Isolated linear blaschkoid psoriasis

Linear psoriasis (LPs) is considered a rare clinical presentation of psoriasis, which is characterized by linear erythematous and scaly lesions along the lines of Blaschko. We report the case of a 20‐year‐old man who presented with asymptomatic linear and S‐shaped erythematous, scaly plaques on right side of his trunk. The plaques were arranged along the lines of Blaschko with a sharp demarcation at the midline. Histological examination of a skin biopsy confirmed the diagnosis of psoriasis. Topical calcipotriol and betamethasone dipropionate ointments were prescribed for 2 months. A good clinical improvement was achieved, with reduction in lesion thickness and scaling. In patients with linear erythematous and scaly plaques along the lines of Blaschko, the diagnosis of LPs should be kept in mind, especially in patients with asymptomatic lesions of late onset.     

Lichen planus-like dermatosis with Blaschko line distribution: a case report

The authors describe the case of a healthy 46-year-old woman with a unilateral linear papular band on the left side of the trunk that followed the lines of Blaschko from the lower back extending to the left anterior side of her abdomen. The lesions were flat-topped, slightly elevated, violaceous, agglomerated lichenoid papules. The biopsy specimen demonstrated the typical histology of lichen planus. A working diagnosis of linear lichen planus was confirmed. Because congenital and/or nevoid skin disorders in a blaschkolinear distribution may have a delayed onset after birth, these lesions must be differentiated from acquired dermatoses following the lines of Blaschko. This distinction should be made in cases with isolated lesions, such as the case presented here.     

A case of lichen striatus following Blaschko lines

We present a case of lichen striatus (LS) in a 36-year-old woman with skin lesions on the right side of the chest distributed along Blaschko lines that showed a swirling s-shaped pattern. The differential diagnosis of LS includes a variety of nevoid and acquired conditions following Blaschko lines, particularly acquired relapsing self-healing Blaschko dermatitis. The extent to which these 2 conditions overlap remains contentious.     

Analysis of 36 Cases of Blaschkoid Dyspigmentation: Reading Between the Lines of Blaschko

Genetic mosaicism indicated by lines of Blaschko pigmentary changes has been described under a number of different and confusing terms, including hypomelanosis of Ito, linear and whorled nevoid hypermelanosis, nevus depigmentosus, and cutis tricolor. Moreover, extracutaneous findings, particularly serious neurologic defects, have been reported in a large number of these cases. We reviewed the cutaneous and extracutaneous findings in 36 patients referred to the Harriet Lane Pediatric Dermatology Clinic, Johns Hopkins University, from June 12, 2008, to May 24, 2009, for evaluation of macular lesions along the lines of Blaschko. Patients with dyspigmentation along the lines of Blaschko and no history of preceding inflammatory skin lesions were identified for inclusion in a database at their initial visit. Information on age at presentation; sex; age when first diagnosed; type, pattern, and location of the pigmentary anomaly; and extracutaneous abnormalities noted on a review‐of‐systems questionnaire and physical examination was recorded for each child. Patients were asked to follow up within 6 to 12 months of the initial visit. Patients included 13 boys and 23 girls ages 3 months to 15 years with lesions noted from birth to 12 years. Lesions were hypopigmented in 21 patients and hyperpigmented in 15. No patients presented with hypopigmented and hyperpigmented lesions. Extracutaneous findings were noted in five children (13.9%). Historically, cases of Blaschkoid hypopigmentation and hyperpigmentation have been associated with a high percentage of extracutaneous manifestations, particularly neurologic and neurodevelopmental defects. In our study, only five patients (13.9%) were noted to have extracutaneous abnormalities, and these findings may have been coincidental. We propose the term ‘Blaschkoid dyspigmentation’ to describe the cutaneous findings. Although serious extracutaneous findings may occur in children with Blaschkoid dyspigmentation and results of careful physical examination and review of systems should direct an evaluation, serious extracutaneous findings occur in a minority of patients.     

A case of linear lichen planus

Linear lichen planus (LLP) is a very rare form of lichen planus (LP) characterized by a linear distribution of the lichenoid lesions. It usually follows the lines of Blaschko with unilateral involvement. We report such a case of LLP. A 50-year-old man presented with slightly pruritic, linear, violaceous, papular lesions on the right side of his jaw. A biopsy specimen demonstrated the typical histology of LP. Based on the clinical and pathological findings, the diagnosis was LLP.     

The clinical profile of children in India with pigmentary anomalies along the lines of Blaschko and central nervous system manifestations

The aim of this study was to describe the clinical profile of children with congenital pigmentary anomalies along Blaschko lines and the associated manifestations in the central nervous system. Twenty-six children aged 12 years or less (14 boys and 12 girls), with hypopigmentation and hyperpigmentation along Blaschko lines and central nervous system manifestations were included during the period June 2001 to December 2003. Detailed physical, cutaneous, and systemic examinations were carried out. Relevant investigations included histopathology, karyotyping, electroencephalogram, computerized tomography scans, and magnetic resonance imaging of the brain whenever possible. Twenty children had hyperpigmentation along the lines of Blaschko, four had hypopigmentation, and two had a combination of the two. Eight children had diffuse involvement. Of these, two each had hypomelanosis of Ito, incontinentia pigmenti and linear and whorled nevoid hypermelanosis. The remaining 20 patients could not be categorized as any of the described entities. The majority (92.3%) of patients manifested skin and central nervous system disorders before the age of 2 years. Hyperpigmentation along Blaschko lines was significantly higher in patients with central nervous system manifestations (p = 0.01). Developmental delay was the most frequent central nervous system presentation. Multiple systems were affected, including the eyes. Histology was useful to distinguish incontinentia pigmenti from other types of nevoid hyperpigmentation.     

The treatment of linear morphoea with D-penicillamine

Localized morphoea (localized scleroderma) generally has a good prognosis and only rarely evolves into systemic sclerosis. The linear form, however, can cause considerable disfigurement and disability due to the involvement of skin, subcutaneous tissue, muscle, bone, and synovium. When it occurs in childhood, growth arrest, limb shortening, and joint contractures may result in severe local deformity and rarely even loss of a limb.¹ A lowered ratio of soluble to insoluble collagen in skin biopsies from patients with scleroderma has been demonstrated², and the discovery that D-penicillamine could normalize this ratio led Moynahan³ to treat 14 children with linear morphoea with low-dose D-penicillamine. He reported uniformly good results in all patients. Kesler and colleagues⁴, however, found no benefit when comparing two children with linear morphoea, one of whom was treated with D-penicillamine. We report two patients with linear morphoea who showed striking improvement following treatment with 250 mg D-penicillamine daily.     

Subacute cutaneous lupus erythematosus on the lines of Blaschko

We describe a 42-year-old woman who had erythemateous plaques with sharply demarcated margins and fine scaling on her right trunk and leg. The lesions had a linear distribution following the lines of Blaschko. Histological findings supported the diagnosis of subacute cutaneous lupus erythematosus (SCLE) on the lines of Blaschko. Treatment with systemic corticosteroids and anti-malarial agents resulted in remission. Up to now, eighteen cases of linear lupus erythematosus have been described. Linear lupus erythematosus mostly affects children presenting with lesions on the scalp that were classified as discoid lupus erythematosus. To our knowledge, this is the first case of subacute cutaneous lupus erythematosus on the lines of Blaschko.     

Expression of insulin-like growth factor-I in lesional and non-lesional skin of patients with morphoea

Background  Morphoea (scleroderma) is a chronic disorder characterized by circumscribed sclerotic plaques with the hallmark of increased fibroblast activation and fibrosis. Through its effect on connective tissue cells and immune cells, insulin-like growth factor (IGF)-I has been found to play a role in some autoimmune connective tissue diseases and has been implicated in the pathogenesis of several fibrotic disorders.
Objectives  To evaluate the role of IGF-I in the pathogenesis of morphoea.
Methods  The study was carried out on 15 patients with morphoea and nine healthy controls. Two 5-mm punch skin biopsies were taken from every patient (one from lesional and one from non-lesional skin) and a single biopsy was taken from the normal skin of each control. A 10-mL blood sample was also taken from each patient and control. Quantitative detection of tissue and serum levels of IGF-I was done using an enzyme-linked immunosorbent assay technique.
Results  IGF-I in lesional skin was significantly higher than in non-lesional and control skin ( P  =   0·001 and P  =   0·021, respectively). Moreover, a significantly higher level of IGF-I was detected in patient serum when compared with control serum ( P  <   0·001). A direct significant correlation existed between lesional and non-lesional skin level ( r  =   0·618, P  =   0·014), and between lesional skin level and Rodnan score ( r  =   0·538, P  =   0·039).
Conclusions  Despite the small sample size, this study suggests that IGF-I plays an important role in the pathogenesis of fibrosis, characteristic of morphoea. Studies on a larger number of patients with morphoea as well as on patients with systemic sclerosis are recommended. Furthermore, therapeutic trials using IGF-I antagonist (octreotide) are highly recommended in patients with morphoea.     

Linear scleroderma along Blaschko's lines in a patient with systematized morphea

We have previously shown that frontoparietal scleroderma en coup de sabre, a type of linear scleroderma that affects the face and scalp, follows the lines of Blaschko, but the question whether linear scleroderma that occurs in the limbs follows Blaschko's lines has not been answered. We describe the case of a 4-year-old girl with multiple morphea showing remarkable unilateral systematized distribution and whose linear lesions in the limbs appeared to follow Blaschko's lines. We suggest that linear scleroderma of the limbs, as well as frontoparietal scleroderma, may occur along the lines of Blaschko. Since both the unilateral distribution and the lesions along Blaschko's lines are the patterns created by genetic mosaicism, we suggest that a significant part of linear scleroderma and perhaps a smaller part of multiple morphea could be related to cutaneous mosaicism.     

Cutis marmorata telangiectatica congenita: a prospective study of 27 cases and review of the literature with proposal of diagnostic criteria

Background.  Cutis marmorata telangiectatica congenita (CMTC) is a congenital vascular anomaly of unknown aetiology. About 300 cases have been reported in the literature. The rate of associated anomalies varies between 20% and 70%.
Methods.  We report a series of 27 children with CMTC, 18 of whom were followed-up prospectively for a median of 22 months (range 2 months–5.3 years).
Results.  Both genders were equally affected (13 male/14 female). The legs were involved in 20 cases (74%), the arms in 10 (37%), the face in 4 (15%) and the trunk in 18 (67%). There were 20 (74%) patients who presented with involvement of both trunk and limbs, a further 20 patients had lesions affecting the limb on only one side of the body, and 7 children (26%) had bilateral lesions; 1 child had generalized CMTC lesions. The involved areas covered a mean of 18% of body surface area (range 3–90). Associated anomalies were found in 15 patients (56%), with some exhibiting more than one. There was body asymmetry (hypertropy or hypotrophy of the affected limb) in nine patients (33%), seven patients had a variety of other malformations (congenital glaucoma, syndactyly, lipoma, macrocephaly, renal hypoplasia, Kartagener's syndrome), and other vascular lesions were present in four patients (15%). There was no correlation between the extent of skin lesions and likelihood of associated anomalies. On follow-up, fading of skin lesions was noted in 67% of our patients.
Conclusion.  Body asymmetry is the most common anomaly associated with CMTC; other associations might be pure chance. In order to separate CMTC from other vascular malformations, notably Klippel–Trénaunay syndrome, we suggest diagnostic criteria for their differentiation.     

How are the Blaschko lines arranged on the scalp?

Linear inherited or nevoid skin lesions mostly follow a characteristic pattern which was delineated by Blaschko in 1901. The system of lines published by this author covered the entire human body, with the exception of the scalp because of lack of pertinent case reports regarding this area. We here report an observation which enables us to fill the blank area in the atlas of the lines of Blaschko. On the scalp of a girl affected with the oral-facial-digital syndrome, we observed an alopecia distributed in several spiral streaks. The pattern resembled that of the normal vertex, but the direction of the hairless streaks did not correspond completely to the direction of hair growth. As the oral-facial-digital syndrome is inherited as an X-linked dominant trait, the linear alopecia probably reflects functional X-chromosome mosaicism. Arguments are presented in favor of the assumption that the hairless streaks of this child follow the lines of Blaschko.     

Lichen planus in the lines of Blaschko

We describe three adolescents with eruptive linear lesions of the back, abdomen, and extremities distributed along the lines of Blaschko. Clinically one of these lesions resembled an epidermal nevus in its morphology and distribution. A biopsy specimen demonstrated the typical histology of lichen planus (LP). There have been fewer than 20 reported cases of childhood linear LP. Our patients demonstrate that linear LP sometimes may closely resemble an epidermal nevus and may auger the onset of more generalized LP or more involvement along the lines of Blaschko, that is, "Blaschkoian" disease. Previous studies have referred to this variant as zosteriform. Our cases are the fourth, fifth, and sixth cases of childhood LP reported to occur along the lines of Blaschko. Based on the clinical findings in our patients and a review of the English-language literature, we believe that linear LP is usually distributed along the lines of Blaschko and should be renamed lichen planus, Blaschko subtype.     

Multiple linear lichen planus in the lines of Blaschko

Summary The lines of Blaschko describe a distribution pattern which may represent embryologlcal development pathways. The distribution may occasionally be followed by some skin disorders. We report a patient who had multiple lesions of linear lichen planus which followed Blaschko's lines. There were multiple streaks affecting several different areas of the body.     

Congenital melanocytic nevi and nevus spilus have a tendency to follow the lines of Blaschko: an examination of 200 cases

There have been few reports about the characteristics of the pigmentary arrangement of congenital melanocytic nevi (CMN) and nevus spilus (NS). Though the lines of Blaschko, which don't correspond to other lines such as Langer's lines and skin dermatomes, are well known to be followed by some rare pigmentary disorders, the relationship between these two disorders and the lines of Blaschko has not been discussed. Therefore, through an examination of 200 cases, we investigated whether or not the arrangement of CMN and NS follow the lines of Blaschko. Thirty-nine out of 92 cases (42.4%) of CMN and 67 out of 108 cases (62.0%) of NS were considered to follow the lines of Blaschko. Today, the idea is widely accepted that two functionally different cells proliferate during early embryogenesis of the skin which is caused from mosaicism and, as a result, the lines of Blaschko appear visually. In our results, we found that there were some cases of CMN and NS which showed a relationship with the lines of Blaschko, which is meaningful considering that the lesions and the lines are both related to pigmentary gene anomalies. The result was compared with CMN and NS by the chi(2) test. NS showed a predominant tendency to follow the lines with a significant difference (P = 0.0055). This may indicate that the lines of Blaschko reflect more clearly the proliferation of the epidermal cells than other cells in growth of the skin of the embryo. Further studies are required to elucidate the reason why some cases follow these lines and others do not.     

Mixed vitiligo of Blaschko lines: a newly discovered presentation of vitiligo responsive to combination treatment

Vitiligo, depigmenting disorder of the skin and mucous membranes, affects up to 1% of the population worldwide. It is classified into four major types: segmental, non‐segmental, mixed, and unclassified type. Non‐segmental vitiligo refers to non‐dermatomal distribution of lesions, while dermatomal distribution of lesions is present in patients with segmental vitiligo. Segmental vitiligo can also follow Blaschko lines – pathways of epidermal cell migration and proliferation during the development of the fetus. Here, we present patient with segmental and non‐segmental vitiligo following Blaschko lines with excellent therapeutic response to combined therapy. Prior to our report, a case of segmental and non‐segmental vitiligo followed by Blaschko lines was never described, therefore we suggest the term “mixed vitiligo of Blaschko lines” to describe this entity. This is also a rare case in which 90% repigmentation was achieved in patient with segmental and nonsegmental vitiligo following Blaschko lines in only 2 months of combined therapy.     

Unilateral Blaschkoid lichen planus involving the entire half of the body, a unique presentation

A large number of congenital as well as acquired skin disorders assume a characteristic morphological pattern following the Blaschko lines. Lichen planus (LP) is an acquired inflammatory disorder that can come with different faces. Linear lesions are frequently seen but cases of zonal (zosteriform) or whorled appearance have rarely been described in the literature. We describe an even rarer presentation in a 30-years-old man who had unilateral pruritic violaceous lesions over right half of body characteristically following lines of Blaschko with clinical and histological morphology typical of LP.     

Pilot study of dual-wavelength (532 and 633 nm) laser Doppler imaging and infrared thermography of morphoea

Background  Little is known about the pathophysiology of localized scleroderma (skin fibrosis, also termed 'morphoea'), although it is likely that microvascular dysfunction is a contributing factor.
Objectives  Our aim was to investigate different components of blood flow in morphoea using infrared thermography and dual-wavelength laser Doppler imaging (LDI).
Methods  Eight plaques of morphoea (in eight patients) were studied. Skin temperature and blood flow were assessed in both affected (within plaque) and adjacent unaffected (perilesional) skin.
Results  Skin temperature (representing blood flow) was higher in all areas of morphoea when compared with uninvolved skin. Perfusion within the plaques was found to be increased, when compared with uninvolved skin; in all cases as imaged by red wavelength (633 nm) LDI (representing blood flow through large, thermoregulatory vessels) and in six of eight cases by green wavelength (532 nm) LDI (representing nutritive capillary blood flow). The median (range) skin temperature difference between plaque and perilesional skin was 1·1 (0·7–2·2) °C and the median (range) ratios of plaque/perilesional perfusion as measured by red and green wavelength LDI were 1·3 (1·1–1·9) and 1·1 (0·8–1·5) arbitrary perfusion units, respectively.
Conclusions  Microvascular perfusion is increased within morphoea plaques and the increased response detected by both thermography and red wavelength LDI, as compared with green wavelength LDI, suggests that the increase in perfusion is more marked in deeper, larger, rather than in superficial, smaller vessels.     

Lichen striatus: a Blaschko linear acquired inflammatory skin eruption.

An illustrative case report and a series of 18 well-documented cases of lichen striatus are presented. The mean age at diagnosis was 3 years (6 months to 14 years; median 2 years). The lesions were predominantly distributed on the trunk in 33% of cases and on the limbs in the remaining two thirds (upper limb: 48%; lower limb: 19%). Pruritus was noted in only 1 of 18 cases. Six cases were associated with clinical features of atopy and/or minor signs of atopic dermatitis (e.g., pityriasis alba). Two cases were considered to be clinically associated with lesions consistent with psoriasis. The mean duration was 9.5 months (4 weeks to 3 years; median 6 months). In one patient, two relapses occurred in 4 years. Hypochromic sequelae were noted in 50% of cases. Lichen striatus is the most common acquired self-limited linear eruption in childhood that follows Blaschko's lines. A new acronym is proposed to emphasize the developmental background of the disease: BLAISE for Blaschko linear acquired inflammatory skin eruption.     

Blaschko线及相关疾病的研究

在临床工作中经常可以见到一些沿Blaschko线受累的皮肤病,皮损常具有一定的特征性.Blasehko线是由基因组嵌合体引起,临床表现有一定规律性.皮损外观比较奇特,常呈线状,节段型,旋涡状等分布.很多疾病都可沿Blaschko线分布,既包括先大或遗传性疾病,也包括后天或获得性疾病.一些皮肤外器官组织的疾病也有呈线状、带状、旋涡状等分布的规律.由于Blaschko线能从病因发生机制的方面阐述一些疾病,从而可对一些疾病的分类、病程发展等提供一些新认识.