Krista Reznik – Derm In-Review
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Krista Reznik

Sep 03
Love15

August 2024 Case Study

By 2024 Case Studies

August 2024 Case Study

Author: Nagasai Adusumilli, MD, MBA1

  1. Department of Dermatology, The George Washington University School of Medicine and Health Sciences

Patient History

A 64-year-old female with a medical history of chronic microcytic anemia presented to the clinic with 1 month of intensely pruritic discolored lesions on the lower legs. They have been increasing in number and extending onto the lower thighs, prompting her visit. She denies any changes to her chronic medical history or any daily medications. Skin findings of the bilateral extensor knees and ankles are shown in Figures 1 and 2. No primary lesions were appreciated in the oral cavity or genital region. Preliminary bloodwork was pertinent for a negative HIV and syphilis screen.

Based on the clinical presentation, which phenomenon/sign is illustrated, and in what other condition can this be appreciated?

A.) Meyerson; pemphigus vulgaris
B.) Asboe-Hansen; hemangioma
C.) Darier’s; dermatomyositis
D.) Gorlin’s; keratosis pilaris atrophicans faciei
E.) Koebner; hemangioma
F.) Samitz; pemphigus vulgaris
G.) Hertoghe’s; mastocytoma
H.) Koebner; vitiligo
I.) Darier’s; neurofibromatosis type I
J.) Gorlin’s; Ehlers-Danlos
K.) Meyerson; dermatomyositis
L.) Samitz; pityriasis rubra pilaris
M.) Hertoghe’s; lichen nitidus

 

        

Correct Answer: H

Explanation/Literature review:

This patient has lichen planus, with the typical morphology of well-demarcated, flat-topped, dark brown to violaceous papules in clusters.1 Classic distributions include distal lower extremities, as with this patient, wrists, forearms, and lower back.1 The linear configuration seen in both figures, in the context of the intense symptom of pruritus, is suggestive of the Koebner phenomenon or isomorphic response.1,2 A cluster of lesions arranged in a linear pattern result from injury to previously uninvolved skin, including from scratching, arthropod bites, piercing, tattoos, and surgery.2 True Koebnerization can be seen in a wide range of conditions, including but not limited to psoriasis, lichen nitidus (M), cutaneous small vessel vasculitis, juvenile rheumatoid arthritis, vitiligo, Darier’s disease (keratosis follicularis), and pityriasis rubra pilaris (L).2,3 Answer choice H correctly identifies both the illustrated phenomenon and another condition in which it can be observed.

Incorrect answer choices:4

Although eponyms in dermatology are slowly phasing out, we remain in a transition period in which distinct findings and conditions are still commonly referred to by historical names in both clinical practice and academic writing. As they build their dermatology lexicon, trainees must still be familiar with these eponymous signs.

Meyerson phenomenon (A, K) – halo of eczematous dermatitis of scale and erythema overlying melanocytic lesions5

Asboe-Hansen sign (B) – enlargement or extension of a vesicle or bullae to surrounding skin by applying downward finger pressure. Can be seen in various vesicobullous conditions, including pemphigus vulgaris (A, F).

Darier’s sign (C, I) – Rubbing induces urticaria, swelling, or vesiculation due to histamine release. Classically seen in a mastocytoma (G). Can also be seen in leukemia cutis, juvenile xanthogranuloma, and Langerhans cell histiocytosis.

Gorlin’s sign (D, J) – Patients with Ehlers-Danlos can extend their tongue to touch the nasal tip. While answer choice J is has appropriate associations, this does not fit the vignette.

Hemangioma (B, E) – The Kasabach-Merritt phenomenon can be seen in tufted angiomas and kaposiform hemangioendotheliomas. Platelets are trapped in vascular lesions, leading to rapid growth of the lesion and thrombocytopenia.

Samitz sign (F, L) – Frayed or ragged nail folds characteristic of nail changes in dermatomyositis (C, K).

Hertoghe’s sign (G, M) – Loss of lateral eyebrow hair that can be seen a wide range of conditions, including keratosis pilaris atrophicans faciei (D), alopecia areata, follicular mucinosis, discoid lupus, and trichotillomania.

Neurofibromatosis type I (I) – Crowe’s sign refers to the axillary freckling that can be part of the diagnostic criteria for NF1.

 

References:

  1. Shiohara T & Mizukawa Y. Chapter 11 – Lichen Planus and Lichenoid Dermatoses. In: Dermatology. 5th Edition. Elsevier; 189-209.
  2. High WA, Tomasini CF, Argenziano G, et al. Chapter 0 – Basic Principles of Dermatology. In: Dermatology. 5th Edition. Elsevier; 1-99.
  3. Sharma RK, Gupta M, Gulati A. Koebner phenomenon in classic juvenile onset pityriasis rubra pilaris. Indian Dermatol Online J. 2019 Jul-Aug;10(4):469-470. PMID: 31334074.
  4. Madke B, Nayak C. Eponymous signs in dermatology. Indian Dermatol Online J. 2012 Sep;3(3):159-65. PMID: 23189246.
  5. Nicholls DS, Mason GH. Halo dermatitis around a melanocytic naevus: Meyerson’s naevus. Br J Dermatol. 1988 Jan;118(1):125-9. PMID: 3342172.
Jul 01
Love15

June 2024 Case Study

By 2024 Case Studies

June 2024 Case Study

Author: Nagasai Adusumilli, MD, MBA1

  1. Department of Dermatology, The George Washington University School of Medicine and Health Sciences

Patient History

A 60-year-old male with a medical history of HIV (updated CD4 count of 30) presented to the emergency department with headaches, disorientation, and acute onset vision loss. He was found to have an increasing number of skin lesions on the face for the past 3 weeks. Multiple well-demarcated, eroded papulonodules were seen on the nose and forehead (Figure 1). A punch biopsy of the dorsal nose showed the findings on hematoxylin and eosin in Figure 2.

Based on the clinical presentation and biopsy findings, which three stains would be most useful to highlight the underlying pathology?

A.) Verhoeff-Van Gieson
B.) Thioflavin T
C.) Periodic acid-Schiff
D.) Von Kossa
E.) Fontana- Masson
F.) Masson trichrome
G.) Toluidine blue
H.) Mucicarmine
I.) Fite
J.) HHV-8

 

        

 

Correct Answer: C, E, H

Explanation/Literature review:

In the setting of AIDS (CD4 count less than 50), the scattered, umbilicated, and eroded papules and nodules across the head and neck distribution (Figure 1) are suspicious for opportunistic infections. Even within just the infectious category, the differential diagnoses are broad, including molluscum, tuberculosis, leishmaniasis, monkeypox, cat scratch, secondary syphilis, and various mycoses. Histoplasma, Cryptococcus, Coccidioides, and Talaromyces are among the deep fungal infections that fit this clinical morphology.1 Figure 2 shows a dermal infiltrate on high power, with innumerable fungal yeasts of different sizes within clear spaces of circular capsules. Yeasts of variable size and shape clustered within capsules are distinctly characteristic of Cryptococcus, particularly in the context of central nervous system (CNS) symptoms and molluscoid cutaneous findings.2 Consequently, a fungal stain like PAS (answer choice C) can help highlight the yeasts.2 Although Fontana-Masson is typically used to highlight melanin and the absence of melanin in vitiligo, the pleomorphic cryptococcal yeasts stain yellow-brown to black with Fontana-Masson (answer choice E).2,3 Mucicarmine (answer choice H) stains pink the organism’s classic polysaccharide capsule,2 which serves as the antiphagocytic virulence factor.

Cryptococcus is an encapsulated fungus found in bird droppings, with human transmission occurring through inhalation of the yeasts.4 The fungus then lies dormant in the hilar lymph nodes and lungs, walled off with granulomatous inflammation.4 During periods of immunocompromise, the fungus proliferates and disseminates throughout the body, including to the brain, bone, and skin.4 Cryptococcus demonstrates a tropism for the skin and brain,5,6 so dermatologists must recognize cutaneous cryptococcal infection both clinically and under histopathology. Meningoencephalitis drives mortality in disseminated cryptococcal infection, so the CNS symptoms seen in this patient case must raise suspicion for disseminated Cryptococcus.7 The HIV pandemic led to a spike in incidence in many opportunistic infections, including cryptococcosis.8 Now with an estimated global incidence of 1 million cases per year,4 cryptococcosis must be on the differential for umbilicated papules and nodules, particularly in patients with immunocompromise. Additional settings of immunocompromise include patients with chronic corticosteroid use, solid organ transplants, and chronic tumor necrosis factor inhibitor use.5 Skin-limited disease from traumatic inoculation has been described but is rare.7 If Cryptococcus is identified in the skin, investigation for systemic involvement, especially in the CNS and lungs, is crucial.7

 

Incorrect stains in answer choices:3

Verhoeff-Van Gieson (choice A) – elastic fibers stain black. Example conditions: pseudoxanthoma elasticum, anetoderma, cutis laxa.

Thioflavin T (choice B) – amyloid stains yellow-green under fluorescence.

Von Kossa (choice D) – calcium salts stain black. Example conditions: calciphylaxis, calcinosis cutis, pseudoxanthoma elasticum.

Masson trichrome (choice F) – Mature collagen stains blue-green whereas smooth muscle stains red. Example conditions: scar vs. leiomyoma.

Toluidine blue (choice G) – mast cell granules and mucin stain purple. Example conditions: urticaria pigmentosa, connective tissue disease.

Fite (choice I) – partially acid-fast organisms such as Nocardia and atypical mycobacteria stain pink.

HHV-8 (choice J) – Kaposi sarcoma stains brown.

 

References:

  1. Elewski BE, Hughey LC, Hunt KM, Kay RJ. Chapter 77 – Fungal Diseases. In: Dermatology. 5th Edition. Elsevier; 1343-1375.
  2. Elston DM. Chapter 18 – Fungal infections. In: Dermatopathology. 3rd Elsevier; 306-320.
  3. Ferringer T, Ko CJ. Chapter 1 – The basics. In: Dermatopathology. 3rd Elsevier; 1-35.
  4. Meya DB, Williamson PR. Cryptococcal disease in diverse hosts. N Engl J Med. 2024 May 2;390(17):1597-1610. PMID: 38692293.
  5. Maziarz EK, Perfect JR. Cryptococcosis. Infect Dis Clin North Am. 2016 Mar;30(1):179-206. PMID: 26897067.
  6. Moe K, Lotsikas-Baggili AJ, Kupiec-Banasikowska A, Kauffman CL. The cutaneous predilection of disseminated cryptococcal infection in organ transplant recipients. Arch Dermatol. 2005 Jul;141(7):913-4. PMID: 16027320.
  7. Perfect JR, Dismukes WE, Dromer F, et al. Clinical practice guidelines for the management of cryptococcal disease: 2010 update by the infectious diseases society of america. Clin Infect Dis. 2010 Feb 1;50(3):291-322. PMID: 20047480.
  8. Durden FM, Elewski B. Cutaneous involvement with Cryptococcus neoformans in AIDS. J Am Acad Dermatol. 1994 May;30(5 Pt 2):844-8. PMID: 8169258