A Woman with Fever, Malaise, Myalgias and Rash

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White Islands in a Sea of Red

Presented by Dr Henry B.B. Foong

from Foong Skin Specialist Clinic

Ipoh, Malaysia

A 45-year-old woman presented with a one-week history of fever and generalised rash. She initially had fever and sore throat and subsequently developed shoulder myalgia.  She saw her general practitioner who prescribed antihistamines and analgesics.  She then developed rashes on the legs.  Thinking this could be a drug eruption, the patient consulted her general practitioner again who did not think this was a cutaneous drug eruption.  Instead, he prescribed amoxicillin/clavulanate, but the rash spread further to her trunk and upper limbs.  The rash was only mildly pruritic.  She had no eye, oral or genital symptoms. There was no history of contact with patients with similar illness.

Physical examination showed stable vital signs with no dehydration. There was generalized confluent erythema distributed mainly on the legs but extending to trunk and upper extremities.  Prominent “islands of white” within the erythema over the lower legs were noted. It did not blanch completely, leaving behind petechiael lesions when pressed.

Clinical Photographs

Lab: Blood tests were positive for Dengue IgG and IgM antibody.

TWBC 5,100 (N38% L 36% M 22% E3% B1%) platelets 71,000 and Hct 38%

Pathology: 2 skin biopsies were taken from the right leg. One from the confluent generalised erythema and another from the white islands. Histopathology is pending

Diagnosis: Exanthem of Dengue Fever

Discussion

Dengue Fever ranks among one of the most important arthropod borne viral infections with high morbidity and mortality in the tropics and subtropics including urban populations. It is caused by Dengue virus, a flavivirus which is transmitted by mosquitos Aedes aegypti and Aedes albopictus.  There are 4 distinct serotypes DENV-1,2,3 and 4. Each episode of infection induces a life-long protective immunity to the homologous serotype but confers only  partial  and  transient  protection  against  other  serotypes.

The cutaneous manifestations of dengue fever are characteristic.  Skin eruptions appear in 80% of patients during the remission of the fever. Approximately one-half of patients develop a centrifugal macular, maculopapular, scarlatiniform, or petechial eruption. The cutaneous eruptions may become confluent, with small, round islands of sparing, the so-called white islands in a sea of red. The rash characteristically starts on the dorsum of the hands and feet and spreads to the arms, legs, and torso; the face is rarely involved. The eruption seen in dengue is usually short-lived, lasting from a few hours to several days.

Reference:

eMedicine:  Dermatologic Manifestations of Dengue.

from eMedicine reference

Atypical Melanocytic Lesions

The patient is an 87 yo man with a history of dysplastic nevi and melanoma-in-situ.  He is seen every four to sic months.  The current exam found a 1.5 cm atypical melanocytic lesion on his left mid back.

Clinical and Dermatoscopic Images

What are your thoughts?

PVLE Variant?

May 12, 2017
The patient is a 75 yo man with a 7 day history of a pruritic papular eruption on his legs.  This began on a recent trip to Florida after sun exposure.  He is on no photosensitizing medications.

O/E:  Erythematous non-blanching papules on both lower extremities.

Clinical Images:

Lab:  CBC, ANA normal

Pathology:  No evidence of vasculitis

Diagnosis:  Could this be an example of Papulo-vesicular Light Eruption?

References:

1.  Papulovesicular light eruption. A defined subset of polymorphous light eruption.

Elpern DJ, Morison WL, Hood AF.  Arch Dermatol. 1985 Oct;121(10):1286-8.

Abstract:  A distinctive photodermatitis is seen commonly in tourists visiting Hawaii. Analysis of 150 cases revealed that the eruption is acute in onset following exposure to sunlight, is confined to exposed areas, and mainly affects young to middle-aged white women. The clinical presentation is remarkably uniform, consisting of papules, papulovesicles, or vesicles. These findings are consistent with a diagnosis of polymorphous light eruption (PMLE) and the histologic picture supports this diagnosis. Other clinical variants of PMLE were not seen in our patients, however, which suggests that this condition is more monomorphous than polymorphous. We suggest papulovesicular light eruption as a suitable name for this common and distinctive subset of PMLE. 

2.  Papulovesicular light eruption. A defined subset of polymorphous light eruption.

Elpern DJ, Morison WL, Hood AF. Arch Dermatol. 1985 Oct;121(10):1286-8.

Abstract: A distinctive photodermatitis is seen commonly in tourists visiting Hawaii. Analysis of 150 cases revealed that the eruption is acute in onset following exposure to sunlight, is confined to exposed areas, and mainly affects young to middle-aged white women. The clinical presentation is remarkably uniform, consisting of papules, papulovesicles, or vesicles. These findings are consistent with a diagnosis of polymorphous light eruption (PMLE) and the histologic picture supports this diagnosis. Other clinical variants of PMLE were not seen in our patients, however, which suggests that this condition is more monomorphous than polymorphous. We suggest papulovesicular light eruption as a suitable name for this common and distinctive subset of PMLE.

A Case for Diagnosis

The patient is a 58 yo woman with asymptomatic lesions under breasts, in the axillae and groin.  She is on no medications,  She has breast cancer seven years ago treated with lumpectomy and chemotherapy (no radiation) and has been off therapy for 6 years.

O/E:  slightly hyperpigmented macules in affected areas.  KOH negative.

Clinical Photos:

Left axilla

Pathology:  2 representative punch biopsies were taken. 

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Report: Focal basal layer vacuolization, a mild superficial perivascular and focally band-like lymphocytic infiltrate with rare eosinophils, papillary dermal fibrosis, numerous melanophages, and telangiectasia, consistent with a lichenoid interface dermatitis with post-inflammatory hyperpigmentation.

NOTE: The differential diagnosis could include lichen planus (possibly lichen planus pigmentosus) or a lichenoid drug eruption.

Diagnosis: Lichen Planus Variant.  Is this annular atrophic lichen planus?

Reference:
Annular atrophic lichen planus.

Morales-Callaghan A Jr1, et. al. J Am Acad Dermatol. 2005 May;52(5):906-8.

Abstract: We describe a new case of annular atrophic lichen planus. Annular atrophic lichen planus is a rare variant of lichen planus that is clinically characterized by violaceous plaques of annular morphology with central atrophy and histopathologic findings of annular and atrophic lichen planus, respectively. There have been 4 cases of this entity reported previously.

Recessive X-Linked Ichthyosis

The patient is a 65-year-old engineer who presents for evaluation of skin lesions.  He grew up in a Southern state and during high school and college worked summers doing roadwork.  He wants a screening for skin cancer. The patient has had a B-cell lymphoma 3-4 years ago. 

He has had a form of ichthyosis since about age 2.  Other family members are affected.  They are all on his mother’s side.  His mother’s brother and some male maternal cousins are similarly affected. He always felt like an outsider in his school, and was bullied in grade and high school because of this scaly skin.  This has informed his self-image as an adult.

EXAMINATION:  A complete skin examination was done. He has scaly polygonal, somewhat “dirty-appearing” plaques on the legs, torso, and arms.  He has normal palms.  Head and neck are normal.  He has no lesions that are worrisome for melanoma, nonmelanoma skin cancer, or actinic keratoses. 

Clinical Images:  

Polygonal Scale

Normal Palm in X-Linked Ichthyosis

IMPRESSION:  Recessiive X-linked ichthyosis.

PLAN:  We discussed his ichthyosis.  One can get free Aquaphor from the drug company and we will order some for him. 

I will run his case by some pediatric dermatology colleagues.  I do not know whether any further testing is needed for a 65-year-old man who is not going to have any children.  

Corneal opacities are relatively common in these patients and may be evident with slit-lamp examination both of adult males who are affected and of women who are carriers. Cryptorchidism has been reported in 20% of patients. A few cases of testicular cancer have developed in patients with X-linked ichthyosis and cryptorchidism.

Reference:
FIRST: The Foundation for Ichthyosis and Related Skin Types is a great resource for patients and providers.  See FIRST’s page on Recessive X-Linked Ichthyosis.

60 yo man with scaly rash upper chest

This 60 yo disabled man has had a sic moth history of a mildly pruritic eruption of his upper chest.  I expected the KOH prem to be postive, but it was not.  He has lots of psychosocial problems (wife an alcoholic, daughter a heroin addict) -- not enough time to properly evaluate; so a biopsy was done.  If that is negative for fungal elements I would think about Casal's necklace whigh I have never seen.

Pathology:  The biopsy showed a mild superficial spongiotic dermatitis with no evidence offungal elements.

Follow-up:  When seen back for suture removal, another KOH prep was done.  It was positive for numerous septate hyaline hyphae.  The original KOH prep was taken from the advancing scaly border as was the biopsy.  Sampling error caused the misdiagnosis.  The patient was started on terbinafine 250 mg daily for six weeks.  Follow-up photos will be posted when he returns.