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Dermatology > PAPULOSQUAMOUS DISEASES
Psoriasis, Guttate
Article Last Updated: Feb 1, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 11
Author: Elma D Baron, MD, Assistant Professor of Dermatology, Case Western Reserve University, University Hospitals of Cleveland; Director of Skin Study Center, University Hospitals Research Institute; Acting Chief of Dermatology, Veterans Affairs Medical Center, Cleveland
Elma D Baron is a member of the following medical societies: American Academy of Dermatology, American Society for Photobiology, Photomedicine Society, and Society for Investigative Dermatology
Coauthor(s):
Charles R Taylor, MD, Assistant Professor of Dermatology, Harvard Medical School; Director of Phototherapy Unit, Department of Dermatology, Massachusetts General Hospital
Editors: Mark G Lebwohl, MD, Chairman, Department of Dermatology, Mount Sinai School of Medicine; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Christen M Mowad, MD, Assistant Professor, Department of Dermatology, Geisinger Medical Center; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
guttate psoriasis, perianal streptococcal infection, Streptococcus pyogenes, S pyogenes, upper respiratory infection
Background
Guttate psoriasis refers to a distinctive, acute clinical presentation of an eruption characterized by small, droplike, 1-10 mm in diameter, salmon-pink papules, usually with a fine scale (see Images 1-3). The word guttate is derived from the Latin word gutta, meaning drop. This variant primarily occurs on the trunk and the proximal extremities, but it may have a generalized distribution. More common in individuals younger than 30 years, a history of upper respiratory infection secondary to group A beta-hemolytic streptococci (eg, Streptococcus pyogenes) often precedes the eruption by 2-3 weeks. Although recurrent episodes may occur, especially those due to pharyngeal carriage of streptococci, isolated bouts are known to occur. The sudden appearance of the papular lesions may be either the first manifestation of psoriasis in a previously unaffected individual or an acute exacerbation of long-standing plaque psoriasis. On the other hand, guttate psoriasis may be chronic and unrelated to a streptococcal infection.
Pathophysiology
The exact pathophysiologic mechanism is undetermined. The disease is believed to result from an immune reaction triggered by a previous streptococcal infection in a genetically susceptible host.
Recent studies indicate the importance of chromosome 6 in determining the resultant psoriatic phenotype. HLA-Cw*0602–positive patients are more prone to develop the guttate form. Interactions of the HLA-C with killer immunoglobulin-like receptors (KIR) on natural killer cells or natural killer T-cells can be deregulated by streptococcal infection. T lymphocytes and cytokines are believed to cause the characteristic inflammatory changes appreciated on histopathologic examination of lesional skin samples. An autoimmune phenomenon has also been postulated because some streptococcal products and components have been found to cross-react with normal human epidermis. Electron microscopic studies have shown that mast cell degranulation is an early and constant feature in the evolution of guttate psoriatic lesions.
Frequency
United States
The guttate form of psoriasis is relatively uncommon, occurring in less than 2% of the psoriatic population.
International
Surveys on the occurrence of the guttate form of eruption among patients with psoriasis range widely from 1.6-44%.
Mortality/Morbidity
Guttate psoriasis is a nonfatal eruption that either can run a limited course over several weeks to a few months or develop into the chronic plaque-type of psoriasis. Scarring is not a problem. Previously affected areas may show postinflammatory hypopigmentation or postinflammatory hyperpigmentation.
Race
Guttate psoriasis affects people of all races.
Sex
Both sexes are affected equally.
Age
Guttate psoriasis is more common in individuals younger than 30 years, and it is generally believed to be the type of psoriasis most likely to affect children and adolescents.
History
- In most cases, a history of an antecedent streptococcal infection, usually of the upper respiratory tract, such as pharyngitis or tonsillitis, 2-3 weeks prior to the eruption can be elicited.
- Perianal streptococcal infections or viral infections, such as varicella, rubella, and roseola, have also been postulated as provocative factors, especially in children.
- A positive family history of psoriasis may be present.
- The onset of the skin lesions is often acute, with multiple papules erupting on the trunk and the proximal extremities. The lesions are often accompanied by slight pruritus.
- Drug therapy, including biologic agents, may sometimes precipitate a guttate-type flare.
Physical
- Examination of the skin reveals characteristic lesions consisting of multiple, discrete, 1-10 mm in diameter, droplike papules with a salmon-pink hue. A fine scale, which is usually absent in early-stage lesions, may be appreciated on the more established ones (see Image 3).
- Beginning on the trunk and the proximal extremities, the lesions may sometimes spread to involve the face, the ears, and the scalp.
- The palms and the soles are rarely affected.
- Nail changes in the form of pits, ridges, and the oil-drop sign, which are characteristic of chronic psoriasis, may be absent.
- Additional findings may include pharyngeal or perianal erythema in cases associated with acute streptococcal infections.
Causes
- Genetic predisposition: As in other types of psoriasis, genetic predisposition seems to play an important role in the development of an acute guttate psoriasis flare. Compared with control populations, a significant excess of HLA-BW17 has been found in patients with guttate psoriasis. Others have found an increase in HLA-B13 positivity. Moreover, the inability to produce normal amounts of antibody to streptolysin-O by HLA-B13–positive individuals might explain the high prevalence in guttate psoriasis. Interestingly, an increased prevalence of HLA-CW6 has also been found. Thus far, psoriasis is the only disease associated with HLA-C gene expression. Recently, proteomic studies have been able to demonstrate that guttate psoriasis and chronic plaque psoriasis are phenotypically distinguishable in their protein expression patterns.
- Streptococcal infection
- The association of guttate psoriasis with streptococcal infection has been recognized for more than 50 years. As many as 80% of patients with guttate psoriasis have clinical or laboratory evidence of streptococcal infection, usually in the form of tonsillopharyngitis. Aside from group A streptococci, groups C and G streptococci have also been related to guttate psoriasis.
- The ability of the streptococcal organism to trigger an eruption is not serotype specific. The serotypes present in patients with guttate psoriasis are similar to those seen in the general population. A number of cases of guttate psoriasis in children have also been triggered by streptococcal perianal cellulitis. Presumably, absorption of streptococci by-products produced by the streptococci occurs across the rectal mucosa, as with pharyngeal infections. Unfortunately, although the association is definite, details regarding the exact mechanism by which streptococcal infection influences the actual formation of the psoriatic lesions are still largely theoretical.
- Role of T lymphocytes
- Histologic studies of early-stage psoriatic skin lesions revealed that the activation of T lymphocytes, endothelial cells, and macrophages precedes epidermal proliferation. The increased proliferation of the epidermal layer characteristic of psoriasis might be induced by activated T lymphocytes via the production of cytokines. Indeed, group A streptococcal antigen-specific T lymphocytes, which secrete high levels of gamma interferon, can be consistently isolated from guttate psoriatic skin lesions.
- Consistent with the role of T lymphocytes is the concept of superantigenic stimulation by certain streptococcal components or products. Examples of superantigens produced by group A beta-hemolytic streptococci are streptococcal pyogenic exotoxins (SPE) types A, B, and C; a 22-kd pepsin fragment of M type-5 protein; S pyogenes–derived cytoplasmic membrane-associated protein (CAP); and secretion-type CAP (SCAP).
- In general, unlike a conventional peptide antigen, a superantigen stimulates T cells almost solely through the beta variable (Vß) portion of the T-cell receptor and induces an expansion of both CD4+ and CD8+ T cells. Therefore, an increased representation of Vß2+ T lymphocytes, such as that in both the epidermis and the dermis of guttate psoriatic lesions, compared with that of lymphocytes from the peripheral blood of the same patients and lymphocytes in normal skin strongly suggests that T-cell stimulation by a superantigen is probably involved.
- Role of autoantibodies
- Immunoblotting has demonstrated intense antistreptococcal antibody activity in the sera of patients with guttate psoriasis. Immunoglobulin G (IgG) antibodies against 3 different S pyogenes proteins, namely, a 60-, a 70-, and a 14-kd antigen, have been identified. Indirect immunofluorescence studies of these antibodies showed that they only react with autologous skin in patients with guttate psoriasis and not with normal skin or lesional skin from patients who do not have psoriasis.
- Autoantibodies in psoriatic sera may recognize certain structures in the transformed keratinocytes of affected psoriatic skin. These autoantibodies cross-react with streptococcal antigens, and it has been demonstrated on immunofluorescent microscopy by using a monoclonal antibody (mAb 111-15504) to group A streptococci, which does not cross-react with antigens in normal human skin. These antigens were associated with class 1M protein and were mostly concentrated in the dermal papillae around the capillaries and inside the cells of the epidermal basal layer.
Cutaneous T-Cell Lymphoma
Lymphomatoid Papulosis
Nummular Dermatitis
Parapsoriasis
Pityriasis Lichenoides
Pityriasis Rosea
Syphilis
Other Problems to be Considered
Psoriasiform drug eruption
Viral exanthem
Lab Studies
- Serology: Levels of antibodies to streptolysin O, hyaluronidase, and deoxyribonuclease B may be elevated in more than one half of patients. Significant elevations of antistreptococcal M6 protein have been documented.
- Cultures: A bacteriologic culture of the throat or the perianal area may be helpful to isolate the organism in selected cases.
- Urinalysis: Urine results are usually negative, although blood and protein have been detected in rare cases associated with a concomitant poststreptococcal acute glomerulonephritis.
Histologic Findings
Because the clinical appearance is so characteristic, biopsies are seldom necessary to confirm the diagnosis. Histopathologic changes may not be diagnostic when samples of early-stage papules are obtained at biopsy. The epidermis shows hyperplasia and small foci of parakeratosis with absence of the granular layer. Dermal changes consisting of capillary dilatation and edema may be more pronounced, and an infiltrate consisting of lymphocytes and macrophages is seen mostly in the upper dermis. A few polymorphonuclear leukocytes may be found at all levels.
In fully developed guttate lesions, vacuolated keratinocytes eventually disappear, leaving areas of agranulosis with overlying parakeratosis. Degenerated polymorphonuclear leukocytes on an otherwise orthokeratotic stratum corneum may be the earliest presentation of Munro microabscesses. The term squirting papillae has been used to describe a phenomenon wherein neutrophils are discharged from the papillary capillaries resulting in collections of neutrophils in association with parakeratotic mounds. In some cases, marked exudation may lead to the formation of the highly diagnostic spongiform pustule of Kogoj, which is seen in psoriasiform variants.
Medical Care
Usually, this type of psoriasis spontaneously disappears in a few weeks without treatment. Simple reassurance and emollients may be sufficient care. As in other conditions, the choice of treatment should be tailored to the individual. For example, applying topical steroids, although effective, could be cumbersome, especially when the eruption is extensive as in most cases of guttate psoriasis.
- Antimicrobials: Because of the clear association between guttate psoriasis and streptococcal infection in most cases, obtaining a throat culture for each bout of pharyngitis in patients with a known history of psoriasis and immediately starting the proper antibiotic treatment depending on the culture results are imperative. See Medication below for specific drugs.
- Phototherapy: The clearance of guttate lesions can be accelerated by judicious exposure to sunlight or by a short course of either broadband UV-B or narrowband UV-B phototherapy. More resistant cases may benefit from oral psoralen plus exposure to ultraviolet A radiation (PUVA). The suit PUVA technique has been used in this setting. Aside from the usual mechanisms by which UV light is believed to exert its beneficial effects in psoriasis, a specific fibrosing response to PUVA via increased mast cell activation has been observed in guttate psoriasis and might underlie the mechanism of action behind UV-induced resolution of the lesions. However, considering the developments in photomedicine over the last several years, particularly regarding the clinical efficacy of narrowband UV-B phototherapy, versus the risk of cutaneous malignancies with PUVA, treatment with narrowband UV-B is favored over treatment with PUVA.
Surgical Care
Although unproven by large controlled clinical trials, tonsillectomy for patients with recurrent or chronic guttate psoriasis associated with poststreptococcal tonsillitis may be helpful.
Many physicians have questioned the usefulness of antibiotics in the management of psoriasis. Nevertheless, some experts have used empiric therapy with the following antimicrobials in streptococcal-related guttate psoriasis. Azithromycin, which is commonly prescribed for community-acquired pneumonia, is likewise an option.
Drug Category: Antimicrobials
Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.
| Drug Name | Erythromycin (E.E.S., E-Mycin, Ery-Tab) |
| Description | DOC for pregnant patients. Has the added advantage of being a good anti-inflammatory agent by inhibiting migration of polymorphonuclear leukocytes. |
| Adult Dose | >1 g/d PO for 7-14 d |
| Pediatric Dose | 30-50 mg/kg/d PO for 7-14 d |
| Contraindications | Documented hypersensitivity; hepatic impairment |
| Interactions | Coadministration may increase toxicity of theophylline, terfenadine, digoxin, carbamazepine, astemizole, and cyclosporine; may potentiate anticoagulant effects of warfarin; coadministration with lovastatin and simvastatin increases risk of rhabdomyolysis |
| Pregnancy | B - Usually safe but benefits must outweigh the risks.
|
| Precautions | Caution in liver disease; estolate formulation may cause cholestatic jaundice; GI adverse effects are common (give doses pc); discontinue use if nausea, vomiting, malaise, abdominal colic, or fever occur |
| Drug Name | Penicillin VK (Veetids, Beepen-VK) |
| Description | Inhibits biosynthesis of cell wall mucopeptide and is effective during active multiplication. Inadequate concentrations may produce only bacteriostatic effects. |
| Adult Dose | >1 g/d PO for 10-14 d |
| Pediatric Dose | 30-50 mg/kg/d PO for 10-14 d |
| Contraindications | Documented hypersensitivity |
| Interactions | Probenecid can increase effects by decreasing clearance; coadministration of tetracyclines can decrease effects |
| Pregnancy | B - Usually safe but benefits must outweigh the risks.
|
| Precautions | Caution in impaired renal function |
| Drug Name | Rifampin (Rifadin, Rimactane) |
| Description | Recommended for resistant cases that may progress to chronic carrier state. Usually given in addition to either erythromycin or penicillin. |
| Adult Dose | 600 mg/d PO for 5 d |
| Pediatric Dose | 10-20 mg/kg PO/IV; not to exceed 600 mg/d |
| Contraindications | Documented hypersensitivity |
| Interactions | Induces microsomal enzymes, which may decrease effects of acetaminophen, oral anticoagulants, barbiturates, benzodiazepines, beta-blockers, chloramphenicol, oral contraceptives, corticosteroids, mexiletine, cyclosporine, digitoxin, disopyramide, estrogens, hydantoins, methadone, clofibrate, quinidine, dapsone, tazobactam, sulfonylureas, theophyllines, tocainide, and digoxin; blood pressure may increase with coadministration of enalapril; coadministration with isoniazid may result in higher rate of hepatotoxicity than with either agent alone (discontinue 1 or both agents if alterations in LFTs occur) |
| Pregnancy | C - Safety for use during pregnancy has not been established.
|
| Precautions | Obtain CBC counts and baseline clinical chemistries prior to and throughout therapy; in liver disease, weigh benefits against risk of further liver damage; interruption of therapy and high-dose intermittent therapy are associated with reversible thrombocytopenia if therapy is discontinued as soon as purpura occurs; if treatment is continued or resumed after appearance of purpura, cerebral hemorrhage or death may occur |
Complications
- Physicians should watch for possible hypersensitivity reactions to the above-mentioned antimicrobials, especially to penicillin. If hypersensitivity is suspected, the drug should be immediately discontinued. Patients who are sensitive to penicillin generally do well on erythromycin. Cephalosporins can also cover streptococci, but some cross-sensitivity with penicillins has been documented.
- Areas of the skin that have been treated with high-potency topical steroids for long periods may show some atrophy, telangiectases, and hypopigmentation. Shifting to a preparation with a lower potency or to another treatment modality should be considered.
- Patients on PUVA may experience a number of adverse effects, such as nausea and vomiting. These effects are sometimes remedied by taking psoralen pills after a meal. The psoralen-induced photosensitivity persists up to 24 hours after administration of the drug. Patients should be adequately informed about the need to wear protective lenses and to avoid sun exposure during this period.
Prognosis
- Although guttate psoriasis often undergoes a short-lived course, it may also represent the initial stage of chronic plaque-type psoriasis.
- The acute guttate form progresses into the chronic plaque form in an estimated 68% of patients.
- In another study of 15 patients, the probability of an individual developing chronic psoriasis within 10 years of a single episode of acute guttate psoriasis was suggested to be about 1 in 3, although further studies with larger numbers of patients are needed to more accurately determine the risk.
- Like other forms of psoriasis, guttate psoriasis has the tendency to improve during the summer and worsen during the winter. Once cleared, many patients who experience acute guttate psoriasis usually have limited or no evidence of psoriasis for prolonged periods.
Patient Education
- Patients should be advised to minimize all forms of skin trauma, such as scratching or vigorous rubbing, which may lead to new psoriatic lesions on previously unaffected areas (Koebner phenomenon).
- The association between streptococcal infections and guttate psoriasis cannot be overemphasized.
- Early detection and treatment of such infections may prevent an acute flare of the skin disease. Samples should be obtained and cultured in patients who are susceptible and have a sore throat.
- Some advocate early antibiotic therapy of any sore throat in individuals who are susceptible.
- For excellent patient education resources, visit eMedicine's Psoriasis Center. Also, see eMedicine's patient education articles Guttate Psoriasis, What Is Psoriasis?, Types of Psoriasis, Understanding Psoriasis Medications, and Nail Psoriasis.
Medical/Legal Pitfalls
- For patients with atypical disease or for those who do not respond to therapy, alternative diagnoses should be considered.
- A careful history should be taken to exclude certain drugs, such as beta-blockers and lithium, which may cause an eruption similar to that of guttate psoriasis.
- Especially in patients with palmar and plantar lesions, serologic analysis should be performed to exclude secondary syphilis. Skin biopsy is probably the single most useful diagnostic test if the clinical diagnosis is not certain.
| Media file 1:
The distinctive, acute clinical presentation of an eruption or guttate psoriasis characterized by small, droplike, 1-10 mm in diameter, salmon-pink papules, usually with a fine scale. Courtesy of Hon Pak, MD. |
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Media type: Photo
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| Media file 3:
Note characteristic lesions consisting of multiple, discrete, droplike papules with a salmon-pink hue. A fine scale, which is usually absent in early-stage lesions, may be appreciated on the more established ones. Courtesy of Hon Pak, MD. |
 | View Full Size Image | |
Media type: Photo
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Psoriasis, Guttate excerpt Article Last Updated: Feb 1, 2007
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