Correlation clinicopathologic of attributes morphologic in more than 60 patients diagnosed as having KLC

 
Review of the literature pertinent to the subject of KLC uncovers more than 60 patients in whom was made the diagnosis of KLC or of one of the designations that have come to be used synonymously with it, among those being Nekam disease, lichen verrucosus et reticularis, lichenoid tri-keratosis, keratose lichenoide striee, porokeratosis striata, morbus moniliformis, and lichen ruber moniliformis. Twelve of these reports were published subsequent to the work of Massi, Chiarelli, and Ackerman in 1997. Three categories emerge based on whether the findings presented in a particular article
 
1) do not permit any diagnosis to be rendered,
 
2) do allow a diagnosis specific to be made, such as of lichen simplex, lichen planus, or lupus erythematosus,
 
3) do not correspond to any disease well defined, such as lichen simplex, lichen planus, lupus erythematosus, but seem to show attributes morphologic, clinically and histopathologically, that are repeatable.
 
A summary of all patients studied for this article can be found in Table 1.
 

Category 1

 
No small number of patients reported on with a diagnosis of KLC display features morphologic very dissimilar from one another and different also from what was pictured in the seminal articles of Kaposi, Nekam, and Margolis. [9, 16, 34–45] Figs. 10 and 11 capture lesions clinical and histopathologic in patients diagnosed with KLC, the findings pictured being very different from what was depicted in the reports original by Kaposi, Nekam, and Margolis. A denominator in common is that the findings shown do not enable a diagnosis to be reached with surety. In the very few pieces in which more than a single patient was presented, the illustrations convey changes of more than just one disease; [16, 33, 40] e.g., in the publication by Menter, one patient had systemic sclerosis and another prurigo nodularis. Some patients diagnosed as having KLC probably had several diseases concurrently rather than a single entity distinctive. [10, 36, 39, 46] In a number of brief "reports of a case," neither photographs of lesions clinical nor photomicrographs were shown and, moreover, the findings described by the authors do not permit a specific diagnosis to be established. [10, 47, 48]

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Figs. 10A–B  Diagnosis not apparent. In this patient reported on by Jayaraman in 2003, the features clinical and findings histopathologic do not conform to those of any disease established well. They are very different from those in patients with what was purported to be KLC by Kaposi, Nekam, and Margolis. Reproduced with permission [35].

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Figs. 11A–C  Diagnosis not apparent. The attributes morphologic in the skin of this patient reported on by Mevorah in 2001 do not fit neatly with those of any disease established firmly and they do not correspond to what was described as KLC by Kaposi, Nekam, and Margolis. Reproduced with permission [43].
 

Category 2

 
Several patients diagnosed as having KLC show variations on the theme of lichen simplex chronicus, including those known as severe "atopic dermatitis" and prurigo nodularis. [2, 9, 20, 49] Although many authors claimed to have observed "typical features" histopathologic of KLC, they usually do not picture them, [50] the lesions clinical actually pictured being those, for example, of "atopic dermatitis."
 
In many patients, "lichenoid dermatitides" of different types established were not excluded from consideration definitively, e.g., in a young patient reported on by Patrizi and coworkers in whom changes in photomicrographs could be interpreted as those of pityriasis lichenoides. [51] In other articles, lichen planus could be considered on the basis of findings histopathologic. [16, 23, 46, 52–56] A child observed by Raynaud, [41] a child reported on by Arata et al., [42] and a 37-year old woman presented by Nabai [44] probably had lupus erythematosus, a disease mentioned in the differential diagnosis put forth by the authors, but excluded by them because studies serologic were negative for autoantibodies. A patient told of by Pinol-Aguade and associates in 1974 presented himself with plaques whose border was prominent. [17] On examination by conventional microscopy, the lesions showed cornoid lamellation centered in acrosyringia of eccrine units. The authors designated the condition "lichenoid tri-keratosis," a term other authors deemed to be synonymous with KLC. The findings pictured are different from those shown in the articles by Kaposi, Nekam, and Margolis, and seem to be ones of a particular type of porokeratosis. A patient reported on by Kint and colleagues had a blistering disease, not KLC; the lesions resolved with formation of milia. [57] The changes pictured could be interpreted as being those of a type of dystrophic epidermolysis bullosa. The presentation clinical of the patient is somewhat reminiscent of that of the patient reported on by Mehregan and coworkers in 1984. [6] Figs. 1217 are taken from articles in which authors made a diagnosis of KLC, but the lesions clinical and the photomicrographs indicate that the diagnosis is something other than KLC, e.g., lichen simplex, prurigo nodularis, hypertrophic lichen planus, lupus erythematosus, and dystrophic epidermolysis bullosa.

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Figs. 12A–B  This patient, diagnosed as having KLC by Remling et al. in 2002, had lichen simplex chronicus as judged both by criteria clinical and histopathologic. Note the signs of lichenification and of excoriation, and the hypergranulosis and hyperkeratosis. Reproduced with permission [49].

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Figs. 13A–B  Lesions of this patient, diagnosed as having KLC by Chikama in 1999, could be interpreted as being those of hypertrophic lichen planus. Reproduced with permission [53].

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Figs. 14A–B  In this patient, diagnosed as having KLC by Haas et al. in 2001, the attributes of lesions clinical could be those of lichen planus on which has been imposed evidences of rubbing forcefully and persistently. Reproduced with permission from [46].

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Figs. 15A–B  This condition cutaneous said to be KLC by Raynaud in 1983 more likely is lupus erythematosus. Reproduced with permission [41].

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Figs. 16A–B  The disease in this patient was diagnosed by Arata in 1993 as being KLC. The changes pictured, however, could be those of chronic discoid lupus erythematosus. Reproduced with permission [42].

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Figs. 17A–B  This patient was diagnosed by Kint and colleagues in 1976 as having KLC. The findings histopathologic are those of a subepidermal blistering disease that resolves with scarring and formation of milia. Among the possibilities for diagnosis is dystrophic epidermolysis bullosa. The changes are similar to those of the patient reported on by Mehragan in 1984. Reproduced with permission [57].
 

Category 3

 
Only a few patients reported on presented themselves with lesions very similar to one another clinically, namely, an eruption that involved the face in a manner reminiscent of seborrheic dermatitis and with tiny papules on the trunk and extremities, that assumed linear and reticulate shapes by way of confluence of lesions. [19,27, 29–31, 58] In several reports, the lesions pictured seem to be ones of the very same condition. [2,5,12,21,22,28, 59–65] Unfortunately, some of those reports were devoid of even a single photomicrograph, [18, 22, 29, 59–61, 64] excluding thereby the possibility of coming to a diagnosis with certainty. Pictures of lesions clinical and photomicrographs are reproduced in Figs. 1829, illustrations of lesions in one patient of our own being included.

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Figs. 18A–D  These pictures come from an article by Duperrat in 1977. Lesions were present on the face and extremities, individual ones being tiny papules that assumed a linear and a netlike outline by way of confluence of them. Histopathologically, there is uneven acanthosis and plugging of infundibula by parakeratosis. The composition of the infiltrate in the papillary dermis cannot be determined with surety. Reproduced with permission [19].

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Figs. 19A–D  These images were published in an article by Fraitag in 1989. Individual lesions tended to become confluent to form a netlike pattern. At sites of long-standing trauma mechanical, like on the foot, lesions became keratotic plaques. The photomicrograph shows an interface dermatitis with atrophy in loci and clusters of necrotic keratocytes. Reproduced with permission [27].

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Figs. 20A–E  This patient reported on by Skorupka in 1992 has lesions on the face and the extremities, they assuming a reticular pattern and being formed by a lichenoid dermatitis with atrophy, jagged acanthosis, and striking hyperkeratosis, most of it parakeratotic. Necrotic keratocytes are situated in the basal layer. Reproduced with permission [31].

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Figs 21A–B  The findings shown here come from the patient of Ruben and coworkers reported on in 1997. Clinically, the lesions are tiny papules that became confluent to create shapes linear and arcuate. Histopathologically, the changes are those of a lichenoid dermatitis with a thinned epidermis and parakeratosis.

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Figs 22A–C  The findings shown here come from a biopsy specimen taken from a patient of Aloi and Tomasini reported on in 1997. Individual papules have become confluent to form outlines linear and arcuate. Findings histopathologic are those of a patchy lichenoid dermatitis beneath an atrophic epidermis covered by hyperkeratosis, foci of which are parakeratotic.

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Figs. 23A–B  Features clinical and findings histopathologic shown in an article by Petrozzi in 1976. Although the photograph is wanting in quality, papules are seen to have assumed linear shape. Histopathologically, a lichenoid infiltrate is situated beneath an atrophic epidermis covered by hyperkeratosis, foci of parakeratosis being present. Reproduced with permission [11].

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Figs. 24A–B  In this patient of Kossard et al., tiny papules have become confluent in loci, the result being a pattern reticular. Kossard and coworkers described an interface dermatitis and took note of prominent parakeratosis in acrosyringia of eccrine units, a finding interpreted by them to be cornoid lamellation. The changes pictured in the photomicrographs, however, are scant. Reproduced with permission [62].

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Figs. 25A–B  Miller, in 1944, illustrated his article with these photographs. Unfortunately he did not include photomicrographs of a section from a biopsy specimen. The lesions are very similar, however, to those shown in Figs. 21–26. Reproduced with permission [56].

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Fig. 26  No photomicrograph was included in the article by Ryatt in 1982 but the lesions clinical are reminiscent of those shown in Figs. 21–27. Reproduced with permission [22].

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Fig. 27  Lesions clinical in a patient reported on by Degos et al. in 1974. Papules are tiny and could be infundibulocentric because some of them seem to be equidistant from one another. Some have become confluent to form an array linear. Reproduced with permission from [35].

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Fig. 28  These lesions from a patient recorded by Mac Donald and Williams in 1974 are very similar to those pictured by Duperrat et al, in 1977. Reproduced with permission [59].

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Figs. 29A–D  These photographs come from a patient of our own. Individual papules were infundibulocentric, some of them having a comedo-like horny plug in the center. Individual papules also were situated on palms and soles, and there seemed to be centered around acrosyringia. Lesions tended to become confluent to form a line or create a pattern reticular, especially on the acra of extremities. At sites exposed to trauma mechanical, papules became confluent to result in plaques covered by scales and crusts.

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Figs. 29E–L  Findings histopathologic in our patient were those of a lichenoid dermatitis, especially around infundibula, but also around acrosyringia. The bandlike infiltrate consisted of lymphocytes mostly. There were clusters of necrotic keratocytes and atrophy was present in loci, whereas uneven acanthosis was evident in other loci. Hyperkeratosis was constituted of zones of parakeratosis in fashion staggered, they sometimes housing debris of nuclei of neutrophils. Plasma cells and histiocytes were present in the vicinity of an erosion or a rupture of infundibular epidermis