Faculty Bibliography

Porphyria cutanea tarda (PCT) is the most common type of porphyria, presenting in middle-aged patients with a photodistributed vesiculobullous eruption, milia, and scars. Porphyria cutanea tarda occurs in relation to inhibition of uroporphyrinogen decarboxylase, a key enzyme in the heme biosynthesis pathway. A number of genetic and acquired factors increase susceptibility to PCT by reducing uroporphyrinogen decarboxylase activity. A handful of other vesiculobullous conditions may mimic PCT both clinically and histologically; therefore, both skin biopsy and laboratory evaluation are helpful in confirming the diagnosis. We report a case of PCT in the setting of cigarette usage and untreated hepatitis C infection.

Lichen planopilaris (LPP) is a cicatricial alopecia that often causes permanent hair loss. Pioglitazone, a peroxisome proliferator activated receptor-gamma (PPAR- γ) agonist, has demonstrated immunomodulatory properties that may offer an effective treatment modality. This retrospective analysis describes 23 patients with LPP treated with adjunctive pioglitazone. Most (18/25) demonstrated significant reduction in patient-reported symptoms and clinical signs of inflammation. No adverse effects were reported.

Well-known causes of zinc deficiency, also referred to as acrodermatitis enteropathica (AE), include defects in intestinal zinc transporters and inadequate intake, but a rare cause of acquired zinc deficiency discussed here is an iatrogenic nutritional deficiency caused by parenteral nutrition administered without trace elements. While zinc-depleted parenteral nutrition causing dermatosis of acquired zinc deficiency was first reported in the 1990s, it is now again relevant due to a national vitamin and trace element shortage. A high index of suspicion may be necessary to diagnose zinc deficiency, particularly because early clinical findings are nonspecific. We present this case of acquired zinc deficiency in a patient admitted to a pediatric intensive care unit for respiratory distress and atypical pneumonia, who subsequently developed a severe bullous eruption due to iatrogenic zinc deficiency but was treated effectively with enteral and parenteral zinc supplementation, allowing for rapid re-epithelialization of previously denuded skin.

"Eosinophils are absent in psoriasis" has been dogma for generations; yet, there is little published to support this statement. Two recent studies examining the presence of eosinophils in psoriasis came to contrasting conclusions. We reviewed skin biopsies from 50 patients with clinically confirmed cases of psoriasis vulgaris to characterize the histologic features, with a focus on the number of eosinophils in the dermis. We noted the presence of eosinophils in nearly half of our study population (n = 23, 46.0%). There was no significant association between the presence of eosinophils and degree of spongiosis (P = 0.405). Eosinophil density ranged from 0 to 8 per tissue section. The mean average eosinophil density was 1.04 (range: 0-8) per tissue section. Among cases with eosinophils, there were 73.9% (n = 17/23) of cases with 1-2 eosinophils, and 26.1% (n = 6) with 3-8 eosinophils. Mild to moderate spongiosis was noted in the majority of cases (n = 48; 96.0%). Eosinophils were only present in psoriasis cases with evidence of spongiosis (n = 23; 47.9%). We conclude that eosinophils are not an uncommon finding in the dermis of psoriasis vulgaris, although the number is often few. The presence of eosinophils should not preclude a diagnosis of psoriasis, particularly if other histologic features are supportive.

Darier disease is an autosomal dominant genodermatosis of abnormal keratinization characterized by hyperkeratotic papules and plaques with a predilection for seborrheic areas. We report a case of a rare vesiculobullous variant of treatment-resistant Darier disease in a 55-year-old woman that failed topical tacrolimus and topical and oral glucocorticoids. Cetirizine was initiated at 10 mg daily and increased to 40 mg daily over four weeks, with resultant marked improvement of the patient’s burning sensation. A punch biopsy revealed a perivascular infiltrate of eosinophils. This patient’s symptomatic improvement with cetirizine, which has antagonizing properties against eosinophils, highlights the potential role of eosinophils in the pathogenesis of vesiculobullous Darier disease. We suggest that major basic protein secreted by eosinophils may propagate blister formation in vesiculobullous Darier disease by disrupting desmosomes.

Inflammatory skin diseases encompass a vast array of conditions. The field continues to expand and evolve with resurgence of conditions, through newly recognized medication adverse effects, and via more detailed descriptions of known dermatoses. The importance of clinicopathologic correlation and an up to date knowledge of dermatologic conditions cannot be overstated. This review focuses on an array of recent important developments in the histologic diagnosis of inflammatory conditions that affect the skin.

The term, acquired perforating dermatoses (APD), represents a group of skin conditions that develop in adulthood and are characterized by transepidermal elimination of dermal connective tissue. This appears clinically as a papulonodule with a keratotic core. Although APD is typically associated with diabetes mellitus, chronic renal failure, and several other conditions causing generalized pruritus, there have been reports in the literature describing an association of APD with select drugs including sorafenib. We present a case of acquired perforating dermatosis in a patient with HIV and hepatocellular carcinoma undergoing treatment with sorafenib.

Kaposi sarcoma (KS) is a vascular neoplasm that is one of the most common human immunodeficiency virus (HIV)-related malignancies. We present the case of a 42-year-old man with a new diagnosis of HIV and acquired immune deficiency syndrome (AIDS)-related epidemic KS.

Systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) overlap syndrome is an inflammatory disorder with a mixed presentation that is characterized by clinical features of both SLE and AAV. Although renal disease predominates, any organ system in the body may be affected. Neurologic manifestation in patients with SLE-AAV overlap syndrome is rare and has only been previously documented as cerebral ischemia. We report a patient with SLE-AAV overlap syndrome diagnosed based on clinical, serologic and biopsy-proven histologic findings who presented with subarachnoid hemorrhage (SAH) secondary to ruptured right anterior cerebral artery aneurysm. To the authors' knowledge, this is the first reported case of SLE-AAV overlap syndrome diagnosed in a patient with a SAH due to an intracranial aneurysm. Neurologic involvement in patients with SLE-AAV overlap syndrome is uncommon and has not been well-studied. Clinicians who encounter patients with neurologic signs that present with symptoms and a serologic profile that correspond to both SLE and AAV criteria, should consider the association between SLE-AAV overlap syndrome and a hemorrhagic stroke, specifically SAH.

Frontal fibrosing alopecia (FFA) was first described as a progressive recession of the frontal hairline in postmenopausal women. Since its initial description, recognition and understanding of FFA has expanded. The condition is now defined as a patterned, symmetric, frontotemporal scarring alopecia that is considered to be histopathologically indistinguishable from lichen planopilaris. Numerous case reports and series have suggested clinical variants of and associations with FFA. In addition to reviewing the literature on FFA's associations, this article includes a case series of 5 women with skin of color (Hispanic and black) who presented with various cutaneous findings in association with FFA, including lichen planus pigmentosus (LPP), facial papules, and eyebrow loss. Recognition of the conditions that can occur in association with FFA in individuals with skin of color is important in further expanding our knowledge and understanding of FFA as a disease entity.