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Linear and whorled nevoid hypermelanosis is a rare skin pigmentation disorder, characterized by linear streaks and whorls of hyperpigmented macules along Blaschko\'s lines. Lesions are commonly restricted to the trunk, neck, and extremities, sparing the face, palms, soles, and mucosae. Associated with this, certain cardiovascular, musculoskeletal, neurological, and developmental anomalies have been reported in the literature. Herein, we present a rare case of linear and whorled nevoid hypermelanosis involving the face, with musculoskeletal, genital, aural, and ocular abnormalities.

Lichen planus pigmentosus (LPP) is a distinctive variant of lichen planus described for the first time in 1974. A wide range of presentations and distribution patterns have been reported, mainly presenting as dark brown hyperpigmented macules and patches, typically involving sun-exposed areas such as the face, neck, and flexural folds. It is more common in dark-skinned patients with a female predominance. Infrequently, LPP may present as a Blaschkoid distribution, with only a few cases reported in the medical literature. We report an unusual case of LPP that developed with a unilateral Blaschkoid distribution on the left side of the trunk in a 32-year-old female following weight reduction surgery.

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Lichen nitidus and morphea are common diseases, but an associated localization of both lesions is rare. Here, we describe the first case of lesions distributed along Blaschko\'s lines. A 24-year-old Japanese woman was referred to our clinic for evaluation of band-like plaques of 18-months history on the right lateral side of her abdomen. In addition, multiple milky-white papules were seen within the plaques. Histopathological examination showed there was sclerosis in the lower half of the dermis and well-circumscribed, dense, papillary dermal lymphohistiocytic aggregations showing a so-called \"claw clutching a ball.\" Immunohistochemical analysis revealed that the morphea and lichen nitidus had similar characteristics. We speculated that unique immunologic events led to the development of lichen nitidus and morphea in our patient.

Hypomelanosis of Ito is a rare heterogeneous neurocutaneous disorder often associated with central nervous and musculoskeletal system involvement. Herein, we report the first case of hypomelanosis of Ito in the literature presenting with unilateral dilation of Virchow-Robin spaces (VRS). A girl aged 16 years old presented with a 1-year history of headache. Her physical and neurological examinations were normal, except for the presence of unilateral cutaneous macular hypopigmented whorls and streaks on lower side of the right trunk and lower limb, termed as Blaschko\'s lines. She had mild deficits in cognitive and adaptive functioning. Hearing, renal, dental, ophthalmologic, metabolic, and cardiac assessments were normal. Brain magnetic resonance imaging (MRI) showed markedly unilateral hemispheric enlarged VRS without contrast enhancement and diffusion restriction. To the best of our knowledge, our case is the first report describing the unilateral hemispheric enlarged VRS in a patient with hypomelanosis of Ito. Our report suggested that hypomelanosis of Ito may have unilateral dilation of VRS in brain MRI.

Scleredema is an idiopathic condition with specific clincopathological findings, including symmetrical and diffuse nonpitting induration of the upper body and thickened dermis due to deposition of acid mucopolysaccharides. Here, we report a case of linear scleredema in a healthy female patient, who presented with firm induration of the skin over her right shoulder and arm in a Blaschkolinear distribution. The onset was insidious in childhood and stable over several years. While the clinical picture has closely mimicked linear morphea, the diagnosis of scleredema was confirmed by the typical histopathological findings. To the best of our knowledge, linear scleredema is a unique presentation that has not been previously reported.

Linear hyperpigmentation is an unusual anatomical configuration in clinical dermatology. Owing to its rarity, consensus on the most effective method of classification is lacking. While linear hyperpigmentation generally follows Blaschko\'s lines, this is not universal. Clinical findings such as adherence to Blaschko\'s lines, associated morphological findings (including other cutaneous lesions), and systemic manifestations can be used to further characterize and diagnose variants of the disorder. Early detection of any underlying disease is vital, especially in cases with effective management, because the disorder may make it difficult to manage hyperpigmentation. Herein, we introduce a logical clinical diagnostic approach that represents a useful tool for dermatologists to efficiently evaluate patients presenting with linear hyperpigmentation. A simplified systematic and evidence-based approach is useful for this clinical condition owing to the heterogeneous causes and lack of specific diagnostic tools.