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Superimposed segmental dermatitis with chronic prurigo

European Journal of Dermatology. Volume 19, Number 4, 337-40, July-August 2009, Genes and Skin

DOI : 10.1684/ejd.2009.0678

Summary

Author(s) : Yasuhiro Kawachi, Shijima Taguchi, Yasuhiro Fujisawa, Junichi Furuta, Yasuhiro Nakamura, Yoshiyuki Ishii, Fujio Otsuka , Department of Dermatology, Institute of Clinical Medicine, University of Tsukuba, 1-1-1, Tennodai, Tsukuba, Ibaraki 305-8575, Japan.

Summary : Common acquired skin diseases with a polygenic background, such as lichen planus, may show linear or segmental manifestations of underlying systemic skin disease. The linear arrangement in such cases is usually consistent with the lines of Blaschko. Happle summarized the various types of segmental arrangement of common polygenic diseases and proposed a novel designation of superimposed segmental dermatosis. Here, we report a unilateral linear dermatitis distributed along the lines of Blaschko on the leg, which was not self-healing and persisted for at least 6 years without complete remission, and was accompanied by preceding chronic prurigo on the extremities. Histological examination showed subacute spongiotic dermatitis and epidermal infiltration of CD4-positive cells. This case report presents a superimposed segmental dermatitis that arose based on systemic eczematous conditions, such as chronic prurigo.

Keywords : Blaschko lines, superimposed segmental dermatitis

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ARTICLE

Auteur(s) : Yasuhiro Kawachi, Shijima Taguchi, Yasuhiro Fujisawa, Junichi Furuta, Yasuhiro Nakamura, Yoshiyuki Ishii, Fujio Otsuka

Department of Dermatology, Institute of Clinical Medicine, University of Tsukuba, 1-1-1, Tennodai, Tsukuba, Ibaraki 305-8575, Japan

accepté le 3 Mars 2009

Patients with systemic monogenic hereditary skin diseases sometimes develop segmental cutaneous manifestations of underlying systemic skin disease. Two different types of segmental manifestation are seen in autosomal dominant skin diseases: type 1 reflects heterozygosity of a de novo postzygotic mutation occurring at an early stage of embryogenesis, while type 2 reflects postzygotic mutation at an early developmental stage and results in loss of heterozygosity (LOH) in heterozygous embryos that later develop skin lesions with a non-segmental, diffuse distribution [1]. On the other hand, Happle recently proposed a “superimposed” segmental manifestation, applicable to segmental cutaneous lesions arising within a background of systemic polygenic skin disorders, because discrimination between type 1 and type 2 segmental manifestations is impossible in polygenic disorders [2]. According to this concept, LOH occurring in one of the important genes involved in predisposition to the underlying systemic disease may be considered the pathogenesis of the segmental linear lesions. Previous case reports describing segmental psoriasis, lichen planus, lupus erythematosis, atopic dermatitis, pemphigus, vitiligo, graft-versus-host disease, granuloma annulare, erythema multiforme, and drug eruption occurring in cases of the same systemic diseases have been re-evaluated with reference to the concept of superimposition [2].

Here, we report a case of superimposed segmental dermatitis that persisted for at least 6 years without complete remission, and was accompanied by preceding chronic prurigo on the extremities. This is the first report of superimposed segmental manifestation within a background of systemic chronic prurigo.

Case report

A 75-year-old man with a 20-year history of chronic prurigo mainly on both arms, presented with a 3-year history of unilateral pruritic linear eruptions on the left leg. Physical examination revealed discrete and grouped erythematous papules and plaques on the flexor side of the left leg, which became flattened on the buttock. The eruptions were arranged in a linear shape corresponding to Blaschko’s lines (figure 1A) and the eruptions on the thigh were apparently eczematous papulovesicles with exudative crusts (figure 1B). The most distal eruptions on the leg became erosive and exudative due to the patient’s scratching (figure 1C). There were also typical pruriginous and eczematous lesions with itching on both arms (figure 1D) and the trunk. A skin biopsy taken from the linear-shaped eruption on the left leg revealed subacute spongiotic dermatitis with hyperkeratosis, acanthosis, exocytosis of lymphoid cells, intercellular edema, spongiosis of epidermis, and a fairly dense lymphohistiocytic infiltrate in the papillary and upper dermis (figures 2A and B). The patient showed no histopathological findings indicative of a lichenoid reaction, such as liquefaction degeneration of the epidermis, Civatte bodies, or band-like infiltration of lymphoid cells beneath the epidermis. Immunohistochemical examination demonstrated that the majority of infiltrating lymphocytes in the epidermis and the upper dermis were CD4-positive cells (figures 2C and D), and numerous CD1a-positive Langerhans cells were detected in the epidermis (figure 2E). A subsequent biopsy from a prurigo lesion on the dorsal aspect of the right forearm indicated more chronic dermatitis with hyperkeratosis, parakeratosis, acanthosis, and less spongiosis in the epidermis (data not shown). Topical treatment with 0.05% betamethasone butyrate propionate ointment partially improved the linear eruption. However, during the 3-year observation period, the linear eruption did not disappear completely and was exacerbated after cessation of therapy.

Discussion

Several acquired inflammatory linear dermatoses, including linear lichen planus, lichen striatus, Ulinear psoriasis, CHILD (congenital hemidysplasia with ichthyosiform erythroderma and limb defects) nevus, and ILVEN (inflammatory linear verrucous epidermal nevus), could be considered in differential diagnosis in the present case. In rare cases, lichen planus may be arranged linearly. However, lichen planus is characterized histologically by a subepidermal inflammatory infiltrate associated with damage to the epidermal basal layer and lack of epidermal spongiosis. Lichen striatus usually occurs during childhood and is not dominated by spongiosis and the infiltrate lacks eosinophils. Moreover, immunohistological examination was performed to determine the T-cell subset (CD4 and CD8) and CD1a-positive cells in the epidermal infiltrate. Lichen planus and lichen striatus frequently show epidermal infiltrate, consisting mainly of CD8-positive T cells [3-5] and decreased CD1a-positive cells [5, 6], which were not noted in the present case. The CD4-dominant epidermal infiltrate and the increased number of CD1a-positive Langerhans cells observed in this case reflected the eczematous nature of the pathogenesis of this disease. Psoriasis rarely shows obvious spongiosis, which was noted in our case. CHILD nevus and ILVEN are usually present at birth or arise in childhood and are recalcitrant to topical steroid treatment.

The Blaschko lines are currently thought to reflect cell migration and clonal expansion of embryonic skin tissue during embryogenesis [7] and the association of Blaschko lines with genetic mosaicism has led to the hypothesis that the pattern represents genetically abnormal skin contrasting with genetically normal skin [8]. The genetic mosaicism that appears along Blaschko lines is considered to result from somatic postzygotic mosaicism. Authentic genetic mosaicism has been confirmed in X-linked diseases, such as incontinentia pigmenti [9]. However, there is little evidence regarding how the clonal hypothesis of Blaschko lines is related to the pathogenesis of acquired inflammatory diseases along the lines of Blaschko. Karyotype analysis carried out in a patient with Blaschko dermatitis showed various abnormalities involving chromosome 18 in lesional skin but not in abnormal-appearing skin [10], while the molecular mechanism through which the genetic mosaicism can lead to linear cutaneous inflammation along the lines of Blaschko remains unclear. However, the segmental manifestation of eczema along Blaschko’s lines indicates a crucial role of lesional skin cells, such as keratinocytes, fibroblasts, or vascular endothelial cells, as well as immune cells, in the pathogenesis of this disease.The designation “acquired Blaschko dermatitis” has been applied to acquired unilateral eczematous linear lesions arranged along the lines of Blaschko. This condition was first described by Grosshans and Marot in 1990 [11] as “Blaschkite de l’adulte”, and a second case of this disease entity was reported subsequently by Megahed et al. as “acquired relapsing self-healing Blaschko dermatitis” emphasizing the recurrent nature of the disease [12]. Only a few cases have since been reported [10, 13-15]. These cases differ significantly from the present case with regard to the absence of an underlying systemic eczematous condition. The coexistence of the segmental eczematous lesion with systemic chronic prurigo in the present case indicates the increased susceptibility of the lesional skin to eczema based on the underlying systemic eczematous immune condition. Happle recently proposed the designation “superimposed” segmental manifestation applicable to segmental cutaneous lesions arising within a background of systemic polygenic skin disorders. According to this concept, the case presented here is an example of superimposed segmental manifestation along Blaschko lines of a systemic eczematous condition due to chronic prurigo. The persistent course in the present case may have been due to the underlying systemic eczematous condition, which augments the activity of the lesional eczema. As examples of superimposed segmental manifestation of systemic eczematous conditions, two cases of linear-shaped dermatitis arising within a background of atopic dermatitis have previously been reported [16, 17].

Acknowledgments

Conflict of interest : none. Financial support : none.

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