The answer to sQuiz: Elephantiasis nostras verrucosa On this basis, a diagnosis of elephantiasis nostras verrucosa (ENV) was made. The patient refused any surgical intervention and he was treated with oral etretinate and topical emollients without noticeable improvement. ENV is an uncommon and singular array of dermatologic manifestations that can complicate chronic lymphedema. ENV can be diagnosed clinically with history and physical examination alone. Skin biopsy and imaging techniques can rule out other causes of secondary lymphedema, such as obstructive malignancy. Differential diagnoses include chromoblastomycosis, filariasis, lipodermatosclerosis, venous stasis dermatitis, verrucous carcinoma, papular mucinosis and pretibial myxedema [1, 2].


References

1. Dean SM, Zirwas MJ, Horst AV. Elephantiasis nostras verrucosa: an institutional analysis of 21 cases. J Am Acad Dermatol 2011; 64: 1104-10. 2. Baird D, Bode D, Akers T, Deyoung Z. Elephantiasis Nostras Verrucosa (ENV): a complication of congestive heart failure and obesity. J Am Board Fam Med 2010; 23: 413-7.

Case 2: A 46-year-old male with no relevant past medical history. A five-month history of a diffuse, erythematous, scaly, cutaneous eruption, with craniocaudal progression, characterized by reddish-orange colored scaling plaques with well defined borders, covering almost the entire body, sparing some areas on the trunk and limbs. Certain histopathologic features are common to both entities, such as parakeratosis, elongated rete ridges, increased number of mitotic figures in the epidermal keratinocytes and the presence of lymphocytes in a sparse infiltrate of inflammatory cells. However, there are some specific features, which help to distinguish the two diseases. In the first case (figures 1A and 2A), the following findings are observed: – Parakeratosis, mostly confluent; – Presence of neutrophils in the cornified layer (Munro’s microabscesses) and in the epidermis (subcorneal spongiform pustules of Kogoj); – Long rete ridges, of approximately equal length, with a club-like appearance of the fusion tips; – Thinning of suprapapillary plates; – Absent/thin granular cell layer; – Dilated, tortuous capillaries in dermal papillae; – Lymphocytic superficial dermal infiltrate. In the second case (figures 1B and 2B) we observe:

doi:10.1684/ejd.2014.2359

Residents’corner April 2014. DeRmpath & Clinic: Differential diagnosis of two psoriasiform dermatoses Ana Rita TRAVASSOS1 , Luís SOARES-DE-ALMEIDA1,2 1

Dermatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisboa, Portugal Faculty of Medicine, University of Lisboa, Lisboa, Portugal e-mail: [email protected]

– Psoriasiform hyperplasia; – Hyperkeratosis with parakeratosis alternating with orthokeratosis in both vertical and horizontal directions (chessboard pattern); – No neutrophils in the cornified layer; – Preservation of the granular zone across the entire front of a section; – Rete ridges longer than normal, but not thin and not of even length; – Thick suprapapillary plates: – Spar se lymphocytic superficial perivascular dermal infiltrate.

2

Case 1: A 43-year-old healthy male with a recurrent chronic dermatosis, characterized by thick plaques covered with a silvery white scale, localized on the extensor surfaces of the knees, elbows, trunk and scalp.

282

Diagnosis: Case 1: Psoriasis Case 2: Pityriasis rubra pilaris (PRP) The differential diagnosis of these two entities is still a controversial issue and, in fact, some authors still believe that the two diseases are indistinguishable from one another by conventional microscopy.

EJD, vol. 24, n◦ 2, March-April 2014

A

B

Figure 1. A) Psoriasis in scanning magnification: confluent parakeratosis, neutrophils in the cornified layer (Munro’s microabscesses), absent granular layer, long and thin rete ridges, with thin suprapapillary plates. B) PRP in scanning magnification: alternating parakeratosis and orthokeratosis in a chessboard pattern, preservation of the granular zone and thick epidermal suprapapillary plates.

B

A Confluent parakeratosis Thin suprapapillary plates

Neutrophils

Chessboard pattern

Absent granular zone

Granular zone Thick suprapapillary plates

Figure 2. A) Psoriasis in high power: Presence of neutrophils in the area of compact parakeratosis and dilated capillaries in dermal papillae. B) PRP in high power: Parakeratosis alternating with orthokeratosis (chessboard pattern), prominent granular zone and thick suprapapillary plates.

Pityriasis rubra pilaris is much less common than psoriasis, has a characteristic abrupt onset and rapidly becomes widespread (specially the acquired form). Furthermore, unlike psoriasis, which tends to persist for many years unless the patient receives therapy, PRP usually remits within one to three years of its onset. doi:10.1684/ejd.2014.2360

Residents’corner April 2014. Nail it!: Eye-catching nail folds Kristina BUDER, Henning HAMM Department of Dermatology, Venereology and Allergology, University Hospital Wuerzburg, Josef-Schneider-Str. 2, 97080 WÜRZBURG, Germany e-mail: [email protected]

A 62-year-old male patient was referred with a 6-month history of an erythematous face and hands. He complained EJD, vol. 24, n◦ 2, March-April 2014

about fatigue and particularly weakness of his shoulders and upper arms since 6 weeks. On clinical examination he presented with deep-red facial erythema sparing the perioral region, significant periorbital oedema, purple erythema on his décolleté, erythematous macules and papules on the dorsal aspect of his fingers, especially over the bony prominences, and periungual erythema. Prominent nail fold changes comprised telangiectasia, haemorrhages and hyperkeratotic cuticulae (figure 1). Neurological examination revealed symmetric proximal paresis of the upper arms. The clinical diagnosis of dermatomyositis was confirmed by markedly elevated creatinine phosphokinase of 3879 U/L (norm