Thirty elderly patients with EPDS were identified (22 men and 8 women). Androgenetic alopecia was present in 19 of 30 patients. Triggering factors included mechanical trauma in 10 of 30 cases, surgical procedures in 4 of 30 cases, and herpes zoster in 1 of 30 cases. Three patients were affected by autoimmune disorders. The vertex was the most common location. Disease presentation varied markedly from tiny, erosive, scaly lesions to crusted and hemorrhagic plaques, mimicking pustular pyoderma gangrenosum. The pathologic changes differed according to lesion type and disease duration. Interestingly, a spongiotic and suppurative infundibulo-folliculitis was observed in 8 of 30 cases. LIMITATIONS: This was a retrospective study. CONCLUSIONS: We believe that the primary lesion of erosive pustular dermatosis of the scalp is a spongiotic, pustular superficial folliculitis. The clinicopathologic similarities with other neutrophilic dermatoses, such as pustular pyoderma gangrenosum, suggest this condition should be included in this spectrum, where pathergy plays a pathogenetic role.

Contact allergy to hair cosmetic allergens in Thailand.

BACKGROUND: Hair cosmetics such as hair dyes, bleaching, waving, and cleansing products are composed of numerous chemical ingredients. Allergic reactions to these contact allergens, other than p-phenylenediamine, are rarely reported. OBJECTIVES: To study the incidence of patch test reactions to hair cosmetic allergens and identify the factors associated with hair cosmetic allergy in Thailand. METHODS: We retrospectively reviewed the case records of patients who underwent patch testing from 2009 to 2018. Patients with at least one positive patch test reaction to a potential contact allergen associated with hair cosmetic ingredients were studied. RESULTS: Overall, 2842 patients were patch tested. Of the hair cosmetic allergen categories, preservatives had the highest rate of positive reactions, followed by surfactants and hair dyes. Perming agents were less problematic in comparison. The hands, head, and neck were the predominately affected sites of allergic reactions to hair cosmetic ingredients. CONCLUSIONS: Preservatives, surfactants, and hair dyes are important contact allergens, whereas perming agents are less likely to cause allergic reactions. Hair cosmetic ingredients are regulated by national agencies, which influences their extent of exposure and rates of contact allergies. Further continuous observation of hair cosmetic allergy is needed to provide the best patient care.

Erosive pustular dermatosis of the scalp - an Australian perspective: Insights to aid clinical practice.

Erosive pustular dermatosis of the scalp has particular relevance in Australia, due to its association with actinic damage. Despite its rarity, the recalcitrant nature of erosive pustular dermatosis of the scalp dictates a protracted recovery fraught with relapse and recurrence, posing inherent challenges to successful treatment and complete recovery. In Australia, erosive pustular dermatosis of the scalp is prevalent in the elderly, who are negatively affected due to the condition. We propose a management outline to aid clinical practice, to improve the quality of life in the elderly, whilst providing insight into the current understanding and treatment of erosive pustular dermatosis of the scalp.

Post-traumatic erosive dermatosis of the scalp: A hypergranulated variant.

Erosive pustular dermatosis of the scalp is a slowly progressive chronic inflammatory disease that predominantly affects elderly male patients with marked actinic damage. The clinical evolution consists firstly of keratotic and erosive plaques surmounted by yellow-brown crusts and non-follicular pustules; later, the active crusting lesions regress in number and the scarring process causes diffuse cutaneous thinning and loss of hair follicles. However, manifestations may be atypical, leading to frequent misdiagnosis. We present a case series of post-traumatic erosive pustular dermatosis on the scalp of 4 elderly patients. The characterising feature was the presence of erosion consisting of abundant hypergranulation tissue, with an almost total lack of crusts and pustules. Dermoscopy showed a unique pattern of stretched and dilated linear, telangiectatic and polymorphous on-focus vessels, milky-red areas and white scarring areas. This clinical entity is rarely reported in the literature. The majority of reported cases were located on the legs.

Adult-onset primary cyclic autoimmune neutropenia: a case report.

BACKGROUND: A few cases of primary autoimmune neutropenia (AIN) have been reported in adults, but cyclic primary AIN, which is characterized by the periodic oscillation of neutrophils, is uncommon in adults. STUDY DESIGN AND METHODS: Herein, we report a 70-year-old man referred to our hospital with severe neutropenia and thrombocytopenia. He had experienced intermittent episodes of low-extremity purpura for the past 3 months, with cellulitis on the skin of the scalp 1 month previously. RESULTS: The patient presented with severely low neutrophil and platelet (PLT) counts. Myeloid progenitors and megakaryocytes were increased in the marrow, but mature neutrophils were remarkably decreased. Anti-neutrophil antibodies to specific epitopes were detected at neutropenia. Based on these findings, AIN accompanied by autoimmune thrombocytopenia was diagnosed. The patient experienced synchronous fluctuations of neutrophil and PLT counts three times. Despite no treatment, the neutrophil count fluctuated within the range of 0.06 × 109 to 1.65 × 109 /L, and the PLT count fluctuated from 0.7 × 1010 to 20.5 × 1010 /L. We identified an inverse relationship between neutrophil count and anti-neutrophil antibody titers, establishing the conclusive diagnosis of cyclic AIN. After prednisolone treatment, the neutrophil and PLT counts normalized, and the patient has maintained long-term remission. CONCLUSION: We report a rare case of cyclic AIN diagnosed from the inverse association between periodic oscillation of anti-neutrophil antibody titers and neutrophil counts. This clinical course suggests that in AIN patients, laboratory data and recurrent signs of infection should be monitored regularly, including shortly after neutrophil recovery.

Fibrous cephalic plaques in tuberous sclerosis complex.

BACKGROUND: Fibrous cephalic plaques (FCPs) stereotypically develop on the forehead of patients with tuberous sclerosis complex (TSC). They constitute a major feature for TSC diagnosis and may present before other TSC-related cutaneous hamartomas. OBJECTIVE: To describe the clinical characteristics of FCPs in TSC. METHODS: A total of 113 patients with TSC were enrolled in an observational cohort study. Retrospective analysis of medical records and skin photography was performed. FCPs were categorized by anatomic location and size. RESULTS: FCPs were observed in 36% of patients (41 of 113). Of 62 total lesions, 58% were 1 to less than 5 cm, 13% were 5 cm or larger, and 29% were of unknown size mostly because of prior excision. The distribution of lesions was 39% on the forehead, 27% on the face (nonforehead), 3% on the neck, and 31% on the scalp. Fourteen patients had similar lesions less than 1 cm in diameter. Histopathologically, FCPs displayed dermal collagenosis, decreased elastic fibers, and features of angiofibromas or fibrofolliculomas. LIMITATIONS: Men were under-represented because the cohort was enriched for patients with TSC with lymphangioleiomyomatosis, which occurs in adult women. CONCLUSION: Two-fifths of FCPs presented on the forehead, with most of the remainder in other locations on the face and scalp. Better recognition of these lesions may lead to earlier diagnosis of TSC.

Folliculocystic and collagen hamartoma of tuberous sclerosis: A new case in a female patient and review of literature.

Folliculocystic and collagen hamartoma (FCCH) of tuberous sclerosis is a rare entity described in 2012 by Torrelo et al. with only 8 cases described, predominantly in males. It presents since birth or early infancy and in the majority of cases is associated with tuberous sclerosis. The hamartoma presents as an exophytic plaque and has distinctive histopathological features including hair follicles, intact or ruptured epidermal cysts, and an increased number of collagen fibers extending to the subcutaneous tissue. Herein we present an additional case of this rare entity in an 18-year-old female who met clinical criteria for tuberous sclerosis. The patient had an exophytic mass in the left temporal area for many years and wanted surgical excision due to its cosmetic appearance. Histopathology of the surgical specimen showed a hamartomatous lesion with multiple large intact epidermal cysts, hairs and increased thickened collagen. The patient has followed up for 1 year after the excision, with no recurrence. Additionally, we provide a literature review of known cases of FCCH as well as its clinical and histopathological differential diagnosis.

Factors associated with p-phenylenediamine sensitization: data from the Information Network of Departments of Dermatology, 2008-2013.

BACKGROUND: Risk factors for p-phenylenediamine (PPD) sensitization include the use of hair dyes, the application of temporary black henna tattoos, working as a hairdresser, and, possibly, exposure to hair dye pretests. OBJECTIVES: To quantify the impact of these (putative) risk factors on PPD sensitization. METHODS: Six items related to PPD exposure were added to the routine Information Network of Departments of Dermatology questionnaire from 2008 to 2013. A retrospective analysis of data from 4314 patients tested with PPD 1% pet. was conducted. RESULTS: Of the PPD-positive patients (n = 271), 80% had their hair dyed, and, of these, 57% subsequently developed scalp dermatitis, whereas only 11% had had a henna tattoo. The self-administrated pretest with hair dye was performed by only a few patients, precluding a more detailed analysis. Hair dyeing [odds ratio (OR) 6.0; 95% confidence interval (CI): 3.9-9.4], henna tattoos (OR 2.4; 95%CI: 1.5-3.7) and being a hairdresser (OR 2.1; 95%CI: 1.3-3.2) increased the risk of PPD sensitization. Neither dyeing of own hair nor application of a temporary henna tattoo seemed to affect PPD sensitization in hairdressers. p-Aminoaryl compounds more often gave positive reactions in patients with henna tattoo. CONCLUSIONS: Hair dyeing is the major risk factor for PPD sensitization in this clinical setting, and application of a temporary black henna tattoo may also lead to (strong) PPD sensitization.

Is cutis verticis Gyrata-Intellectual Disability syndrome an underdiagnosed condition? A case report and review of 62 cases.

Cutis Verticis Gyrata-Intellectual Disability (CVG-ID) syndrome is a rare neurocutaneous syndrome characterized by intellectual disability and scalp folds and furrows that are typically absent at birth and are first noticed after puberty. First reported in 1893, the syndrome was mainly identified in subjects living in psychiatric institutions, where it was found to have a prevalence of up to 11.4%. Most patients were reported in the literature during the first half of the 20th century. CVG-ID is now a less reported and possibly under-recognized syndrome. Here, we report a patient with CVG-ID that was diagnosed using the novel approach of magnetic resonance imaging and we conduct a systematic review of all patients reported in the last 60 years, discussing the core clinical features of this syndrome. © 2016 Wiley Periodicals, Inc.

Prevalence of hormonal and endocrine dysfunction in patients with lichen planopilaris (LPP): A retrospective data analysis of 168 patients.

BACKGROUND: Studies on the pathophysiology and comorbidities associated with lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA) are limited. OBJECTIVE: The purpose of this study was to determine the prevalence of androgen excess in the postmenopausal LPP population, in relation to demographics and comorbidities. METHODS: A retrospective data analysis of 413 patients with LPP, FFA, and LPP/FFA seen in the Department of Dermatology at the Cleveland Clinic Foundation in Ohio between 2005 and 2015 was conducted. Of this cohort, 168 patients met the inclusion criteria. RESULTS: Androgen excess was identified in 31.5% (n = 53) of the 168 patients with LPP and all subtypes (P < .001). Androgen deficiency was identified in 32.1% (n = 17) of the 53 patients with FFA (P < .001). The androgen excess group was significantly more likely to present with hirsutism, seborrheic dermatitis, polycystic ovary syndrome, ovarian cysts, or a combination of these (P < .001). LIMITATIONS: This study was limited by being retrospective. CONCLUSION: Our study demonstrated that LPP is associated with androgen excess, and FFA is associated with androgen deficiency.