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Keratosis lichenoides chronica: Report of a new case with partial response to PUVA therapy

  • Author(s): Douri, Thaer
  • Shawaf, AZ
  • et al.
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Keratosis lichenoides chronica: Report of a new case with partial response to PUVA therapy
Thaer Douri, and AZ Shawaf
Dermatology Online Journal 11(2): 28

Ministry of Health, Syria-Hama. s-dori@scs-net.org

Abstract

Keratosis lichenoides chronica is a rare disorder characterized by a distinctive seborrheic dermatitis-like facial eruption together with development of asymptomatic verrucosities on the limbs and the trunk, with a partially linear distribution. We report a case of a 35-year-old Syrian woman who presented with keratotic plaques and papules of the limbs for several years, and a seborrheic dermatitis-like facial eruption. A diagnosis of keratosis lichenoides chronica was clinically and histologically. Treatment with isotretinoin was without benefit. She had partial response to PUVA.



Clinical synopsis


Figure 1 Figure 2

Figure 3 Figure 4

A 35-year-old Syrian woman had a 5-year history of an asymptomatic eruption that appeared suddenly on her face, legs, and hand. It was initially diagnosed as lichen planus and treated with topical corticosteroid and tar, without benefit. Her general health was otherwise good; routine blood tests were within normal range. She did not use any systemic therapy. No family history of a similar eruption was found.

Examination revealed lesions on extremities consisting of lichenoid-violaceous papules presenting in a linear distribution on the dorsal hands (Fig. 1) and frontal thigh (Fig. 2). Erythematous papules with greasy scale and scattered papulopustules were present on the face (Fig. 3). The hair, mucous membranes, and nails are normal.

Histology showed hyperkeratosis and parakeratosis, irregular acanthosis, and lymphocytic infiltration in upper dermis (Fig. 4).


Discussion

Keratosis lichenoides chronica is a rare, acquired dermatosis of young adults. Its physiopathology remains elusive. The disease was originally described by Kaposi (as lichen ruber verrucosus et reticularis) but was named after Nekam who reported a typical case in 1938. The current descriptive term was introduced in 1972, and the disease is generally thought to represent a special form of lichen planus. Keratosis lichenoides chronica is a rare dermatosis characterized by violaceous papular and nodular lesions, often arranged in a linear and reticulate pattern on the dorsal hands and feet, extremities, and buttocks. The mucous membranes, genitalia, nails, palms, and soles may also be affected.

Seborrheic dermatitis-like scaling and telangiectasias of the scalp, face, and neck may develop [1]. Clinically, the eruption is usually asymptomatic, and keratotic papules disposed in parallel lines or small networks can be seen. In 50 percent of cases, the disorder involves the oral or genital mucous membranes, presenting as ulcerations, infiltration or inflammation [2]. The ocular area may be involved [3], as in the case initially described by Nekam [4]. Extension to the palmo-plantar zones is often reported, with an involvement of the nails in 30 percent of cases [2, 5].

Systemic complaints are absent as a rule or is any relationship to other diseases. Sclerodactylia was reported in a severe case of KLC with early onset [6]. In our patient, we did not find any associated pathology. Reports of KLC are particularly rare in childhood, and no familial cases have been described [7].

The course is chronic and progressive, extending over many years. Some elements may disappear spontaneously, leaving no scar or leaving residual pigmented atrophy [8]. in two cases a spontaneous resolution or remission was observed [9, 10].

KLC is resistant to topical therapy, and most case reports have confirmed the ineffectiveness of systemic corticosteroid drugs, antimalarial agents, sulfones, gold, superficial x-ray therapy, and cyclosporine. In some patients, PUVA [11, 12, 13]. Initially we treated with isotretinoin 25mg/day without benefit, then we decided to treated with PUVA twenty-six sessions (86.5 Joules) [13]; only partial response resulted.

References

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