A Case of Linear Grover Disease Distributed along the Blaschko Line / 대한피부과학회지

Grover disease (also known as transient or persistent acantholytic dermatosis) is a pruritic polymorphic papulovesicular eruption that is histologically characterized by the presence of epidermal acantholysis. It primarily occurs in middle-aged individuals and manifests as scattered erythematous or brown papules as well as papulovesicles on the sun-exposed skin of the trunk. A 52-year-old man had erythematous papules and patches linearly arranged on the left thigh and leg with mild pruritus. The skin lesions were successfully treated with a topical corticosteroid. However, 2 months later, the lesions recurred. The histological examination of a punch biopsy revealed focal acantholytic clefts with dyskeratotic cells, hyperkeratosis, and the infiltration of perivascular lymphocytes and eosinophils. Taken together with the late onset and lack of family history, we diagnosed this condition as Grover disease distributed along the Blaschko line, a condition presented here for the first time.

Linear Lichen Nitidus Following the Blaschko's Line / 대한피부과학회지

No abstract available.

Lupus Profundus with Segmental Distribution on Leg, Following the Lines of Blaschko in a Child / 대한피부과학회지

Lupus profundus is a rare subtype of chronic cutaneous lupus erythematosus, which shows a tender subcutaneous nodule or plaque. The face, arm, buttock, trunk and thighs are frequently involved. Lupus profundus can be associated with or without systemic lupus erythematosus. But due to its rarity, it is difficult to diagnosis and there are not many reports regarding its characteristics, including its distribution. In this case, a 13-year-old boy has visited with several violaceous non-tender nodules on Lt. upper thigh with segmental distribution, which follows the Blaschko's line. Through skin biopsy and its immunoflourescent study, we diagnosed lupus profundus. We here report a case of lupus profundus with segmental distribution following the lines of Blaschko's in a child.

Linear Macular Amyloidosis Along the Lines of Blaschko / 대한피부과학회지

The primary localized cutaneous amyloidosis (PLCA) is classified into three types: macular amyloidosis, lichen amyloidosis, and nodular amyloidosis. Macular amyloidosis is characterized by pruritic, hyperpigmented macules and is most commonly located on the interscapular area. Skin lesion usually shows pigmentation with a reticulated or rippled pattern. We report an unusual case of linear macular amyloidosis along the lines of Blaschko. A 74-year-old male is presented with asymptomatic unilateral linear hyperpigmented macules on his right leg for 20 years. Skin biopsy has revealed eosinophilic cytokeratin-positive globular deposits occupying the dermal papillae.

Linear Hyperpigmentation Along the Blaschko's Line: A Clinicopathologic Study / 대한피부과학회지

BACKGROUND: Many pigmentary disorders can be manifested as linear streaks of hyperpigmentation, along the Blaschko's line. These include progressive cribriform and zosteriform hyperpigmentation (PCZH) and linear and whorled nevoid hyperpigmentation (LWNH). There have been debates on the universally accepted diagnostic criteria differentiating these disease entities. OBJECTIVE: To determine the clinicopathologic characteristics of hyperpigmentation along the line of Blaschko and to examine the acceptability of PCZH or LWNH criteria as a diagnostic tool for differentiating these diseases. METHODS: A retrospective study was conducted on 13 patients who presented with linear hyperpigmentation along the Blaschko's line. The patients' clinicopathologic characteristics were analyzed and matched with the PCZH/LWNH diagnostic criteria. RESULTS: Age of onset widely ranged from birth to 61 years, but predominantly before the age of 4 years. The male-female ratio was 1:1.2. Trunk was the most common site of involvement. Histologic examination commonly showed a basal layer hyperpigmentation in all patients and pigmentary incontinence was observed in 2 patients. Four patients who satisfied all the diagnostic criteria for PCZH also fulfilled the diagnostic criteria for LWNH, except for the timing of onset. An additional 4 patients satisfied all the diagnostic criteria for LWNH and also fulfilled the diagnostic criteria for PCZH, except for the timing of onset. Excluding the age of onset criteria, the other 3 patients fulfilled both diagnostic criteria for PCZH and LWNH. CONCLUSION: These results demonstrate that PCZH and LWNH should not be considered as a different disease entity and that supports the idea that these are part of the same disease spectrum.

Systematized Inflammatory Linear Verrucous Epidermal Nevus / 대한피부과학회지

Inflammatory linear verrucous epidermal nevi (ILVEN) were first described in the literature, in 1971, by Altman and Mehregan. Most cases were reported as solitary lesions. In contrast, systematized ILVEN, involving wide areas of the integument, has only rarely been reported. A variety of treatment modalities has been reported, ranging from topical medications, such as potent corticosteroids or tretinoin 0.1% to variable procedures, including CO2 and pulsed dye laser, cryotherapy, and surgical excision. However, multifocal skin lesion is more difficult to treat. An 8-year old boy was presented with an extensive thick scaly plaques and patches, affecting the trunk and the four extremities sparing face. It developed when he was 3 years old, and he complained pruritus. On physical examination, linear, or whirl-like scaly plaques were seen, along with Blaschko lines. Pathologic finding was in accordance to ILVEN. We treated him with topical calcipotriol, tacrolimus and systemic acitretin. After 8 months, the lesion and symptoms improved.

Linear Atrophoderma of Moulin with Bilateral Involvement / 대한피부과학회지

Linear atrophoderma of Moulin is a rare acquired atrophic skin condition following the lines of Blaschko. It is characterized by asymptomatic, band-like, hypopigmented atrophoderma, without obvious induration or sclerosis. Usually, the lesions begin in childhood or adolescence and are localized on the trunk or extremities with a unilateral distribution. Extensive lesions with bilateral involvement are extremely rare, and only three cases affecting patients bilaterally have been previously described. We herein present the case of a 17-year-old boy with multiple hyperpigmented atrophic patches, arranged in a linear pattern on both sides of the trunk, right buttock and leg, and left arm.

Linear Atrophoderma of Moulin Developed on the Neck / 대한피부과학회지

Linear atrophoderma of Moulin (LAM) is a distinct clinical entity characterized by acquired atrophic band-like skin lesions that often show hyperpigmentation and always follow the lines of Blaschko. In general, no preceding inflammation is noted, and there is no induration of scleroderma. Usually the condition begins in childhood or adolescence, and there is no evidence of any long-term progression. There are only about 30 reported cases and most of them are located in trunk and extremities. LAM that developed on the neck have been reported only once in the English literature. We report an uncommon case of LAM, which developed on the neck of a 57-year old woman.

Linear Lichen Sclerosus along the Blaschko's Line of the Face

Lichen sclerosus et atrophicus (LSA) is an inflammatory disease that primarily causes anogenital lesion in middle aged women. We present here a case of facial LSA with an asymptomatic, well-demarcated, whitish to bluish, atrophic patch in a linear pattern on the forehead of a 48-year-old woman. This case showed an atypical clinical presentation and it mimicked en coup de sabre, but the histopathologic results confirmed the diagnosis of LSA.

A Case of Blaschkitis / 대한피부과학회지

Blaschkitis is an acquired inflammatory dermatosis that develops along the line of Blaschko. It has a similar appearance to lichen striatus but, shows a somewhat different clinical course and histopathologic findings. Spongiotic dermatitis-like histopathology, good response to glucocorticoid therapy and frequent relapses are the characteristic features that can differentiate it from lichen striatus and other dermatoses which follows the line of Blaschko. We report a case of Blaschkitis that showed a typical clinical course and histopathologic findings.

Incontinentia pigmenti: Report of 3 cases from Sarawak

Incontinentia pigmenti, also known as Bloch-Sulzberger syndrome, is a rare X- linked dominant multisystem disease involving ectodermal structures namely cutaneous, ocular, dental, neurological and skeletal systems1. Mutation of the nuclear factor kappa B essential modulator (NEMO) gene in chromosome Xq28 is determined to cause this rare genodermatosis2. The cutaneous manifestations are the most characteristic features of this disorder3. We would like to report 3 cases of incontinentia pigmenti seen in the skin clinic, Sarawak General Hospital.

A Case of Acquired Blaschko Dermatitis in a Child; A Variant of Lichen Striatus, or Not?

Acquired Blaschko dermatitis is a rare inflammatory linear eruption that presents as multiple lines of itching papules and vesicles following the Blaschko's lines. Only ten cases have been reported in literatures since Grosshans' first description in 1990 and all cases have been in adults. A 2-year-old girl presented with a 4-month history of a slightly pruritic papulovesicular eruption, which was limited to the left side of her body, along the Blaschko's line. A skin biopsy revealed interface dermatitis. The eruption initially failed to respond to treatment with topical steroids but responded to systemic steroids. We present the first case of acquired Blaschko dermatitis in a child with a review of the relevant literature.

A Case of Multiple Linear Lichen Planus along the Lines of Blaschko / 대한피부과학회지

Linear lichen planus (LLP) is a rare variant of lichen planus, which accounts for 0.24-0.62% of all patients with lichen planus. Blaschko lines are the pattern assumed by many different nevoid and acquired skin diseases on the human skin, which present with a characteristic S-shape on the abdomen, a V-shape near the posterior midline, a linear pattern on the lower trunk and limbs, and whorls on the scalp and abdomen. Multiple LLP along the lines of Blaschko has been rarely reported. Differential diagnosis of LLP includes lichen striatus, epidermal nevi, linear psoriasis, inflammatory linear verrucous epidermal nevus (ILVEN), linear Darier disease, linear lupus erythematosus, and linear lichen nitidus. We herein describe a 49-year-old female who showed multiple linear erythematous, brownish papules on her right trunk, shoulder, arm, and groin with typical histopathologic findings of lichen planus.

A Case of Linear Atrophoderma of Moulin / 대한피부과학회지

Linear atrophoderma of Moulin is a disease characterized by hyperpigmented linear atrophoderma which follows Blaschko's lines. There is no preceding inflammation or subsequent sclerodermatous changes. The disease had not been reported in Korean literature since Moulin reported for the first time in 1992. The patient was a 21-year-old Korean female who presented multiple hyperpigmented linear macules on the face, arm and lower extremities since birth. All the lesions followed Blaschko" lines. Histopathologic findings showed mild acanthosis and perivascular lymphohistiocytic infiltration. No changes of dermal collagen and elastic fibers were observed.

A Case of Linear and Whorled Nevoid Hypermelanosis / 대한피부과학회지

Linear and whorled nevoid hypermelanosis(LWNH) is a congenital disorder which was first described by Kalter in 1988 and is characterized by asymmetrically distributed linear and whorled hyperpigmentation following Blaschko's lines. It usually appears at birth or during the first weeks of life and is rarely associated with various congenital defects. Its pathogenesis is not well known, but developmental somatic mosaicism appears the most likely cause. It is probably not a rare disease, but not well recognized or is confused with other entities. Herein, we report a typical case of LWNH in 9 month-old boy.