EUH Resident Report: A review of pustular psoriasis

The Bottom Line: Several clinical variants of pustular psoriasis exist: generalized pustular psoriasis (von Zumbusch type), annular pustular psoriasis, impetigo herpetiformis, and two variants of localized pustular psoriasis—(1) pustulosis palmaris et plantaris and (2) acrodermatitis continua of Hallopeau. Treatment of patients with pustular psoriasis depends on the severity of presentation and patient’s underlying risk factors. The literature and data are extremely weak and limited for this type of psoriasis.

Click here for a book chapter on psoriasis, with a section covering pustular psoriasis.

DynaMed Plus provides treatment guidelines recommended by the National Psoriasis Foundation (NPF).

References: 

Gudjonsson, Johann E., and James T. Elder.Chapter 18. Psoriasis.Fitzpatrick’s Dermatology in General Medicine, 8e. Eds. Lowell A. Goldsmith, et al. New York, NY: McGraw-Hill, 2012. n. pag. AccessMedicine. Web. 22 Jan. 2016. <http://accessmedicine.mhmedical.com.proxy.library.emory.edu/content.aspx?bookid=392&Sectionid=41138713&gt;.

DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 116742, Psoriasis; [updated 2015 Dec 21, cited 2016 Jan 22]. Available from http://www.dynamed.com/login.aspx?direct=true&site=DynaMed&id=116742. Registration and login required.

Summary: Pustular psoriasis involves monomorphic sterile pustules on painful inflamed skin. There is localized pustular variant involving soles and palms occurring with or without plaque-type disease (palmoplantar psoriasis). The acute generalized disease also called von Zumbusch variant consists of widespread pustules on erythematous background and is an uncommon severe form of psoriasis associated with fever and toxicity.

Treatment: Treatment should be governed by the extent of involvement and severity of disease. Acitretin, cyclosporine, methotrexate, and infliximab are considered to be first-line therapies for those with generalized pustular psoriasis. Adalimumab, etanercept, and psoralen plus ultraviolet A are second-line modalities in this setting. Pustular psoriasis in children, in pregnant women, and in localized forms alter which agents are first-line modalities as concerns such as teratogenicity need to be factored into the decisionmaking for the individual patient.

 

 

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EUH Resident Report: Sensitivity and specificity of the Nikolsky sign

The Bottom Line: The Nikolsky sign is a moderately sensitive but highly specific tool for the diagnosis of pemphigus.

Reference: Uzun, Soner, and Murat Durdu. “The Specificity and Sensitivity of Nikolskiy Sign in the Diagnosis of Pemphigus.” Journal of the American Academy of Dermatology 54.3 (2006): 411-15

Summary: Presence of the Nikolskiy sign with the modifications of “direct” and “marginal” on 123 consecutive patients with various cutaneous diseases presenting as intact blisters and/or erosions was sought.

A positive Nikolskiy sign was demonstrated in 24 (19.5%) of the 123 patients. Of the positive 24 patients, 18 had pemphigus, 4 had bullous pemphigoid, 1 had linear IgA dermatosis, and 1 had staphylococcal scalded skin syndrome. The sensitivity of “direct” Nikolskiy sign (38%) was less than that of the “marginal” form (69%), but the specificity of “direct” Nikolskiy sign (100%) was higher than that of the “marginal” form (94%) in the diagnosis of pemphigus.

What is the preferred imaging modality for necrotizing fasciitis?

Magnetic resonance imaging (MRI) is the most effective method for documenting the soft tissue lesions and evaluating their distribution.

In practice, several key points deserve emphasis:
• the absence of MRI abnormalities of the intermuscular fasciae virtually rules out necrotizing fasciitis
• the presence of gas (signal-free areas on all sequences) is highly specific but rare
• extensive thickening of the intermuscular fasciae with an appearance suggesting incomplete vascularization supports a diagnosis of necrotizing fasciitis
• the presence of lesions confined to the peripheral fasciae and to small portions of the adjacent intermuscular fasciae is of borderline significance

Malghem, Jacques, et al. “Necrotizing fasciitis: contribution and limitations of diagnostic imaging.” Joint bone spine 80.2 (2013):146-54.

A very important point is that imaging studies play only an ancillary role and must never delay the surgical treatment of deep necrotizing fasciitis, a condition whose outcomes, including patient survival, depend heavily on the promptness of appropriate therapy

jkn 2/9/16

What is the clinical presentation associated with various drugs that incite drug rash with eosinophilia and systemic symptoms (DRESS)?

Bottom line: Typically presents with fever, rash, and solid organ involvement. Commonly associated drugs include aromatic anticonvulsants and sulfonamides.

Walsh SA, et al. Drug reaction with eosinophilia and systemic symptoms (DRESS): a clinical update and review of current thinking. Clin Exp Dermatol. 2011 Jan;36(1):6-11.
Table 2 summarizes diagnostic criteria proposed by a consensus group.

Kano Y.  The variable clinical picture of drug-induced hypersensitivity syndrome/drug rash with eosinophilia and systemic symptoms in relation to the eliciting drug.  Immunol Allergy Clin North Am. 2009 Aug;29(3):481-501
Reviews clinical presentation associated with specific drugs known to elicit DRESS.

Cephalosporins are not generally identified as causative agents in DRESS.  View case reports of DRESS associated with cephalosporins.
“Eosinophilia/chemically induced”[MAJR] AND (“cephalosporins”[MeSH Terms] ) AND “drug rash with eosinophilia”

Is serum eosinophilia associated with scabies?

Bottom line:  Some patients infested with scabies do develop eosinophilia.

Infestations and bites, scabies.  In:  Clinical Dermatology, 2009. [MDConsult].

Table 70-1. Diseases, Syndromes, and Conditions Commonly Associated With Peripheral Blood Eosinophilia and/or Tissue Eosinophilia.  In:  Hematology: Basic Principles and Practice, 6th ed, 2012.  [MDConsult]

What is cutaneouls T cell lymphoma and Sezary syndrome?

Bottom line:  Cutaneous T-cell lymphoma (CTCL) is a group of malignant lymphomas in which malignant T lymphocytes express cutaneous lymphocyte antigen (CLA) to infiltrate the skin.  Mycosis fungoides is the most common variant of CTCL.  Sézary syndrome is a leukemic variant of mycosis fungoides.

Summary: Cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome).  In:  Williams Hematology [AccessMedicine]

Hwang ST, et al. Mycosis fungoides and Sézary syndrome.  Lancet. 2008 Mar 15;371(9616):945-57.

Clinical presentation of mycosis fungoides can vary; skin findings include erythematous patches, plaques, and less frequently, tumors.

Diagnosis of Sézary syndrome is made primarily on the basis erythroderma plus molecular
and flow cytometric evidence of a large clonal population of abnormal T cells in the blood.
Also usually includes lymphadenopathy and pruritis.

 

Where are xanthomas or xanthelasmas likely to occur on the body?

Bottom line:  Xanthelasmas are xanthomas that occur on the eyelids.  Xanthomas also occur on other parts of the body, including hands, tendons, chest, shoulders and back.

Kevaghn P, et al.  Xanthomas: clinical presentation.  Medscape, 2012.

The pathogenesis and clinical significance of xanthelasma palpebrarum.  J Amer Acad Dermatology.  1994; 30(2, pt. 1): 236-42.
Xanthelasmas are the most common form of xanthelomas and occur on the eyelid and surrounding the eye.  Other xanthelomas can occur in other areas including hands, tendons, chest, shoulders and back.  Some research suggests that they occur in pressure areas as well as where “local heat [increasing capillary] leakage rate” of LDL.  Local heat being caused by trauma, inflammation, infection, and possibly friction.  This paper suggests that the friction from the movement of eyelids may be involved in the tendency for xanthlasmas to develop in the setting of hyperlipidemia.