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Michel R. Ibrahim the Psoriasiform Reaction Pattern Michel R. Ibrahim The Psoriasiform Reaction Pattern Michel R. Ibrahim Board-Certified Dermatologist & Dermatopathologist EBDV, Dip of Dermatopath. Frankfort-Germany Visiting Scientist Sanford-Burnham-Prebys (SBP) Medical Discovery Institute Sanford Consortium for Regenerative Medicine San-Diego, USA Associate Lecturer of Dermatology, STDs & Andrology. Faculty of medicine- Minya University- Egypt. 1 Michel R. Ibrahim The Psoriasiform Reaction Pattern PSORIASIS Chronic Plaque Disease: Symmetric distribution of sharply defined erythematous scaly plaques. The scalp, elbows, knees and presacrum. The hands and feet. The genitalia (up to 30% of patients). 2 Michel R. Ibrahim The Psoriasiform Reaction Pattern Histopathology: The histologic picture of psoriasis vulgaris varies with the stage of the lesion and usually is diagnostic ONLY in early scaling papules and near the margin of advancing plaques. The earliest pinhead-sized macules or smooth-surfaced papules → preponderance of dermal changes. At first: 1. Capillary dilatation. 2. Edema in the papillary dermis. 3. Lymphocytic infiltrate surrounding the capillaries. 4 4. The lymphocytes extend into the lower portion of the epidermis → slight spongiosis. Then: early scaling papule → Focal changes occur in the upper portion of the epidermis. 1. The granular cells → vacuolated and disappear. 2. Mounds of parakeratosis. 3. The phenomenon of “Squirting papillae”: Neutrophils → discharged intermittently from the papillary capillaries → 4 attracted to the parakeratotic zones. 4. These mounds of parakeratosis + neutrophils → the earliest manifestation of Munro microabscesses. At this stage → early scaling papule→ histologic diagnosis of psoriasis can be made. Small spongiform pustules of Kogoj → marked exocytosis of 4 neutrophils → aggregate in the uppermost portion of the spinous layer. Lymphocytes remain confined to the lower epidermis, which, as NB more and more mitoses occur, becomes increasingly hyperplastic. The epidermal changes, at first → focal, but later on → confluent, leading clinically to plaques. 3 Michel R. Ibrahim The Psoriasiform Reaction Pattern In the fully developed lesions of psoriasis, as best seen at the margin of enlarging plaques: 1. Acanthosis with regular elongation of the RR with thickening in their lower portion “Clubbing”. 4 2. Elongation and edema of the dermal papillae. 3. Thinning of the suprapapillary epidermis ± small spongiform pustules. 4. Pallor of the upper layers of the epidermis “intercellular edema + × hypogranulosis”. 5. Confluent parakeratosis → plate-like scale. 6. Diminished to absent granular layer. 2 7. Munro microabscesses. 8. Dilated and tortuous capillaries. ONLY the spongiform pustules of Kogoj & Munro microabscesses are truly diagnostic of Ps → in their absence diagnosis can rarely be made with certainty on a histologic basis. At first: Then: Pinhead-sized macules Early scaling papule Smooth-surfaced papules Dermal changes. Focal changes in the upper portion of the epidermis. Fully developed lesions of psoriasis. At the margin of enlarging plaques 4 Michel R. Ibrahim The Psoriasiform Reaction Pattern 1. Small spongiform pustules of Kogoj. 2. Mitoses. 4 NB 3. Auspitz Sign. 4. Munro microabscesses. Mitoses NOT limited to the BCL as in normal skin → but also above the BCL. This, together with a considerable lengthening of the BCL due to elongation of the RR → ↑↑ mitoses. 27 times the number of mitoses in uninvolved skin. Small spongiform pustules of Kogoj → marked exocytosis of neutrophils → aggregate in the uppermost portion of the spinous layer. Munro microabscesses: Site: Parakeratotic areas of the cornified layer. Consist of accumulations of neutrophils and Pyknotic nuclei of neutrophils. Easily found in early lesions but few in number or absent in long-standing lesions. Auspitz Sign: Gentle scraping of the skin → Bleeding points "correspond to the tips of dermal papillae". Parakeratosis. Intracellular edema of keratinocytes in the suprapapillary epidermis. Thinning of the suprapapillary plates. Dilatation of the capillaries in the upper portion of the papillae. 5 Michel R. Ibrahim The Psoriasiform Reaction Pattern Guttate or Eruptive Psoriasis: Histologic features of an early or active lesion of Ps → more inflammatory infiltrate and less acanthosis than in chronic plaque Ps. Because of its acute onset → normal basket-weave orthokeratotic cornified layer overlying the mounds of parakeratosis with neutrophils. Erythrodermic Psoriasis: In some instances, → shows enough of the characteristics of Ps to allow this diagnosis. Frequently → indistinguishable from chronic eczematous dermatitis. 6 Michel R. Ibrahim The Psoriasiform Reaction Pattern GENERALIZED PUSTULAR PSORIASIS As Ps vulgaris + 4 MACRO-pustule: The spongiform pustule forms through the migration of neutrophils from the papillary dermal capillaries to the upper layer of epidermis → aggregation within the interstices of a sponge-like network formed by degenerated and thinned epidermal cells → As ↑ in size → complete cytolysis of the epidermal cells in the center of the pustule → large single cavity forms. At the periphery of the pustule → the network of thinned epidermal cells persists for a much longer time. MACRO-abscess of Munro: The neutrophils of the spongiform pustule → move up into the cornified layer → become pyknotic and assume the appearance of a large Munro abscess. In the healing stage: As Ps vulgaris. The oral lesions → the same spongiform pustule formation as those seen on the skin. Differential Diagnosis: Geographic tongue. Geographic tongue represents an abortive form of pustular Ps. Acute Generalized Exanthematous Pustulosis (AGEP): Site: Widespread. Lesion: Sterile, miliary pustules on an erythematous background. Fever and a peripheral blood leukocytosis. Occurs within hours or days of the ingestion of certain drugs. -lactam, cephalosporin, and macrolide. Spontaneous resolution with superficial desquamation over 1–2 weeks. Histopathology: Subcorneal pustule. Upper dermis → mixed inflammatory cell infiltrate with eosinophils. DIF: -ve. 7 Michel R. Ibrahim The Psoriasiform Reaction Pattern LOCALIZED PUSTULAR PSORIASIS 1. Localized pustular psoriasis, “psoriasis with pustules”. As GPP. 2. Localized annular pustular psoriasis. 3. In localized acrodermatitis continua of Hallopeau: The nail bed is mainly affected → marked epithelial hyperplasia with variable numbers of spongiform pustules, and orthokeratosis with mounds of parakeratosis with neutrophils. 4 4. In pustulosis palmaris et plantaris: Fully developed large intraepidermal unilocular pustule: Elevated only slightly above the surface but presses onto the underlying dermis. Many neutrophils → within the cavity of the pustule. The epidermis surrounding the pustule → slight acanthosis. An inflammatory infiltrate → beneath the pustule. Typical, small, spongiform pustules in the epidermal wall of the pustule: Most commonly at the junction of the lateral walls and the overlying epidermis. These spongiform pustules are identical to those seen in the walls of the pustules of GPP. Very early lesions → exocytosis of lymphocytes in the lower epidermis overlying the tips of dermal papillae→ spongiosis → small intraepidermal vesicle containing mostly lymphocytes → then → massive exocytosis of neutrophils → histologic picture of spongiform pustules. Acute form → “pustular bacterid” → leukocytoclastic vasculitis. 8 Michel R. Ibrahim The Psoriasiform Reaction Pattern Differential Diagnosis of Psoriasis: Two histologic features are of great value in the diagnosis of psoriasis vulgaris: 1. Mounds of parakeratosis with neutrophils at their summits (Munro microabscesses). 2. Spongiform micropustules of Kogoj in the uppermost layers of the spinous 2 layer. Dilatation and tortuosity of capillaries in the papillae may also be of help in the diagnosis. 1. Chronic eczematous dermatitis: Acanthosis with elongation of RR and parakeratosis, can be found also in chronic eczematous dermatitis, such as AD, nummular dermatitis, or ACD → “psoriasiform”. Marked spongiosis. Eosinophils. 2. Lichen simplex chronicus: In contrast to Ps, it shows Prominent granular layer. More irregular acanthosis. Fibrosis of the papillary dermis with vertically oriented collagen bundles “vertically streaked collagen” aligned perpendicularly to the skin surface. 3. Seborrheic dermatitis: May be very difficult to distinguish from Ps vulgaris, especially if overlap occurs. ↑ Spongiosis. Mounds of parakeratosis with neutrophils predominantly at the follicular ostia. Irregular acanthosis. 4. Pityriasis rubra pilaris: 10 9 Michel R. Ibrahim The Psoriasiform Reaction Pattern Spongiform micropustules of Kogoj occur in: 1. Ps. 2. Pustular dermatophytosis. 3. Bacterial impetigo. 4. Pustular drug eruptions. 5. Candidiasis, particularly if pustules are clinically present. PAS and Gram stains are useful to identify the infectious microorganisms. Collections of neutrophils within the stratum corneum (PTICSS): 1. Psoriasis. 2. Tinea. 3. Impetigo. 4. Candida. 5. Seborrheic dermatitis. 6. Syphilis. 10 Michel R. Ibrahim The Psoriasiform Reaction Pattern REITER'S DISEASE 3 Conjunctivitis Urethritis Arthritis Can’t see Can’t pee Can’t bend my knee 3 keratoderma blennorrhagicum Balanitis circinata Subungual area 11 Michel R. Ibrahim The Psoriasiform Reaction Pattern Histopathology: Early pustular lesions on Later Old the palms or soles Psoriasiform The parakeratotic cornified layer: Psoriasiform with + thickens, which correlates with
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