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Dermatopathology: Practical & Conceptual

July – September 2010 | Volume 16, No. 3

Lichen planus or keratosis lichenoides chronica—another case discovered

Ziemer, Mirjana

Introduction

What today is called keratosis lichenoides chronica (KLC) was first described by Kaposi in 1895 [1]. The term “keratosis lichenoides chronica” was introduced only in 1972 by Margolis, when he recorded a patient with features similar to those of Kaposi’s patient. [2] KLC is a rare skin disorder, and some controversy exists as to whether KLC is an authentic disease. More than 60 patients can be found in the literature in whom the diagnosis of KLC was made but the patients seem to have different diseases, the most common among them being lichen simplex chronicus, lichen planus, and lupus erythematosus. [3] The few patients who indeed had KLC presented themselves with lesions very similar clinically, namely, an eruption that involved the face in a manner reminiscent of seborrheic dermatitis and with tiny papules on the trunk and extremities coalescing to linear and reticulate shapes. [3,4] Individual papules were infundibulocentric and acrosyringocentric. Histopathologically, a lichenoid interface dermatitis affiliated with numerous necrotic keratinocytes and covered by parakeratosis housing neutrophils in staggered fashion was encountered in these patients.

The history of KLC has been reviewed in a previous article in this journal. [3] Continued thorough study of historical literature of dermatology, however, time and again reveals surprises. In particular, in browsing the Archiv für Dermatologie und Syphilis from the late 19th and early 20th centuries, further cases of KLC have been identified. In past issues of this journal, two additional historical patients of KLC, one published by Bruck and another by Gunsett, have been added to the list of KLC-cases hitherto known. [5-8]

In this article, another historical case of KLC not yet recognized by contemporary professionals is presented. [9] In 1901, Bukovsky reported a patient of his own with what he thought were findings similar to lichen ruber moniliformis of Kaposi (Figs. 1-4). [9] The patient of Bukovsky was elaborated on in great detail and, moreover, was accompanied by representative clinical pictures and histological drawings. Therefore, the list of historical patients with KLC can be expanded.

Fig. 1

Front page of the volume of the Archiv für Dermatologie und Syphilis from 1901.

Fig. 2

First page of the article by Bukovsky. [9]

Fig. 3

Drawings of histopathologic findings in the patient of Bukovsky.

Fig. 4

Photograph of clinical findings in the patient of Bukovsky.

The patient

A 24-year-old washwoman presented herself to the department for the first time in 1898. The patient had previously been healthy except for measles. There had been no skin diseases in her family. The patient told that the first symptoms of the disease had appeared 2 years previously beginning with small areas on the flexor aspect of the forearms in the form of flat, slightly elevated papules which were isolated and of a bright red color. The patient mentioned a slight burning as an accompanying symptom. The lesions recently had enlarged rapidly and had extended from the upper extremities over face, feet, and trunk finally. At that time, the lesions had begun to form lines and had become confluent to form continuous streaks. The lesions were intensely pruritic but the patient avoided scratching because the slightest friction, even from clothing, led to bleeding. At that time, the patient used several external household remedies as well as Fowler’s solution [comment from the author: a solution containing potassium arsenite], Lassar´s paste [comment from the author: an ointment containing zinc oxide, starch, and salicylic acid in a soft paraffin base] with and without ichthyol, and Hebra’s ointment [comment from the author: containing melted lead], without improvement.

Clinical examination revealed the following: “The face is affected symmetrically including the upper lip, the dorsum and tip of the nose, the upper parts of the cheeks, and the supraorbital regions, scantly also the chin. At these sites, numerous dense, round and conic papules are found protruding significantly over the skin surface and having a pale red color. The surface of the smaller papules is covered by a thin scale. The larger papules show a whitish-yellowish scale adhering intensely to the base. After removal of the scale the denuded papillary dermis is bleeding. The periphery of the lesions is either normal or shows a slight hyperemic halo. The lesions are partially isolated or confluent to smaller groups covered by thicker scales. On the remaining face distinct hints of seborrhoea can be mentioned. Similar lesions are found on the dorsal part of the trunk, symmetrically on both sides, reaching from the shoulders to the haunches. On the back, lesions do not show any tendency to coalesce, only partially small groups are formed resulting in a mosaic-like picture. On the front part of the trunk, analogous lesions are distributed symmetrically, too. The same was found on the buttocks and abdomen. . . . The most interesting distribution was on the extremities. Symmetry was here the most impressive. Lesions had the tendency to line up in rows. . . . On the hands lesions are restricted to the dorsal parts. Volar aspects are totally spared. Isolated lesions on the fingers are always restricted to hair follicles. Lesions on the lower extremities are less numerous, more isolated, and not as prominently arranged in lines and streaks. . . . On the lateral aspects of the feet, however, again a lining up of lesions beginning from the digits up to the malleoli is striking. The involvement of the palms does show solitary horny papules. . . . Concerning mucous membranes the conjunctivae and genital sides were not affected. On the hart and soft palate were few elevated, slightly red, hemp-sized lesions with a whitish epidermis. The mucous membrane of the pharynx was that of a chronic catarrh. . . . The patient was treated with arsenic, however, without sufficient success. Moreover, during the stay in the hospital, new lesions appeared on the nasolabial folds and on the trunk. The patients was lost for follow up for some time and presented again in January 1899. Treatment was commenced again with arsenic and moreover plaster of 10-20% salicylic acid, gauze compresses with Unna´s ointment (comments of the author: ointments with mutton tallow or lanolin placed on both side of a gauze compress) zinc or ichthyol. After 4 weeks of treatment a much better improvement was mentioned compared with the last stay. . . . A third time the patient was investigated in February 1900 with extensively analogous changes. At that time skin biopsies from an isolated as well as from a linear lesion were performed. Biopsies were fixed in formalin and alcohol and serial section were performed. The sections were stained with Cochenille. Histologic investigation of serial sections gave the following results: Papules presented as wedge shaped formations extending over several papillae comprising the epithelial part and the upper half of the corium. It arises, that papules are not confined to adnexal structures such as hair follicles, sebaceous glands and sweat glands, as papules can easily be detected also independently from those structures. Than there are papules which contain in their peripheral parts hair bulges or acrosyringia. It is remarkable that in the middle of each papule there is a depression . . . Depressions are filled with a horny layer. .. In some papules, the horn masses are detached and thereunder were large amounts of leukocytes, than the color of the red blood cells, again thereunder epithelial cells, partially isolated, partially in groups, however, concluding after their stainability definitively dying. . . . Aside from the depression also a hyperkeratosis is found, however not at the same degree as in other places. . . . The granular layer of the conical papules is unevenly developed. . . . The Malipighian layer is thickened. . . . Towards the centre of a papule the cells stain less intense. The intercellular lymphatic gaps become wider and are filled with increasing amounts of leukocytes, so that the demarcation between epithelium and corium becomes blurred. . . . Only at higher magnification solitary degenerated epithelial cells are seen. . . . In the corium changes are found partially in the upper parts, to wit in the papillary and subpapillary parts, partially and mainly around vessels … The infiltrate is not infiltrating the epithelial cells of the adnexae. Considering the cellular components of the infiltrate . . . we find only mononuclear cells with a large round nucleus and scant protoplasm. . . . Among the infiltrate and between collagen bundles numerous erythrocytes are found. “

Author's conclusion

The author came to the conclusion that the condition in his patient represented a variant of lichen ruber in partim accuminatus. Nevertheless, the author recognized that the lesions were much more intense than in previously reported cases of mixed lichen ruber and lichen ruber accuminatus, moreover, they dominated on the extremities. Furthermore, the case differed from others by its immense hyperkeratosis, the bleeding, and the perennial, stable persistence of lesions. Moreover, the flat lesions were not as typical as the authors were used to seeing in lichen planus. In particular, they lacked the waxy bright surface. Bukovsky was convinced that his case proved Kaposi’s theory that lichen planus Wilson and lichen accuminatus Hebra were identical.

Comment

The case nicely documents clinical and histological aspects of KLC. The eruption involved the face in seborrheic areas with tiny papules. On the trunk and mainly on the extremities, lesions assumed linear and reticulate shapes by way of confluence of them. Although histologically individual papules were not restricted to adnexal structures, Bukovsky´s descriptions imply a folliculocentric lesion and, moreover, the drawings of the histopathologic changes show an acrosyringocentric involvement. Moreover, a lichenoid interface dermatitis with necrotic keratinocytes and parakeratosis housing neutrophils was encountered in this patient. The case showed some improvement after topical treatment but did not resolved completely. All these are features typical of what today is called KLC.

Summary

Keratosis lichenoides chronica is a rare skin disorder and some controversy exists as to whether it is an authentic disease. By way of study of the Archiv für Dermatologie und Syphilis from the late 19th and early 20th centuries, another case of keratosis lichenoides chronica has been identified. In 1901, Bukovsky reported a patient of his own with what he thought were findings similar to lichen ruber moniliformis of Kaposi. Descriptions and illustrations given in the publication are typical of what today is called keratosis lichenoides chronica.

Mirjana Ziemer, M.D., is a dermatopathologist at the Department of Dermatology and Allergology, Universitätsklinikum Jena, Friedrich Schiller University, Jena, Germany. This article was reviewed by Almut Böer-Auer, M.D., and Philip LeBoit, M.D. Contact author via e-mail: mirjana.ziemer@derma.uni-jena.de .

References

1. Kaposi M. Lichen ruber acuminatus et lichen ruber planus. Arch Dermatol Syph (Berl) 1895;31:1-32.

2. Margolis MH, Cooper GA, Johnson SA. Keratosis lichenoides chronica. Arch Dermatol 1972;105(5):739-43.

3. Böer A. Keratosis lichenoides chronica: Proposal of a concept Dermatopathol: Pract & Conc 2005;11:4.

4. Ruben B , LeBoit P. Keratosis lichenoides chronica is authentic. Dermatopathol: Pract & Conc 1997;5:310-2.

5. Gunsett A. Ein Fall von Lichen ruber moniliformis den subkutanen Venen folgend. Arch Dermatol Syph 1902;60:179-96.

6. Bruck C. Hyperkeratosis striata et follicularis und Lichen ruber atypicus. Arch Dermatol Syph 1911;106:91-104.

7. Ziemer M , Böer A. Keratosis lichenoides chronica or not? Dermatopathol: Pract & Conc 2005;11.

8. Ziemer M. A patient with keratosis lichenoides chronica. Dermatopathol: Pract & Conc 2007.

9. Bukovsky J. über Lichen ruber moniliformis (Kaposi). Arch Dermatol Syph 1901;57:143-68.