Article:INFLAMMATORY DISORDERS OF THE SKIN AND SUBCUTIS

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Hi Colleen and Group,Couldn't a competant pathologist could differentiate between all these inflammatory disorders from a biopsy? EN, Lupus and PG are towards the bottom of the page.*********INFLAMMATORY DISORDERS OF THE SKIN AND SUBCUTIS:A PRACTICAL AND ANALYTICAL APPROACH

Course Directors:

M. Magro, M.D.

Ohio State University

Columbus, Ohio

A.Neil Crowson, M.D.

Regional Medical Center and

University of Oklahoma

Tulsa, Oklahoma

C. Mihm Jr., M.D.

Massachusetts General Hospital

Harvard Medical School

Boston, Massachusetts

INTRODUCTION

The topic of inflammatory skin disease is a challenging and diverse one. We will attempt to review the

fundamentals of inflammatory skin diseases. We will consider reactions of the epidermis, dermal based

infiltrates and panniculitis.

Classification of Inflammatory Disorders of the Skin and Subcutis

Clinical histories are printed below.

Click on the case numbers for text and references of each case.

Click on the slide images for a larger view of each slide.

SPONGIOTIC AND ECZEMATOUS DERMATITIS

The topic of spongiotic and eczematous dermatitis will be illustrated by 2 cases: allergic contact

dermatitis and pityriasis rosea. A discussion aregarding other forms of spongiotic dermatitis will

follow.

Case 1 - Allergic Contact Dermatitis

A forty-eight year-old woman purchased a new "anti-wrinkle" eye cream. Within forty-eight hours of

applying the cream to her eyelids, she developed redness and swelling of the skin of the eyelids. A skin

biopsy was performed.

Allergic Contact Dermatitis

Allergic Contact Dermatitis

Case 2 - Pityriasis Rosea

A fifteen year-old girl developed a symmetrical macular salmon-colored eruption of papules along the

cleavage lines of the trunk and proximal extremities. Clinical examination showed several of these

lesions to have a cigarette paper-like scale. A skin biopsy was performed.

Pityriasis Rosea

Pityriasis Rosea

PSORIASIFORM DERMATITIS

The topic of psoriasiform dermatitis will be illustrated by 2 cases: psoriasis and pityriasis rubra

pilaris. The definition of psoriasiform dermatitis is one of epidermal hyperplasia characterized by

excessive elongation and expansion of rete ridges. The pattern of rete ridge elongation may be uniform

(i.e. regular psoriasiform hyperplasia) or variable (i.e irregular psoriasiform hyperplasia). The causes

include hyperproliferative states of the epidermis such as psoriasis and pityriasis rubra pilaris,

chronic reactive eczematous reactions, and cutaneous T cell dyscrasia.

Case 3 - Psoriasis

A thirty-eight year-old laboratory technologist developed asymptomatic erythematous, symmetrical plaques

on the knees and elbows. Her family history was positive for a similar condition in her mother.

Clinical examination showed rounded plaques with a silvery scale which, when lifted off, were associated

with bleeding. A skin biopsy was performed.

Psoriasis

Psoriasis

Case 4 - Pityriasis Rubra Pilaris

A sixty-five year-old man developed diffuse scaly erythema of the face, the anterior chest and the upper

back. This was associated with a scaly, orange-hued discoloration of the palms and soles. Treatment

with topical steroids was ineffectual. The rash progressed to cover most of his body, but with islands

of spared, normal skin. A skin biopsy was performed.

Pityriasis Rubra Pilaris

Pityriasis Rubra Pilaris

INTERFACE DERMATITIS

Cell-poor vaculopathic interface dermatitis

Cell-poor vaculopathic interface dermatitis is defined by basilar keratinocyte and subepithelial

vacuolopathy unaccompanied by a significant inflammatory cell infiltrate. Usually, there is some

lymphocyte tagging along the dermal-epidermal junction pointing to the immunopathogenetic basis, namely,

cellular cytotoxicity. This may be either in the context of a type 4 immune reaction or of antibody

dependent cellular cytotoxicity.

The differential diagnoses of a cell-poor interface dermatitis include:

erythema multiforme

graft-versus-host disease

a morbiliform viral exanthum

a morbiliform drug reaction

collagen vascular disease, specifically systemic lupus erythematosus, dermatomyositis, and mixed connective tissue disease

The concept of cell poor interface dermatitis will be illustrated by a case of Erythema Multiforme.

Case 5 - Erythema Multiforme

A twenty year-old man developed annular targetoid erythematous lesions on palms and soles accompanied by

oral blisters. The lesions appeared shortly after a course of Ampicillin for the treatment of otitis

media. A skin biopsy was performed.

Erythema Multiforme

Case 6 - Interface Dermatitis Of Collagen Vascular Disease - Systemic Lupus Erythematosus

A thirty-five year-old woman developed an erythematous malar rash and arthralgias involving the joints of

the hands and the knees. Serological investigations showed a positive anti-nuclear antibody with a titre

of 1:2000. A complete blood count showed leukopenia. A skin biopsy was performed.

Interface Dermatitis Of Collagen Vascular Disease - Systemic Lupus Erythematosus

Case 7 - Interface Dermatitis Of Collagen Vascular Disease - Dermatomyositis

A thirty-five year-old woman had difficulty lifting her baby out of the child's crib and also found it

hard to comb her hair. Clinical examination showed a dusty, violaceus discoloration of her eyelids. A

CT-scan showed bilateral ovarian masses which proved to represent ovarian carcinoma. A skin biopsy was

performed.

Interface Dermatitis Of Collagen Vascular Disease - Dermatomyositis

LYMPHOCYTIC DERMAL INFILTRATES

The next topic of consideration will be one of dermal perivascular lymphoid infiltrates. Among the

causes are lupus erythematosus, polymorphous light eruption, Jessner's lymphocytic infiltrate of skin,

erythema annulare centrifugum, Lyme disease, delayed dermal hypersensitivity reactions and

pseudolymphoma. We will illustrate this concept by presenting 2 of the prototypic causes of dermal

lymphocytic infiltration: polymorphous light eruption and Jessner's lymphocytic infiltrate of skin.

Case 8 - Polymorphous Light Eruption

A nineteen year-old woman experienced recurrent erythematous papules on sun-exposed skin every summer.

These tended to improve in the fall with diminishing sun exposure. A skin biopsy was performed.

Polymorphous Light Eruption

Polymorphous Light Eruption

Case 9 - Jessner's Lymphcytic Infiltrate

A forty year-old woman developed papules and reddish plaques involving the neck and upper back.

Connective tissue disease serology was negative. The lesions resolved spontaneously following a skin

biopsy.

Jessner\'s Lymphcytic Infiltrate

Jessner\'s Lymphcytic Infiltrate

PERIVASCULAR LYMPHOID INFILTRATES ACCOMPANIED BY DERMAL SCLEROSIS

Morphea, the most common of the sclerodermoid tissue reactions, can show prominent perivascular dermal

lymphoid hyperplasia and therefore we felt it was only appropriate to discuss this important entity.

Case 10 - Morphea

A twenty-two year-old woman developed an indurated plaque on the trunk. The plaque had an ivory-colored

center and a lilac-colored border. A skin biopsy was performed.

Morphea

Morphea

HISTIOCYTIC DERMAL INFILTRATES WITH MINIMAL EPIDERMAL ALTERATION

A common reaction pattern in the dermis is one characterized by infiltration of the Interstitum by

histiocytes with variable necrobiotic alteration of the collagen. The two classic cutaneous disorders

associated with palisading granulomatous infiltrates are GRANULOMA ANNULARE and NECROBIOSIS LIPOIDICA.

The concept of palisading granulomatous dermatitis will be illustrated by 2 cases.

Case 11 - Necrobiosis Lipoidica

A fifty-six year-old woman developed yellow-brown atrophic, depressed

plaques involving both shins. A skin biopsy was performed.

Necrobiosis Lipoidica

Necrobiosis Lipoidica

Case 12 - Granuloma Annulare

A forty-six year-old woman developed papules on her elbows several weeks following a visit with her

nephew who had a fiery , erythematous cheek and malaise. His pediatrician subsequently diagnosed

parvovirus B19 infection ("fifth disease"). In the interim, one of the woman's papules was biopsied.

Granuloma Annulare

Granuloma Annulare

Granuloma Annulare

THE NEUTROPHILIC DERMATOSES

The 2 main dermatoses characterized by prominent interstitial neutrophilia are Sweet's syndrome and

pyoderma gangrenosum. Other conditions which fall into the interstitial neutrophilic dermatosis category

include rheumatoid neutrophilic dermatosis, urticarial vasculitis, and erythema elevatum diutinum.

Case 13 - Pyoderma Gangrenosum

A thirty-six year-old man with intermittent diarrhea, developed violaceus, ulcerating plaques on the

lower extremities. One of these was biopsied.

Pyoderma Gangrenosum

Pyoderma Gangrenosum

Case 14 - Sweet's Syndrome

A forty-eight year-old woman developed reddish-purple plaques, several of which were studded with

pustules, involving her face and upper arms. These followed an upper respiratory tract infection for

which she did not see a doctor and for which she was not treated. She had an intermittent fever, and a

complete blood count showed neutrophilia. Her ESR was elevated. A skin biopsy was performed.

Sweet\'s Syndrome

THE SUBCUTIS

Septal Panniculitis

Case 15 - Erythema Nodosum

A fifteen year-old girl developed plaques over the pre-tibial surfaces following an episode of

pharyngitis. Throat swabs grew Streptococcus sp. A skin biopsy was performed.

Erythema Nodosum

Erythema Nodosum

Lobular Panniculitis

Case 16 - Erythema Induratum

A twenty-eight year-old man had a past history of tuberculosis in the Philippines. He developed

ulcerating plaques on the posterior calves. A skin biopsy was performed.

Erythema Induratum

Erythema Induratum

Connective Tissue Disease Panniculitis

Case 17 - Lupus Profundus

A twenty-four year-old woman with a long-standing history of systemic lupus erythematosus developed

plaques involving her upper arms and thighs. A skin biopsy was performed.

Lupus Profundus

Lupus Profundus

Lupus Profundus