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Introduction: Fibrous cephalic plaques (FCP) are a characteristic manifestation of tuberous sclerosis complex (TSC) and occur in one third of cases. Their natural history and long term course is unknown, as is the outcome of long term follow-up of TSC cases in old age., Phenotype and Methods: We describe an 80 year old with TSC due to a c.2784dupC TSC2 mutation, who was diagnosed in infancy with an FCP and was regularly followed up at the TSC clinic over 8 decades with regular epilepsy treatment and renal monitoring., Results: Regular clinical photography and clinical records document the plaque at different ages. The FCP naturally resolved at 74 years. Facial angiofibromas also faded with time in the last decade. His epilepsy and renal abnormalities remained under control with careful surveillance and monitoring., Discussion: Natural aging in the eighth decade causes progressive laxity of collagen and leads to natural resolution of FCPs. This novel finding with a unique 80 year follow up yields valuable insights into the aging changes within FCPs and facial angiofibromas as the pathways linking facial angiofibromas and FCP's through the TGF-β1 pathway are now being elucidated., Conclusion: We present a clinical odyssey showing the natural progression and history of FCPs in TSC and comment on the mechanistic pathways allowing potential interventions in this disfiguring condition. TSC cases can be successfully managed and complications - particularly in the brain and kidney, can be avoided over an entire lifetime. This is encouraging for long term prospects for patients with TSC., Competing Interests: Provenance: externally peer-reviewed, (Copyright © 2020 Ulster Medical Society.)

Background: Photodynamic therapy (PDT) is an effective treatment for actinic keratosis (AK), particularly for patients with large areas of field cancerization. Among the approved protocols in Europe, the most widely used requires irradiation with the Aktilite CL 128 lamp. However, pain during irradiation and the suboptimal adaptability of the lamp relative to the treatment area are two limiting factors of this protocol. To overcome these limits, a new protocol (referred to as the Flexitheralight protocol) involving irradiation with a light-emitting, fabric-based device was developed., Objectives: This paper aims to assess the noninferiority, in terms of PDT efficacy for treating AK, of the Flexitheralight protocol compared with the conventional protocol, which requires irradiation with the Aktilite CL 128 lamp., Methods: A monocentric, randomized, controlled, phase II clinical study was performed. Twenty-five patients with grade I-II AKs of the forehead and scalp were treated with methyl aminolaevulinate PDT in two symmetrical areas. One area was treated with the conventional protocol (n = 154 AKs), whereas the other area was treated with the Flexitheralight protocol (n = 156 AKs). The primary end-point was the lesion complete response (CR) rate at 3 months (an absolute noninferiority margin of -10% was used). The secondary end-points included patient-reported pain at the end of the irradiation., Results: At 3 months, the lesion CR rate with the Flexitheralight protocol was noninferior to that obtained with the conventional protocol (66·0% vs. 59·1%, respectively; absolute difference, 6·9%; 95% confidence interval -0·6% to 14·5%). Patient-reported pain was significantly lower with the Flexitheralight protocol than with the conventional protocol (mean ± SD: 0·4 ± 0·6 vs. 5·0 ± 2·6; P < 0·0001)., Conclusions: The Flexitheralight protocol is noninferior in terms of efficacy and superior in terms of tolerability to the conventional protocol for treating AKs of the forehead and scalp., (© 2018 British Association of Dermatologists.)

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., (Published by Elsevier Inc.)

Dermatomyositis is a systemic, autoimmune diseasewith a variety of clinical features that often includemyositis and characteristic cutaneous findings. Asubset of patients with dermatomyositis developcutaneous ulcers, often in the setting of vasculitis orvasculopathy. We present a case of dermatomyositiswith cutaneous ulcers that show perforatingcollagenosis on histopathologic examination.Acquired reactive perforating collagenosistypically occurs in the setting of diabetes mellitus,chronic renal failure, and other pruritic conditions,and this case represents a rare association withdermatomyositis, which may ultimately be helpful inelucidating the pathophysiology of this perforatingdisorder.

Background: Photodynamic therapy with methyl aminolevulinate (MAL-PDT) and ingenol mebutate gel (IMB) are approved therapeutic options for multiple actinic keratoses (AKs)., Objective: The aim of this intraindividual, split-face, randomized clinical trial was to compare treatment outcomes of MAL-PDT and IMB., Methods: Two symmetrical contralateral areas with a similar number of AKs were selected and randomly assigned to 3 days of an IMB treatment cycle or a single session of MAL-PDT. The next day, the local skin reaction (LSR) score was registered. The patients scored pain and time to healing of the treatment area., Results: After 90 days, the complete remission rate of lesions, the number of patients with complete remission of all lesions, cosmetic outcome, and patient preference were assessed., Conclusion: According to our results, IMB and MAL-PDT had a similar efficacy, but the cosmetic outcome was superior with MAL-PDT. Pain was higher with PDT, but LSR was more severe and time to healing was longer with IMB. Patients preferred MAL-PDT., (© 2016 S. Karger AG, Basel.)

A case of localized lichen planopilaris over the face, extending to the scalp is presented.This case highlights the different variations, which can occur in the presentation of localized lichen planus. The case was unique because of the extension of the lesions into the scalp. We also wanted to demonstrate the peculiar dermoscopic patterns of active lichen planopilaris when occurring over the face.

Patients with chronic graft versus host disease may exhibit a range of sclerotic features. Herein we present a patient with confirmed porphyria cutanea tarda who subsequently developed chronic graft versus host disease.

Background: Syphilitic alopecia (SA) is mainly described in single case reports, and there are only a few epidemiological studies., Objective: To investigate the clinical and dermoscopic features of SA in patients with secondary syphilis and to record its actual prevalence., Methods: All patients with a diagnosis of secondary syphilis were clinically and trichoscopically evaluated in search of hair and scalp alterations., Results: Five of 12 patients (41.6%) diagnosed with secondary syphilis had SA. The 'moth-eaten' pattern was detected in 4 patients (80% of cases): 1 had 'essential' SA, whereas 3 patients had 'symptomatic' SA. The 'diffuse' SA pattern was found in 1 patient. Trichoscopy of the 'moth-eaten' areas showed that alopecia is mainly due to a reduction in the number of terminal hairs. One patient with secondary syphilis showed trichodynia that regressed completely after antibiotic therapy., Limitations: The small number of patients included and the fact that none of our patients accepted to be biopsied in an affected area., Conclusion: Since SA appears to be present much more frequently than reported in the literature, when secondary syphilis is suspected, the patient should be examined for the presence of signs compatible with SA and the scalp should be examined by dermoscopy.

Background: Daylight photodynamic therapy (DL-PDT) of actinic keratosis (AK) has shown preliminary efficacy and safety results comparable to conventional photodynamic therapy (c-PDT), using methyl aminolevulinate (MAL) cream., Objectives: To demonstrate the efficacy and safety of DL-PDT vs. c-PDT in treating mild facial/scalp AK., Materials and Methods: This 24-week randomized, controlled, investigator-blinded, multicentre, intra-individual efficacy (non-inferiority) and safety (superiority regarding pain) study enrolled 100 subjects. AKs on the face/scalp were treated once, with DL-PDT on one side and c-PDT on the contralateral side. Primary end points for DL-PDT at week 12 were efficacy [non-inferiority regarding complete lesion response (mild AK)] and safety (superiority regarding subject's assessment of pain). Lesions with complete response 12 weeks after one treatment session were followed until week 24. The safety evaluation included incidence of adverse events. Subject satisfaction was classified using a questionnaire., Results: At week 12, the complete lesion response rate with DL-PDT was non-inferior to c-PDT (89·2% vs. 92·8%, respectively; 95% confidence interval -6·8 to -0·3), confirmed by intention-to-treat analysis. Additionally, regardless of the treatment used, 96% of mild lesions were maintained in complete response 24 weeks after the PDT session. For DL-PDT, subject-reported pain was significantly lower (0·8 vs. 5·7, respectively; P < 0·001), with better tolerability and significantly higher subject satisfaction regarding convenience and outcome., Conclusions: Daylight-mediated PDT was not inferior in efficacy to Metvix c-PDT (mild AK response rate), better tolerated, nearly painless and more convenient for patients., (© 2014 British Association of Dermatologists.)

Background: Daylight-mediated photodynamic therapy (DL-PDT) using methyl-5-aminolaevulinate (MAL) is effective for thin, grade I, actinic keratoses (AK). There are no published studies of other photosensitizers used in DL-PDT., Objectives: To compare the efficacy and adverse effects of 5-aminolaevulinic acid nanoemulsion (BF-200 ALA) with MAL in DL-PDT of grade I-III AKs., Methods: In 13 patients, 177 AKs were randomized symmetrically for a split-face prospective observer-blinded study and received either BF-200 ALA or MAL DL-PDT. Grade I AKs were treated once and grade II-III AKs twice with a 0·25-mm layer of photosensitizer precursors. Pain was assessed during and after the daylight exposure. Efficacy at 3 months was assessed clinically and histologically., Results: BF-200 ALA cleared 71/84 (84·5%) and MAL 69/93 (74·2%) of the AKs (P = 0·099), all grades responding equally, but with new AKs appearing during follow-up (n = 4, BF-200 ALA; n = 8, MAL). In per patient half-face analysis BF-200 ALA showed significantly higher clearance rates for grade I AKs than did MAL (P = 0·027), but for thicker grades, clearance was equal (P = 0·564). BF-200 ALA and MAL treatments resulted in 61·5% and 38·5% complete histological clearance (P = 0·375), respectively. p53 expression decreased by 54·4% and 33·7%, respectively (P = 0·552). Both treatments were nearly painless with similar adverse reactions and no difference in patient preference., Conclusions: BF-200 ALA showed a trend towards improved efficacy results compared with MAL. Thicker lesions in both groups responded when treated repeatedly. Importantly, a thin 0·25-mm layer of the photosensitizer precursors was sufficient, which may lead to lower expense., (© 2014 British Association of Dermatologists.)

Background: Rosacea is a common dermatosis that can involve the bald area of the scalp. We report the case of a man presenting clinical symptoms of rosacea of the forehead and the scalp, but with a histological picture of familial chronic benign pemphigus., Patients and Methods: A 47-year-old man with a history of Hailey-Hailey disease had been presenting facial dermatosis for 5 years. The clinical features were erythema with pustules and scales located on the mid-forehead and the androgenic bald area of the frontal scalp. The histological aspect of the skin biopsy showed suprabasilar clefting and ancantholysis at all levels of the epidermis and sparse perivascular infiltrate. Direct immunofluorescence was negative. These findings were typical of Hailey-Hailey disease. Based on clinical findings, and without taking account of the skin biopsy results, treatment with doxycycline and a topical antifungal was administered for 3 months, leading to remission of symptoms., Discussion: The site of rosacea on the bald area of the scalp in males is described in the literature, and when present, is probably enhanced by exposure to UV radiation. In this patient, the histological features were interpreted as histopathologically equivalent to Köbner phenomenon., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

A 58-year-old man presented for evaluation and treatment of non-tender, non-pruritic, annular patches on the right temple and frontal aspect of the scalp that reddened with exercise. A biopsy specimen showed a purpuric dermatitis with features of lymphocytic vasculitis; a diagnosis of exercise-induced progressive pigmentary purpura was made. Whereas progressive pigmentary purpura is purported to be caused by exercise, other similar appearing entities are associated with exercise, namely exercise-induced vasculitis (EIV). EIV may be considered as an acute microcirculatory deficiency and thermoregulation decompensation that occurs after episodes of exhaustive major muscular activity or after unusual or excessive exercise. The combination of age greater than 50 years, heat, and prolonged exercise are the most potent contributing factors. This is the first report of exercise-induced progressive pigmentary purpura.

Background: The origin of the distribution of segmental vitiligo (SV) has not yet been clearly elucidated. Segmental configurations of cutaneous disorders have been explained using two main interpretations, i.e. following either dermatomal or blaschkolinear distributions. However, facial SV does not always correspond to either of these distributions., Objectives: We classified facial SV into several distinctive subtypes according to specific distributions based on long-term observations., Methods: In total, 257 patients with facial SV were included, all of whom were closely observed for more than 1 year. The distribution patterns of facial SV were classified according to morphological similarities based on clinical observations., Results: The lesions of facial SV were categorized into six subtypes: types I-a and I-b, and types II-V. Type I-a and type IV broadly involved the mid-level face from the forehead to the lower cheek, but type IV lesions selectively appeared on the right side of the face and did not cross the midline. Type I-b lesions chiefly involved the forehead and scalp hair. Types II and III involved the lower face and, frequently, the neck area, and type V lesions were distributed mostly around the right orbital area. The most frequent type of lesion in this study was type I-a (28·8%), followed by types II (16·0%), III (14·4%), IV (10·9%), I-b (10·5%) and V (8·6%)., Conclusions: Newly established patterns of facial SV may be valuable for certain aspects of prognosis, such as the likely degree and path of lesion spreading., (© 2011 The Authors. BJD © 2011 British Association of Dermatologists.)

The histopathologic presence of a cornoid lamella is often associated with a diagnosis of porokeratosis. However, this feature is not pathognomonic for porokeratosis and can be found in a number of other dermatologic conditions, which include seborrheic keratosis, verruca vulgaris, actinic keratosis, squamous-cell carcinoma in situ, basal-cell carcinoma, milia, and scar. Notably, the etiology of none of these entities is inflammatory. Wade and Ackerman consider cornoid lamellation to be a distinctive histopathologic reaction pattern that reflects the disordered progression of epidermal cells during cornification. As such, this pattern is not specific for any given disease process. We report a case in which the lesions appeared inflammatory clinically as well as histopathologically, did not resemble porokeratosis despite the presence of cornoid lamellae, and responded to topical glucocorticoids.

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a condition that is characterized histopathologically by a characteristic pattern of granulomatous inflammation in the presence or absence of leukocytoclastic vasculitis. It has been associated with systemic diseases, especially autoimmune conditions such as rheumatoid arthritis and Behçet's disease. A 44-year-old woman with underlying limited systemic sclerosis presented with painful erythematous nodules located on her face and scalp. Histopathologic analysis confirmed a diagnosis of PNGD, which self-resolved within weeks of the biopsy. To our knowledge, this is the first report of a case of PNGD associated with systemic sclerosis. A review of the literature revealed that PNGD is a female-predominant disease that is most commonly associated with rheumatoid arthritis, followed closely by lupus erythematosus. Most patients with PNGD respond to treatment of the underlying systemic disease, although spontaneous resolution is not uncommonly observed.

A 57-year-old man is presented with blue pseudochromhidrosis affecting the face and neck following combination treatment with lansoprazole, a proton pump inhibitor, and ranitidine, a type two histamine receptor antagonist. The diagnosis was made on the basis of clinico-histological features and growth of Malassezia furfur, and Bacillus species, not Bacillus cereus, in the absence of lipofuscin. The pseudochromhidrosis resolved on stopping both medications and did not recur on restarting only the proton pump inhibitor.

Background: Actinic keratoses (AKs) are the most common epithelial precancerous lesions, especially among individuals with light complexions. AKs are believed to progress to in situ squamous cell carcinoma (SCC) and potentially, to invasive SCC. AKs and invasive SCCs share certain histopathological features and both share genetic tumour markers and p53 mutations. Given these facts, the treatment and management of AKs are integral components to quality dermatological health care., Objectives: Topical aminolaevulinic acid-based photodynamic therapy (ALA-PDT) has been extensively studied over the last several years. This study seeks to characterize further the efficacy and safety of ALA-PDT by extending previous work to: (i) assess the long-term recurrence rate of AKs that have resolved after ALA-PDT; (ii) to characterize the histopathology of treated AK lesions that do not completely respond to ALA-PDT or recur in long-term follow up; (iii) to characterize the histopathology of untreated clinically diagnosed AK lesions in the study population at baseline; and (iv) to evaluate ALA-PDT in darker skin types than previously studied., Methods: Patients enrolled in this study had six to 12 discrete AK lesions, either on the face or the scalp. Individual AK lesions designated for treatment were graded as either grade 1 (lesions slightly palpable and more easily felt than seen) or grade 2 (moderately thick AKs, easily seen and felt). Patients with grade 3 (very thick and/or hyperkeratotic) lesions were excluded. For each subject, two lesions at baseline were randomized to biopsy, and were not followed as part of the study while the remaining lesions (target lesions) were treated with ALA-PDT (baseline and month 2, if required) and followed for 12 months., Results: Of the 110 patients enrolled, 101 completed the study. The target AK lesions in the per-protocol population clearing completely in the first and second months following a single ALA-PDT treatment (baseline) were 76% and 72%, respectively. Sixty per cent of the patients received a second ALA-PDT treatment, limited to the target AKs still present at month 2. The percentage of treated target lesions that cleared completely peaked at 86% at month 4 then decreased gradually over time to 78% at month 12. The overall recurrence rate for all lesions that were noted to be cleared at some visit during the 12-month period was 24% (162/688). Of the 162 recurrent lesions 16 were lost to follow up, seven spontaneously cleared and 139 were biopsied. With respect to the lesions biopsied, 91% (127/139) were diagnosed histopathologically as AK, with the balance of lesions being SCC (nine of 139: 7%), basal cell carcinoma (one of 139: 0.7%) and other non-AK diagnoses (two of 139: 1%). The recurrence rate for histologically confirmed AKs was 19%. The clinical diagnosis of AK by investigators appeared to be accurate, with 91% (200/220) of the untreated clinically diagnosed AK lesions being histopathologically confirmed to be AK (AK, 142/220: 65%; advanced AK, 29/220: 13%; macular AK, 29/220: 13%). Despite concentrated efforts to recruit patients with Fitzpatrick skin types IV-VI, the distribution was as follows: I, 11%; II, 36%; III, 41%; IV, 11%; V, 2%. The demographics of this study population are typical of a patient population with AK., Conclusions: ALA-PDT was shown to be an effective and safe therapy for the treatment of AKs of the face and scalp in skin types I-V, with an acceptable rate of recurrence over 12 months of histologically confirmed AKs of 19%. Phototoxicity reactions were all expected, nonserious and had essentially resolved after 1 month post-treatment independent of skin type.

Background: Topical 5-fluorouracil (5-FU) cream is widely used in the treatment of actinic keratoses (AKs) but the optimum treatment regimen that provides efficacy while minimizing side-effects remains unclear., Objectives: A randomized trial to compare the efficacy and side-effects of daily vs. weekly application of 5% 5-FU in the treatment of AKs of the scalp and face., Patients/methods: Twenty patients were recruited and randomized to two groups. Group 1 (13 patients) applied 5% 5-FU twice daily for 3 weeks, group 2 (seven patients) applied 5% 5-FU twice daily for 1 day per week for 12 weeks. Patients were reviewed at weeks 3, 12, 24 and 52. At each review a lesion count and lesion map were completed and patients were asked to score efficacy and inflammation., Results: At week 0 the median lesion count was the same in both groups, 17.5 lesions. At 12 weeks the median lesion count in group 1 had fallen to 0 where it remained for the duration of follow-up. In group 2 the median lesion count fell to 6 at 12 weeks, 5.5 at 24 weeks and was 3 at 52 weeks. The difference in the lesion count was significant at all time points after week 0: P < 0.05 at weeks 12 and 52, and P < 0.01 at week 24. The mean inflammation score was higher in patients clear of AKs at 12 weeks compared with those who had not cleared, 3.8 compared with 1.9. This difference was statistically significant (P < 0.05) suggesting that inflammation is necessary for efficacy., Conclusions: We conclude that daily application of 5% 5-FU cream is more effective than weekly application at clearing AKs from the scalp and face. Our results also suggest that inflammation is likely to be required to achieve a therapeutic effect.

We report a case of chronic active Epstein-Barr (EB) virus infection (CAEBV) associated with skin eruptions mimicking hydroa vacciniforme (HV) in a 4-year-old boy. The patient had repeated episodes of vesiculo-necrotic eruptions on the face, scalp, and bilateral forearms one year before the first visit to our department. General symptoms including fever, hepatosplenomegaly, abnormal liver function, and cervical lymph node swelling were noted three months before the first visit. At the first visit, small, bean-sized, erythemic papules with central necrosis were observed on the face and anterior chest wall. Thumb-sized ulcers with crust were present on the bilateral forearms. Histopathological examination of an erythematous lesion in the submandibular area revealed parakeratosis with a thick crust, mild spongiosis in the epidermis, and a dense infiltration of lymphoid cells into the dermis and perivascular space. Laboratory examination showed EBNA x 40, EBV VCA IgG x 1,280, and EBV DNA (PCR) 8 x 10(4). EBV-encoded small nuclear RNA (EBER) positive cells were detected in the dermis by an in situ hybridization (ISH) method. Large granular lymphocytes (65%) with the NK cell phenotype were found in the peripheral blood. A real time PCR method showed 171,741 copies/ micro g DNA in CD 16 positive cells. Although latent EBV infection-associated eruptions have been documented, detailed skin manifestations in CAEBV are less well known.

Severe facial and scalp dermatitis following the use of permanent hair dyes has been reported in several cases. Para-phenylenediamine (PPD) is known as a potent contact allergen, and PPD is allowed in hair dye at a concentration of 6%. Hair dye reactions are usually diagnosed by the patients themselves, and adverse reactions to hair dye may not necessarily be recorded by the health care system, unless the reactions are especially severe. Based on this assumption, we suspected that hair dye dermatitis was occurring more frequently than reported in the literature. Consumer complaint-based data were obtained by advertising for persons with adverse reactions to hair dye. Among those responding to the advertisement, 55 cases of severe, acute allergic contact dermatitis were identified. The main symptoms were severe oedema of the face, scalp and ears, and clinically this was often mistaken for angio-oedema. The 55 cases comprised a total of 75 visits to the health service and 5 admissions to hospital. 18 persons had sick leave, which supports the impression of very severe dermatitis reactions. 60% were treated with antihistamine, while 52% were treated with corticosteroids. 29% of the cases were patch tested and all were found positive to PPD. Our data presented here clearly show that PPD and its derivatives in hair dye at the present concentrations presents a significant health risk for the population. Furthermore, the severe acute allergic skin reactions are often misdiagnosed in the health care system. The frequency of allergic contact dermatitis resulting from hair dye is likely to be underestimated. New methods to survey the frequency of adverse reactions should be considered.

The safety and efficacy of treating individuals who presented with multiple actinic keratosis (AK) lesions with 5-aminolevulinic acid (ALA) in combination with photodynamic therapy (PDT) were documented in a phase III trial. This report highlights results of this phase III trial and reviews 4 specific cases of sustained AK lesion clearance 4 years after treatment with ALA/PDT Long-term recurrence data were collected from patients who participated in clinical trials of ALA/PDT Long-term evaluation extended to 36 to 48 months (4 years) supports primary efficacy findings of the phase III pivotal trial, with a low incidence of AK recurrence in patients treated with ALA/PDT

The safety and efficacy of treating individuals who presented with multiple actinic keratosis (AK) lesions with 5-aminolevulinic acid (ALA) in combination with photodynamic therapy (PDT) were documented in a phase III trial. This report highlights results of this phase III trial and reviews 2 specific case studies treated with ALA/PDT who presented with both multiple nonhyperkeratotic AK lesions and moderate-to-severe photodamage. Treatment consisted of a 2-step process performed by the investigator. In addition to the pretreatment evaluation, each subject was evaluated at 1- and 2-month intervals after each treatment of ALA/PDT. Clinical findings reveal complete healing at the site of ALA/PDT without scarring or changes in pigmentation, as well as significant changes in signs of photodamage, such as improvement in skin elasticity and reduction in skin thickening.

A 30-year-old male presented with multiple, yellowish to skin-colored, firm to soft nodules on the forehead, temple, postauricular area and neck for the previous seven years. Examination of the scalp revealed a single, soft, cystic swelling on the occiput and the patient complained of occasional discharge from two, 0.1 x 0.1 cm, openings in front of both ears. Histopathology of the facial lesion was suggestive of steatocystoma multiplex, while that of the scalp revealed a pilar cyst. An otorhinolaryngeal evaluation confirmed the presence of bilateral pre-auricular sinus. We present this previously unreported pilosebaceous hamartoma, constituting facial steatocystomas and pilar cyst, associated with bilateral preauricular sinus.

We describe three patients with frontoparietal scleroderma showing multiple lesions. The lesions were in two different lines that seemed to belong to Blaschko's lines. It remains controversial whether linear scleroderma follows Blaschko's lines, but our observations presented here suggest that frontoparietal scleroderma occurs along the lines of Blaschko.