Lupus Miliaris Disseminatus Faciei Part I: Significance of Histopathologic Undertones in Diagnosis

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SKINmed: Dermatology for the ClinicianTM (ISSN 1540-9740) is published bimonthly (Jan., March, May, July, Sept., Nov.) by Le Jacq Ltd., Three Parklands Drive, Darien, CT 06820-3652. Copyright ©2005 by Le Jacq Ltd. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopy, recording, or any information storage and retrieval system, without permission in writing from the publishers. The opinions and ideas expressed in this publication are those of the authors and do not necessarily reflect those of the Editors or Publisher. For copies in excess of 25 or for commercial purposes, please contact Sarah Howell at [email protected] or 203.656.1711 x106.

Lupus Miliaris Disseminatus Faciei Part I

O r i g i n a l

C o n t r i b u t i o n

Lupus Miliaris Disseminatus Faciei Part I: Significance of Histopathologic Undertones in Diagnosis Virendra N. Sehgal, MD;1 Govind Srivastava, MD;2 Ashok K. Aggarwal, MD;2 Vishwanath Reddy, MD;2 Sonal Sharma, MD3

Background. Since its clinical discovery, lupus miliaris disseminatus faciei has sporadically been reported to have different modes of clinicopathologic expression. Objective. The purpose of this study was to work up a list of histopathologic undertones and to project and propagate lupus miliaris disseminatus faciei as an exclusive entity. An upcoming Part II of this study will present an overview of the disease. Methods. All patients visiting the outpatient clinic conforming to hitherto accepted clinical features were included to study patients’ age and sex, duration of the disease, and above all, conduct a detailed histopathology review. Using the detailed information obtained, an endeavor was made to organize the disease into early, fully developed, and late lesions. Results. The details of the various parameters were of great help in evolving this modus operandi. The clinical as well as histopathologic features of lupus miliaris disseminatus faciei are fairly distinct and facilitate visualizing the entity as a spectrum comprising early, fully developed, and late lesions. Further studies are called for. (SKINmed. 2005;4:151–156) ©2005

Le Jacq Ltd.

L

upus miliaris disseminatus faciei (LMDF), a chronic inflammatory disorder, has widely been regarded as an intriguing entity.1 Its etiopathogenesis is largely speculative. Precise description of its morphology, characterized by multiple, discrete, smooth, 1–3 mm, brown/red or brown May • June 2005

to yellowish dome-shaped indolent and scaly papules associated with erythema of varying degrees was well conceived. The lesions are usually distributed over the central and lateral side of the face, infrequently extending to involve the neck. Eyebrows and eyelids may also be affected. Reports highlighting variations in its morphologic expressions have continued to be published.1–11 Accordingly, interest in naming the condition differently has been generated, with suggestions such as disseminated follicular lupus acnitis,1 micropapular tuberculid,6 lupoid rosacea, rosacea-like tuberculid,9 acne agminata,11 and more recently facial idiopathic granulomas with regressive evolution5 being discussed in the literature. The fascinating nature of the disease persuaded us to take up the challenge to identify the condition on the basis of detailed histopathology supplemented by its varied morphologic options and develop a viable consensus that may ultimately help establish it as an exclusive entity with an explicit spectrum.

From the Dermato-Venereology (Skin/VD) Centre, Sehgal Nursing Home, Panchwati, Azadpur, Delhi, India;1 Department of DermatoVenereology, Skin Institute and School of Dermatology, Greater Kailash, Delhi, India;2 and the Department of Pathology, Vallavbhai Patel Chest Institute, University of Delhi, Delhi, India3 Address for correspondence: Virendra N. Sehgal, MD, Dermato-Venereology (Skin-VD Centre), Sehgal Nursing Home, A/6 Panchwati, Delhi 110 033, India E-mail: [email protected] www.lejacq.com ID: 3510

Materials and Methods All 16 patients attending our outpatient clinics over a span of 9 years (1995–2003) who were diagnosed with LMDF provided material for the study. The clinical criteria were, namely, multiple red-brown to yellowish, discrete, tiny, dome-shaped papular lesions on the face including the eyelids, lips, varying telangiectasia, and diascopy revealing apple jelly 151

SKINmed: Dermatology for the ClinicianTM (ISSN 1540-9740) is published bimonthly (Jan., March, May, July, Sept., Nov.) by Le Jacq Ltd., Three Parklands Drive, Darien, CT 06820-3652. Copyright ©2005 by Le Jacq Ltd. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopy, recording, or any information storage and retrieval system, without permission in writing from the publishers. The opinions and ideas expressed in this publication are those of the authors and do not necessarily reflect those of the Editors or Publisher. For copies in excess of 25 or for commercial purposes, please contact Sarah Howell at [email protected] or 203.656.1711 x106.

Lupus Miliaris Disseminatus Faciei Part I

Table I. Lupus Miliaris Disseminatus Faciei: Details of Histopathology PILOSEBACEOUS GRANULOMA LOCATION UNIT

PIGMENT INCONTINENCE

PATHOLOGIC STAGE Fully (well) developed (second stage)

HYALINIZED COLLAGEN

GRANULOMA CONTENTS Histiocytes, lymphocytes, neutrophils (neutrophilic abscess)

CASEATION NECROSIS

GRANULOMA HUGGING EPIDERMIS …

CASE NO. EPIDERMAL CHANGES Upper dermis extending to deep dermis





Present*





Present

Present





Early (developing)‡

Fully (well) developed (3rd stage) Fully developed (first stage)

Late

Fully (well) developed (first stage) Fully developed (first stage)

Late**

Fully (well) developed (first stage)

Late

Fully (well) developed (first stage)

Conspicuous Early (developing)

Fully (well) developed (second stage)

Fully (well) developed (third stage)*

Present

Dilated

Naked granuloma (a few lymphocytes) Granuloma, lymphocytes, a few neutrophils Lymphocytes, a few histiocytes

Histiocytes, lymphocytes, (neutrophilic abscesses) Histiocytes, lymphocytes, a few epithelioid/ plasma cells Histiocytes, lymphocytes, a few neutrophils A few histiocytes, lymphocytes Histiocytes, lymphocytes, a few neutrophils A few histiocytes, lymphocytes (without a granuloma) Histiocytes, lymphocytes, neutrophils Histiocytes, lymphocytes, a few neutrophils

Histiocytes, lymphocytes, occasional neutrophils



Hyperkeratosis, follicular plugging

Yes



1

Upper dermis extending to deep dermis

Present



Present

Hyperkeratosis, follicular plugging, and ulcer …

Present

2

Upper/middle dermis

Present

Dilated

...



Hyperkeratosis, follicular plugging Upper dermis



3

Dilated

...

Hyperkeratosis, follicular plugging Yes

...

4 Upper dermis

Present

Dilated

Present

Hyperkeratosis, follicular plugging





5 …



Dilated

Conspicuous

Hyperkeratosis



6

Present

Yes

...

Upper dermis

...

Dilated

...

Hyperkeratosis, follicular plugging

Conspicuous

7

...

...

Present

...

Present

Present





Yes

Yes

...

...



Present

Lichen planus like, early (developing)

Hyperkeratosis, follicular plugging



Conspicuous

Conspicuous

Fully (well) developed (first stage)†

8







Yes

Histiocytes, lymphocytes



Present

Upper dermis



A few histiocytes, lymphocytes



...



Histiocytes, a few lymphocytes (sarcoidal)

Hyperkeratosis



9

Hyperkeratosis, 10 Dilated Upper dermis follicular plugging Hyperkeratosis, 11 Dilated … follicular plugging Hyperkeratosis, 12 … … follicular plugging, hypergranulosis Upper extending to 13 Hyperkeratosis Dilated lower dermis (perifollicular) Entire upper and some 14 Hyperkeratosis … lower dermis Upper extending to Hyperkeratosis, 15 Dilated lower dermis follicular plugging Hyperkeratosis, 16 Dilated Upper dermis follicular plugging *See Figure 5; **see Figure 6; †see Figure 4; ‡see Figure 3

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Lupus Miliaris Disseminatus Faciei Part I

nodules1,2,7 (Figures 1 and 2). Spontaneous regression leaving pock-like scars, in addition to precise initial description punctuated by variations described from time to time, was also taken into account. Each patient underwent skin biopsy of a representative lesion. The biopsy was taken with a 4-mm punch. The biopsy specimens were processed to prepare serial sections. The sections were stained with hematoxylin-eosin stain. Each section was thoroughly examined to define the inflammatory undertones, both in the dermis and the subsequent effect on the epidermis, with emphasis on the former. Several parameters around which the details of histopathology were formed are depicted in Table I. Furthermore, the details provided the basis for delineating histopathology into early (where lymphocytes and a few histiocytes around the blood vessels and adnexa are seen), fully developed (with either sarcoidal granuloma or sarcoidal granuloma with an abscess or sarcoidal granuloma around area of caseation necrosis), or late (characterized by the presence of scattered lymphocytes, histiocytes, and neutrophils in the extensive perifollicular fibrosis).4 In addition, day-to-day investigations were undertaken wherever indicated.

Figure 1. Lupus miliaris disseminatus faciei: erythematous papules and telangiectasia

Figure 2. Lupus miliaris disseminatus faciei: erythematous papules and telangiectasia

Results In all, 16 patients with LMDF were seen among a total of approximately 297,000, giving it a prevalence of 0.0053%. There were 10 men and six women among LMDF patients. Their ages ranged from 21 to 72 years. No efforts to classify/delineate the clinical parameters were made because of the morphologic variations and their explicit overlap. The histologic features were fairly interesting and reflected changes both in the dermis and the epidermis. Moderate to marked hyperkeratosis alone was seen in four of the 16 patients whereas a combination with

follicular plugging was seen in 12 of the 16. Corresponding epidermal changes in the stratum malpighii and basal cells were also seen. Pilosebaceous units were found to be dilated in 10 patients. Presence of inflammatory cells

Table II. Lupus Miliaris Disseminatus Faciei: Criteria for Histologic Stages

EARLY (DEVELOPING) Infiltrate comprising lymphocytes, histiocytes, and a few neutrophils Located in upper to midlower dermis

FIRST STAGE Infiltration containing histiocytes, a few lymphocytes, and neutrophils Sarcoidal (epithelioid) granuloma

INTERMEDIATE FULLY (WELL) DEVELOPED SECOND STAGE Infiltrate comprising histiocytes, a few lymphocytes, and neutrophils

THIRD STAGE

LATE

Infiltrate comprising histiocytes, a few lymphocytes, and sarcoidal (epithelioid) granuloma

Infiltrate comprising a few histiocytes, lymphocytes, and conspicuous hyalinized collagen

Sarcoidal (epithelioid) Caseation necrosis granuloma with neutrophilic abscess

Upper extending to the lower dermis

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Lupus Miliaris Disseminatus Faciei Part I

Figure 3. Lupus miliaris disseminatus faciei: depicting infiltrate comprising lymphocytes, histiocyte, and a few neutrophils (hematoxylin-eosin stain ×50) (early lesion)

Figure 4. Panel A) lupus miliaris disseminatus faciei: low-power view of a dilated hair follicle with follicular plugging, surrounded by large sarcoidal (naked) granulomas and fibrosis (hematoxylin-eosin stain ×50) (fully developed lesion). Panel B) Lupus miliaris disseminatus faciei: large area of sarcoidal (naked) granulomas surrounded by fibrosis (hematoxylin-eosin stain ×50) (fully developed lesion, later half)

A

comprising lymphocytes, histiocytes, and neutrophils was conspicuously a common denominator. They were either found in the form of compact or loose granuloma or were scattered throughout the dermis, extending from upper to the lower dermis. In six patients, the granulomas were approximating (hugging) the epidermis. The cellular contents of the granulomas were remarkably variable, and in two patients neutrophilic abscess(es) were conspicuous; however, caseation necrosis was recorded in only two patients. Hyalinized collagen (fibrosis) was yet another feature in eight of the 16 patients and it was invariably studded with lymphocytes and histiocytes. Pigment incontinence was another feature identified in 10 cases. In view of the preceding histologic changes, the patients were further defined into various stages: early changes were seen in three patients while 10 had fully developed LMDF and three patients had changes corresponding to late lesions (Table I).

Discussion

B

Notwithstanding its well conceived clinical features, LMDF has always evoked discussion centered around its nomenclature. It is, therefore, essential to define the details of its morphologic features in each case because they may overlap with several other clinical conditions. The differentiation may, at times, be a bottleneck and frustrating not only for the patient, but also for the attending physician. This is especially true because it primarily affects the face, resulting in perceptible disfigurement. Thus it is imperative to surmount the diagnostic dilemma by excluding simulating clinical conditions such as small-nodular sarcoidosis,12,13 granulomatous rosacea,14,15 lupoid/granulomatous periorificial dermatitis, steroid dermatitisresembling rosacea,16 perioral dermatitis,17,18 acne vulgaris, polymorphous light eruption, seborrheic dermatitis, eruptive forms of syringomas, and multiple trichoepithelioma. Each of these conditions has distinct clinical features, the details of which will be discussed in Part II of this article, to facilitate diagnosis at a glance. Despite the consideration of the preceding features, the diagnostic dilemma may still continue to haunt clinicians and deprive patients of precise treatment. At this point in time, intervention is absolute and can be achieved through one-time skin

154

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Lupus Miliaris Disseminatus Faciei Part I

biopsy of a representative lesion to clinch/ confirm/establish a viable diagnostic parameter for LMDF. Hematoxylin-eosin stained serial sections of the biopsy are the mainstay, and these were thoroughly exploited in our study to define various characters/diversification. It was beneficial to ultimately evolve these characters into early (developing), fully (well) developed, and late lesions.3 The early lesions were seen in three cases only, whereas fully developed lesions were seen in 10 cases and late lesions were seen in the other three cases, findings similar to those made by other researchers.2,3,7,14 The aforementioned findings are interesting and warrant perspective study because only a few worthwhile studies are thus far available.2,3,7,14,19,20 It is meaningful to clearly delineate the histologic features that may prove useful for their ability to be replicated in future studies. The early (developing) lesions are characterized by cellular infiltrate comprising lymphocytes, a few histiocytes, and occasional neutrophils disposed around the blood vessels and/or appendages,20 whereas fully (well) developed lesions have a perifollicular epithelioid cell granuloma invading the hair follicle wall of one or more hairs. Fully (well) developed lesions may further be subdivided into first, second, and third stages.3 The first stage is characterized by sarcoidal (epithelioid) granuloma only, whereas the second stage also has neutrophilic abscesses, and the third stage is recognized by the presence of caseation necrosis in sarcoidal (epithelioid) granuloma (Table II). It is significant to conclude that histopathology should form one of the major devices in the diagnosis of this captivating entity. The manifest spectrum of LMDF is apparent from our study and is comprised of early (developing), fully (well) developed, and late lesions.

A

Figure 5. Panel A) Lupus

miliaris disseminatus faciei: histopathologic section of skin showing edge of a large neutrophilic abscess in upper dermis lined by epithelioid cell granulomas and giant cells (hematoxylin-eosin stain ×50) (fully developed lesion). Panel B) Lupus miliaris disseminatus faciei: high-power view of a granuloma with central fibrillary necrosis and interspersed neutrophils (hematoxylineosin stain ×100) (fully developed lesion)

B

Figure 6. Lupus miliaris disseminatus faciei: section showing peri-appendageal inflammation and fibrosis (hematoxylin-eosin stain ×100) (late stage)

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Lupus Miliaris Disseminatus Faciei Part I

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