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Clinical/Introduction
Jessner's lymphocytic infiltrate of skin, first described in 1953, presents clinically as papules and
indurated erythematous plaques primarily confined to sun-exposed areas, namely the neck and upper trunk.
Although it can occur in childhood, the majority of patients are in the third through fifth decades of life.
Lesions are usually several in number and resolve spontaneously without scarring. Administration of
antimalarials can facilitate clearing of the eruption. The question arises as to whether or not Jessner's
lymphocytic infiltrate represents a form of non-scarring discoid lupus erythematosus (DLE). There is a
considerable overlap clinically and histologically between Jessner's lymphocytic infiltrate of skin and
non-scarring discoid lupus erythematosus. The absence of serologic and/or extra cutaneous stigmata of
collagen vascular disease are observed in both entities. Furthermore, immunofluorescent testing in lesions
of non-scarring/tumid lupus erythematosus is negative.
Histopathology
One observes superficial and deep perivascular and peri-adnexal mature lymphocytic infiltrates. In a
minority of cases, there may be extension into the subcutaneous fat. An active interface dermatitis
involving the interfollicular epidermis is not observed.
Although there may be permeation of the outer root sheath epithelium by lymphocytes, degenerative epithelial
changes are not observed. There is variable splaying of the dermal connective tissue fibers by mucin.
Lupus band testing is negative. There is no decoration of the keratinocytes for either IgG or C5b-9.
Immunophentoypic studies show a predominance of T cells. There may be a greater number of B cells in
lesions of Jessner's lymphocytic infiltrate relative to lupus erythematosus.
Differential Diagnosis
The differential diagnosis is primarily with non-scarring discoid lupus erythematosus (DLE)/tumid lupus
erythematosus, reticular erythematous mucinosis and lymphocytoma cutis. With respect to non-scarring DLE,
usually some element of interface change with epithelial destruction is observed involving either the
interfollicular epidermis or hair follicle. In a non-scarring DLE, the degree of epithelial injury is less
than in typical DLE, and hence, the absolute light microscopic distinction of Jessner's lymphocytic
infiltrate from this variant of discoid lupus erythematosus is very difficult. Practically speaking, it
likely does not matter as patients with this variant of lupus erythematosus do not have serologic stigmata
of collagen vascular disease and extracutaneous symptomology of collagen vascular disease is not present.
Both conditions respond to antimalarials. Regarding the distinction from PMLE, perhaps the most useful
light microscopic discriminators that would favor PMLE over Jessner's lymphocytic infiltrate is the presence
of striking papillary dermal edema and tissue eosinophilia. As well, there is no tendency for the
infiltrate to arrange itself around adnexal structures. With respect to lymphocytoma cutis, likely some
cases of exuberant Jessner's lymphocytic infiltrate could be classified as lymphocytoma cutis.
Immunophenotypically, Jessner's lymphocytic infiltrative skin, reticular erythematous mucinosis, and lupus
erythematosus is dominated by T-cells, whereas in lymphocytoma cutis, there is either an equal distribution
of T and B cells, or a dominance of B-cells. Lymphocytoma cutis may be triggered by various infective
stimuli including herpes infection and insect bite reactions.
NON-SCARRING DISCOID LUPUS ERYTHEMATOSUS/
TUMID LUPUS ERYTHEMATOSUS
Introduction/Clinical
We have encountered a distinctive group of patients who manifest skin lesions for which we use the
designation non-scarring discoid lupus erythematosus (DLE). These patients present with indurated
photosensitive plaques classically involving the head and neck, upper back and anterior chest which follow a
waxing and waning course and respond to intra-lesional injection of steroids and/or systemic administration
of Plaquenil. Patients usually feel well and have no other extracutaneous stigmata of collagen vascular
disease. Serologic testing is negative. Lupus band testing may fail to disclose a positive lupus band
test. Most consider the entity of tumid lupus erythematosus to be a variant of DLE, this is an exceedingly
uncommon form of lupus erythematosus in our experience. It presents clinically as a boggy plaque
classically involving the head and neck area.
Histopathology
Non-scarring DLE, as discussed above, shows overlap clinically and histologically with Jessner's lymphocytic
infiltrate. In common with Jessner's lymphocytic infiltrate are dense angiocentric and peri-adnexal
lymphocytic infiltrates accompanied by pandermal mucinosis. In non-scarring DLE, there is evidence of an
active interface dermatitis involving the interfollicular epidermis and/or the follicular epithelium with
some element of degenerative epithelial alterations although not of a sufficient magnitude to result in
scarring. As these patients do not have antibodies to extractable nuclear antigens, demonstration of
epidermal staining for C5b-9 or IgG is not demonstrated. The histopathology of tumid lupus erythematosus is
one of florid dermal mucinosis with a sparse perivascular and peri-adnexal lymphocytic infiltrate without
significant degenerative epithelial alterations.
OTHER DERMAL PERIVASCULAR LYMPHOCYTIC INFILTRATES:
Superficial and deep EAC histopathologically manifests discrete perivascular cuffs predominated by
lymphocytes which are tropic to blood vessels producing a "sleeve-like" histomorphology. In addition to
these sharply demarcated perivascular lymphoid infiltrates which spare the overlying epidermis in the deep
variant of the EAC, endothelial cell swelling and hemorrhage may be present and eosinophils are seen in up
to twenty percent of cases. Although an interstitial component may attend the sharply demarcated
perivascular infiltrates, a significant interstitial neutrophilic or eosinophilic infiltrate should call to
mind urticarial allergic eruptions, complement-mediated urticaria such as seen in the setting of collagen
vascular disease, and insect bite reactions. The supercial variant has concomitant low grade eczematous
changes with concomitant basilar vacuolar change.
With respect to polymorphous light eruptions, a few neutrophils are usually seen within the infiltrate and
papillary dermal edema may be more striking. In addition there are concomitant eczematous dermal changes.
Similarly, the dermal-based delayed-type hypersensitivity contact reactions generally have a significant
interstitial component, and lack sharp perivascular definition. In addition to the drugs mentioned above,
penicillins, thiazides and gold are associated triggers for EAC. With respect to differentiating deep EAC
from discoid lupus erythematatosus and Jessner's lymphocytic infiltrate, the latter two entities manifest
peri-adnexal infiltrates and significant dermal mucinosis; other inflammatory cell elements specifically
eosinophils are not present.
Discoid lupus erythematosus can be distinguished from Jessner's infiltrate, however, by degenerative
epithelial alterations involving follicular structures predominantly, but also the inter-follicular
epidermis and the eccrine coil and straight ducts.
References
- Clark WH Jr, Mihm MC Jr, Reed RJ, Ainsworth AM. The lymphocytic infiltrates of the skin. Hum Pathol
1974; 5:25-43.
- Crowson AN. Superficial and deep perivascular dermatitis. In : Barnhill R, Crowson AN, Busam K,
Granter S ed's. Textbook of Dermatopathology. New York : McGraw-Hill Co, 1998:69-81.
- Crowson AN, Magro CM. The cutaneous pathology of lupus erythematosus. J Cutan Pathol 2001;28:1-23.
- Elder D, Elenitsas R, Jaworsky C, Johnson B, Ed's. Lever's Histopathology of the skin, 8th Ed'n,
Philadelphia: Lippincott-Raven, 1997:
- Weedon D. Skin Pathology. Edinburgh: Churchill Livingstone. 1997.
OTHER GYRATE ERYTHEMAS
Erythema chronicum migrans, the characteristic cutaneous hallmark of Lyme disease demonstratese prominent
perivascular lymphocytic infiltrates with variable plasmacellular infiltration. There may be endothelial
cell swelling accompanied by variable dermal mucin deposition. In addition to a prominent angiocentric
disposition of the infiltrate a neuritis is frequently seen.
Erythema gyratum repens manifests as broad polycyclic patches which resemble the rings on the cut surface of
a tree. This eruption is associated with internal organ malignancies, tuberculosis, ichthyotic states,
pityriasis rubra pilaris, and CREST syndrome. Clinical mimics include atypical cases of autoimmune bullous
dermatoses such as bullous pemphigoid or linear IgA disease, and subacute cutaneous lupus erythematosus.
The histopathology of erythema gyratum repens comprises spongiosis with parakeratosis, focal mild
perivascular lymphoid infiltrates and variable edema or eosinophilia of the dermis.
Erythema maginatum is associated with rheumatic fever in less than 10% of cases and comprises erythematous
macules with a raised edge and a pale centre. Skin biopsies in erythema maginatum show perivascular
neutrophilic, lymphocytic and eosinophilic infiltrates with perivascular debris but absent vascular fibrin
deposition.
The overlying epidermis shows rare dyskeratotic cells and occasional intraepithelial neutrophils.
Differential diagnosis
With respect to erythema marginatum, other conditions which combine perivascular lymphocytic infiltrates
with a neutrophilic and eosinophilic component in the presence of perivascular leukocytoclasia but absent or
minimal fibrin deposition include toxic shock syndrome, serum sickness and, urticarial vasculitis.
References
- Brauner GJ, Mihm MC Jr, DesGroseilliers JP. Erythema marginatum in streptococcal endocarditis without
rheumatic heart disease. Cutis. 1973; 12: 206-215.
- Clark WH Jr, Mihm MC Jr, Reed RJ, Ainsworth AM. The lymphocytic infiltrates of the skin. Hum Pathol
1974; 5:25-43.
- Crowson AN. Superficial and deep perivascular dermatitis. In : Barnhill R, Crowson AN, Busam K,
Granter S ed's. Textbook of Dermatopathology. New York : McGraw-Hill Co, 1998:69-81.
- Campion RH. Annular erythemas. In : Champion RH, Burton JL, Ebling FJG. Textbook of Dermatology, 5th
Ed'n. Oxford : Blackwell Scientific Publications 1992:1839-1842.
- Magro CM, Crowson AN. The clinical and histological spectrum of IgA-associated vasculitis. Am J
Dermatopathol 1999;21:234-240.
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