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.     

Extragenital bullous lichen sclerosus: A case report and literature review

Lichen sclerosus (LS) is a chronic dermatosis characterized by atrophic white papules or plaques, most commonly occurring on the anogenital skin. Blisters have been rarely described developing in the background of extragenital LS. A 74-year-old woman with a 4-year history of sclerotic patches on the trunk showed a flaccid bulla on the lower back for 3 months. The histopathological finding of the skin biopsy was consistent with the diagnosis of bullous LS. In this paper, we present this uncommon case, review the literature on extragenital bullous LS, discuss the pathogenesis, and provide some treatment options for the case.     

Nail dystrophy due to lichen sclerosus?

Lichen sclerosus (LS) affects anogenital skin alone in 80% of cases. When extragenital disease occurs, it usually affects the trunk, neck, axillae and wrist flexures. Nail involvement with LS is rare. In contrast, lichen planus (LP) commonly affects extragenital skin. Mucosal lesions occur in 50% of cases, affecting the mouth and genitalia. Nail disease in LP is common, and, if severe, can lead to destruction of the nail bed. LS and LP can coexist. We report two cases of LS with nail involvement. In the Case 1 disease was confined to the nail, and nail biopsy confirmed LS. In the Case 2, the nail changes formed part of the widespread genital and extragenital LS, confirmed histologically. We review existing literature on nail disease in LS and discuss the possible aetiology of the nail changes.     

Lichen striatus in a child after immunization. A case report

Lichen striatus is a self-limited, lichenoid eruption particularly common in children. The lesions are located on extremities and less commonly on the trunk, and they follow the developmental lines of Blaschko. The etiology of lichen striatus is as yet unknown. It has been observed after infection or immunization in atopic patients and in siblings. The authors report on a 15-month-old girl that developed lichen striatus along the Blaschko lines on the trunk and one extremity after receiving the combined vaccine against measles, mumps, and rubella. Six months later, complete resolution of the skin lesions occurred without any treatment, leaving only slightly hypopigmented macules on the extremity.     

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.     

Linear cutaneous lupus erythematosus in the lines of Blaschko

Eleven patients with linear cutaneous lupus erythematosus following the lines of Blaschko have been previously reported in the literature. We describe a child with this entity following Blaschko lines on the trunk. The patient responded to oral hydroxychloroquine therapy combined with topical flurandrenolide tape resulting in resolution with atrophic scarring. The clinical and histological features of linear cutaneous lupus erythematosus are discussed and a review of the literature is presented.     

Simultaneous Lichen Striatus in Siblings along the Same Blaschko Line

Abstract:   Lichen striatus (LS) is an asymptomatic, spontaneously resolving linear dermatosis consisting of 2 to 4 mm flat topped papules following the lines of Blaschko. Two siblings presented with a simultaneous occurrence of LS along the same Blaschko line of the left upper extremity. Only four other cases of a simultaneous occurrence of LS in related siblings have been reported, but none of these have occurred in the same extremity. Although 10 years have passed since the last case report of this unique concurrent familial eruption, few discoveries have been made regarding its etiology. Several theories have been proposed including environmental agents, cutaneous injury, viral infection, hypersensitivity, and genetic predisposition. These theories are discussed along with retrotransposons, a possible new explanation for the pathogenesis of this and other Blaschko line disorders.     

Blaschkitis beim Kind – doch eine eigene Entität?

Blaschkitis is an acquired, rare dermatitis that follows the lines of Blaschko. Many consider blaschkitis as a variant of lichen striatus, although authors felt that it is a separate entity. A 2½‐year‐old girl presented with multiple grouped papules along the lines of Blaschko on her trunk. The main differences to lichen striatus are illustrated. Our case supports the hypothesis that blaschkitis is an entity of its own.     

Papular drug eruption along the lines of Blaschko caused by lenalidomide

Cutaneous drug reactions can take many forms. In rare cases these reactions can occur along the lines of Blaschko. A 60-year-old patient received lenalidomide in 3-weeks-cycles for the treatment of a plasmocytoma. After four months of treatment, red papules appeared on the extremities and the trunk following the lines of Blaschko. The lesions nearly disappeared during drug-free intervals and appeared with renewed intensity after restart of a therapy cycle.     

Hypomelanosis of ito-whorled hyperpigmentation combination: a mirror image presentation

Abstract:  We hereby report a 2-year-old boy who presented with a peculiar combination of hyperpigmented and hypopigmented skin lesions along the lines of Blaschko, sharply demarcated at the midline, both on the anterior and posterior trunk. Although combinations of hyperpigmented and hypopigmented skin lesions distributed along the lines of Blaschko have been described, the combination of skin lesion comprised hypomelanosis of Ito and whorled hypermelanosis distributed in the extremely unusual manner described in this patient has not been reported previously.     

Clinical,dermoscopic and histopathologic features of genital and extragenital lichen sclerosus

Background Little is currently known about the dermoscopic patterns of genital and extragenital lichen sclerosus (LS). In order to evaluate and compare the dermoscopic and histopathologic patterns of genital and extragenital lichen sclerosus, a retrospective analysis of clinical, dermoscopic and histopathologic features of genital and extragenital LS, collected between March 2010 and December 2011 at four dermatology clinics in Greece, Italy, Serbia and Uruguay was performed. Observations A total of 29 lesions from 14 (mean age 62.8 years) and 12 (mean age 53.5 years) patients with genital and extragenital LS, respectively were analyzed. Mean duration of disease was 3.5 years for genital and 1.8 years for extragenital LS. White‐yellowish structureless areas were seen in all cases of genital and extragenital LS; however linear vessels occurred at higher frequency in genital than in extragenital lesions (85.7% vs. 33.3%, respectively). Extragenital LS revealed two different time‐related patterns: keratotic plugs were more prevalent in lesions with short duration (<2 years), whereas longer persisting lesions appeared atrophic and revealed fine chrysalis structures. Conclusions Our morphologic study provides novel insights into the morphologic diversity of LS at different body sites and different stages of progression.     

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.     

Acquired Blaschko Dermatitis: Acquired Relapsing Self-Healing Blaschko Dermatitis

Acquired Blaschko dermatitis is a rare disease with acquired unilateral relapsing inflammatory linear lesions along Blaschko's lines. Histopathology reveals spongiotic dermatitis. “Blaschkite de l'adulte” and “acquired relapsing self-healing Blaschko dermatitis” have been suggested as names for this condition. Our patient was a 27-year-old man with a 6 month history of repeated, unilateral, slightly pruritic, discrete and grouped erythematous papules and papulovesicles on the left side of the upper limbs and trunk along Blaschko's lines. Histologic examination showed subacute spongiotic dermatitis. The condition showed excellent improvement after treatment with systemic corticosteroid for 2 months. Only a few cases have been reported. We propose a new designation and describe a patient who represents the first reported case of acquired Blaschko dermatitis in Korea.     

Linear morphoea follows Blaschko's lines

Background  The aetiology of morphoea (or localized scleroderma) remains unknown. It has previously been suggested that lesions of linear morphoea may follow Blaschko's lines and thus reflect an embryological development. However, the distribution of linear morphoea has never been accurately evaluated.
Objectives  We aimed to identify common patterns of clinical presentation in children with linear morphoea and to establish whether linear morphoea follows the lines of Blaschko.
Methods  A retrospective chart review of 65 children with linear morphoea was performed. According to clinical photographs the skin lesions of these patients were plotted on to standardized head and body charts. With the aid of Adobe Illustrator a final figure was produced including an overlay of all individual lesions which was used for comparison with the published lines of Blaschko.
Results  Thirty-four (53%) patients had the en coup de sabre subtype, 27 (41%) presented with linear morphoea on the trunk and/or limbs and four (6%) children had a combination of the two. In 55 (85%) children the skin lesions were confined to one side of the body, showing no preference for either left or right side. On comparing the overlays of all body and head lesions with the original lines of Blaschko there was an excellent correlation.
Conclusions  Our data indicate that linear morphoea follows the lines of Blaschko. We hypothesize that in patients with linear morphoea susceptible cells are present in a mosaic state and that exposure to some trigger factor may result in the development of this condition.     

Elastophagocytosis in extragenital lichen sclerosus

Background: Elastophagocytosis, or elastic fiber phagocytosis by multinucleate macrophages, has been observed in different skin conditions that may or may not occur on sun‐exposed skin. Although loss of elastic fibers has been well documented in the homogenized papillary dermal zone in lichen sclerosus (LS), elastophagocytosis, to the best of our knowledge, has never been observed. Methods: We encountered striking elastophagocytosis in a case of extragenital LS which prompted us to review all cases of LS diagnosed at the Skin Pathology Laboratory at Boston University over a 2‐year period to assess for the presence of elastophagocytosis. Results: In 7 of 35 patients diagnosed with LS (20%), we found prominent elastophagocytosis to be present either immediately below or at the junction of the homogenized collagen and the normal underlying reticular dermis. Interestingly, all the cases in which elastophagocytosis was observed were in extragenital locations. Conclusion: Elastophagocytosis was observed in 20% of LS cases, all of which were extragenital. We hypothesize that elastophagocytosis in LS, especially in extragenital sites, may not be an epiphenomenon but rather represents a contributing factor to elastic fiber loss in the hyalinized papillary dermal collagen that typifies this disease. Abbas O, Chatrath V, Goldberg LJ. Elastophagocytosis in lichen sclerosus.     

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.     

Linear and Whorled Nevoid Hypermelanosis

Abstract: A case of hyperpigmentation distributed in streaks and whorls was recently delineated and separated from a confusing number of pigmentary disorders, A 13-year-old boy had hyperpigmented, reticu-late spots on his trunk and neck in a linear and whorted distribution fol-lowing the lines of Blaschko. Onset was at age 1 year. Histologically, the spots showed increased melanin In the basal layer of the epidermis, but no increase In melanocytes, Incontinence of pigment, or melanophages were seen in the dermis. The karyotype was 46, XY and there was no evi-dence of chromosomal mosaicism or chlmerlsm. This entity represents a disorder separated recently from other pigmentary alterations that follow Blaschko lines, such as Bioch-Sulzberger incontinentia pigmentl, hy-pomelanosis of ito, early systematized epidermal nevus, and chimerism.     

Bilateral Systematized Epidermolytic Verrucous Epidermal Nevus: A Rare Entity

Verrucous epidermal nevi are congenital, noninflammatory cutaneous hamartomas composed of keratinocytes. They follow the lines of Blaschko and show hyperkeratosis without cellular atypia. The routine histology shows variable amount of hyperkeratosis, acanthosis and papillomatosis and rarely epidermolytic hyperkeratosis. We saw a 3-year-old boy with bilaterally symmetrical, systematized verrucous plaques along the lines of Blaschko extensively involving the trunk and extremities but sparing the face and palmoplantar skin. Histopathology showed features of epidermal nevi with prominent epidermolytic hyperkeratosis. We report here the case for the rarity of this entity.     

Diagnosis and Treatment of Lichen Sclerosus

Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin. LS is a debilitating disease, causing itch, pain, dysuria and restriction of micturition, dyspareunia, and significant sexual dysfunction in women and men. Many findings obtained in recent years point more and more towards an autoimmune-induced disease in genetically predisposed patients and further away from an important impact of hormonal factors. Preceding infections may play a provocative part. The role for Borrelia is still controversial. Trauma and an occlusive moist environment may act as precipitating factors. Potent and ultrapotent topical corticosteroids still head the therapeutic armamentarium. Topical calcineurin inhibitors are discussed as alternatives in the treatment of LS in patients who have failed therapy with ultrapotent corticosteroids, or who have a contraindication for the use of corticosteroids. Topical and systemic retinoids may be useful in selected cases. Phototherapy for extragenital LS and photodynamic therapy for genital LS may be therapeutic options in rare cases refractory to the already mentioned treatment. Surgery is restricted to scarring processes leading to functional impairment. In men, circumcision is effective in the majority of cases, but recurrences are well described. Anogenital LS is associated with an increased risk for squamous cell carcinoma of the vulva or penis. This review updates the epidemiology, clinical presentation, histopathology, pathogenesis, and management of LS of the female and male genitals and extragenital LS in adults and children.     

"Pediatric blaschkitis": expanding the spectrum of childhood acquired Blaschko-linear dermatoses

Abstract:  We describe two young children who developed relapsing, pruritic, papulovesicular eruptions in multiple bands along Blaschko lines on the neck, trunk, and extremities. Skin specimens in both revealed spongiotic dermatitis. This represents the first report of "blaschkitis" in children, providing further evidence that lichen striatus and blaschkitis are related acquired Blaschko-linear dermatoses that exist on a spectrum rather than as the childhood and adult form of a single disease entity. We highlight the features that differentiate blaschkitis from lichen striatus, review the potential roles of cutaneous mosaicism, environmental triggers, and background immunologic state in their pathogenesis, and discuss the spectrum of inflammatory dermatoses that can follow Blaschko lines.