The answer to sQuiz: Segmental Darier’s disease Darier’s disease is an autosomal, dominantly inherited, genodermatosis, mainly characterized by hyperkeratotic papules in seborrheic regions, which aggravate during the summer. In segmental Darier’s disease, the hyperkeratotic papules show a linear distribution along Blaschko’s lines, reflecting a postzygotic mosaicism in the ATP2A2 gene. Here, the diagnosis was confirmed histologically and the mutation was detected. There are two types of Darier’s disease. Type 1 is purely segmental with no diffuse skin involvement. In type 2 the segmental dermatosis is superimposed on a milder, nonsegmental manifestation of the same trait [1]. Epidermal naevi can share clinical and histological features with segmental Darier’s disease. The former are mainly congenital and show no variation with sun-exposure. Nevertheless, the definitive differential diagnosis can be made by the demonstration of ATP2A2 gene mosaicism, by the detection of a mutation in a biopsy of the affected skin, which has to be absent in uninvolved skin [2].
References
1. Sanderson EA, Killoran CE, Pedvis-Leftick A, Wilkel CS. Localized Darier’s disease in a Blaschkoid distribution: two cases of phenotypic mosaicism and a review of mosaic Darier’s disease. J Dermatol 2007; 34: 761-4. 2. Huh WK, Fujiwara K, Takahashi H, Kanitakis J. Congenital acantholytic dyskeratotic epidermal naevus following Blaschko’s lines versus segmental Darier’s disease. Eur J Dermato 2007; 17: 130-2. doi:10.1684/ejd.2014.2339
A
Residents’corner February 2014. DeRmpath & Clinic: Differential diagnosis in palisading non-infectious granulomas 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]
2
Case 1: 31 year-old female with no relevant past medical background. Six-month history of papules and plaques with an annular pattern on the upper limbs. Case 2: 34 year-old female, with poorly controlled type 1 diabetes. Sixteen-month history of a yellow-brownish atrophic plaque surrounded by a raised, violaceus border, on the left leg. Palisaded and interstitial granulomatous dermatitis characterize both cases. Other aspects may help to determine the differential diagnosis. In the first case (figures 1A,2A), the following findings are observed in the dermis: – upper half of the dermis is more involved than the lower part; – granulomas with mucin centers surrounded by palisades of histiocytes; – degeneration of collagen bundles; – areas of spared dermis between palisaded granulomas; In the second case (figures 1B,2B) we observe: – thinned epidermis with effacement of rete ridges; – lower part of the dermis more affected than the upper part; – diffuse involvement of the dermis by histiocytic infiltrate arranged in palisades and interstitial array with no intervening normal dermis;
B
Figure 1. A) Granuloma annulare in scanning magnification: palisading granulomas in the upper dermis (H&E). B) Necrobiosis lipoidica in scanning magnification: palisaded granulomas in a linear horizontal arrangement throughout the dermis (H&E).
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A
B
Granuloma Mucin Histiocytes
Histiocytes
Figure 2. A) Granuloma annulare in high power: epithelioid histiocytes around mucin forming a nodule in the upper dermis (H&E). B). Necrobiosis lipoidica in high power: degenerated collagen surrounded by palisaded histiocytes (H&E).
– linear arrangement of inflammatory cells in two or three horizontal tiers in the dermis; – palisades of histiocytes around foci of degenerative collagen, without mucin in the center;
Residents’corner February 2014. Clues in DeRmosCopy: Bloody parallel ridges
Diagnosis: Case 1: Granuloma annulare (GA); Case 2: Necrobiosis lipoidica (NL) Besides granuloma annulare and necrobiosis lipoidica, sarcoidosis and rheumatoid nodules also constitute differential diagnosis in non-infectious granulomas. A strong association between NL and diabetes has been described. Diabetes mellitus (especially type I) was found in about 65% of NL patients, while only 0.03% of diabetic patients presented NL. Therefore, all NL patients should be followed closely to ensure that diabetes is not concurrent nor manifests later. In a different way, the association between GA with diabetes is still controversial (and mainly related with widespread GA).
Ben A ESDAILE, Rubeta N MATIN, Jonathan C BOWLING Dermatology Department, Churchill Hospital, Old Road, Headington, Oxford OX3 7LJ. United Kingdom e-mail: [email protected]
Dermoscopy of pigmented lesions on the palms and soles often display a parallel pigment pattern due to the unique anatomy of acral skin.
The anatomy of the acral skin
doi:10.1684/ejd.2014.2340
The eccrine ducts open at the top of the ridges (crista superficialis) (figure 1). Benign naevi tend to localise to the crista limitans and transfer their pigment to keratinocytes associated with the furrow, giving the classical benign parallel furrow pattern seen on dermoscopy (figure 2A).
A
B
D
C
E
Figure 1. The anatomy of acral skin. A: eccrine duct opening, B: furrow (sulcus superficialis), C: ridge (crista superficialis), D: benign melanocytes cluster at crista limitans and E: paired capillaries (reproduced with permission, ‘Diagnostic Dermoscopy: The Illustrated Guide’ – Wiley- Blackwell 2011).
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EJD, vol. 24, n◦ 1, January-February 2014
References
1. Sanderson EA, Killoran CE, Pedvis-Leftick A, Wilkel CS. Localized Darier’s disease in a Blaschkoid distribution: two cases of phenotypic mosaicism and a review of mosaic Darier’s disease. J Dermatol 2007; 34: 761-4. 2. Huh WK, Fujiwara K, Takahashi H, Kanitakis J. Congenital acantholytic dyskeratotic epidermal naevus following Blaschko’s lines versus segmental Darier’s disease. Eur J Dermato 2007; 17: 130-2. doi:10.1684/ejd.2014.2339
A
Residents’corner February 2014. DeRmpath & Clinic: Differential diagnosis in palisading non-infectious granulomas 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]
2
Case 1: 31 year-old female with no relevant past medical background. Six-month history of papules and plaques with an annular pattern on the upper limbs. Case 2: 34 year-old female, with poorly controlled type 1 diabetes. Sixteen-month history of a yellow-brownish atrophic plaque surrounded by a raised, violaceus border, on the left leg. Palisaded and interstitial granulomatous dermatitis characterize both cases. Other aspects may help to determine the differential diagnosis. In the first case (figures 1A,2A), the following findings are observed in the dermis: – upper half of the dermis is more involved than the lower part; – granulomas with mucin centers surrounded by palisades of histiocytes; – degeneration of collagen bundles; – areas of spared dermis between palisaded granulomas; In the second case (figures 1B,2B) we observe: – thinned epidermis with effacement of rete ridges; – lower part of the dermis more affected than the upper part; – diffuse involvement of the dermis by histiocytic infiltrate arranged in palisades and interstitial array with no intervening normal dermis;
B
Figure 1. A) Granuloma annulare in scanning magnification: palisading granulomas in the upper dermis (H&E). B) Necrobiosis lipoidica in scanning magnification: palisaded granulomas in a linear horizontal arrangement throughout the dermis (H&E).
EJD, vol. 24, n◦ 1, January-February 2014
139
A
B
Granuloma Mucin Histiocytes
Histiocytes
Figure 2. A) Granuloma annulare in high power: epithelioid histiocytes around mucin forming a nodule in the upper dermis (H&E). B). Necrobiosis lipoidica in high power: degenerated collagen surrounded by palisaded histiocytes (H&E).
– linear arrangement of inflammatory cells in two or three horizontal tiers in the dermis; – palisades of histiocytes around foci of degenerative collagen, without mucin in the center;
Residents’corner February 2014. Clues in DeRmosCopy: Bloody parallel ridges
Diagnosis: Case 1: Granuloma annulare (GA); Case 2: Necrobiosis lipoidica (NL) Besides granuloma annulare and necrobiosis lipoidica, sarcoidosis and rheumatoid nodules also constitute differential diagnosis in non-infectious granulomas. A strong association between NL and diabetes has been described. Diabetes mellitus (especially type I) was found in about 65% of NL patients, while only 0.03% of diabetic patients presented NL. Therefore, all NL patients should be followed closely to ensure that diabetes is not concurrent nor manifests later. In a different way, the association between GA with diabetes is still controversial (and mainly related with widespread GA).
Ben A ESDAILE, Rubeta N MATIN, Jonathan C BOWLING Dermatology Department, Churchill Hospital, Old Road, Headington, Oxford OX3 7LJ. United Kingdom e-mail: [email protected]
Dermoscopy of pigmented lesions on the palms and soles often display a parallel pigment pattern due to the unique anatomy of acral skin.
The anatomy of the acral skin
doi:10.1684/ejd.2014.2340
The eccrine ducts open at the top of the ridges (crista superficialis) (figure 1). Benign naevi tend to localise to the crista limitans and transfer their pigment to keratinocytes associated with the furrow, giving the classical benign parallel furrow pattern seen on dermoscopy (figure 2A).
A
B
D
C
E
Figure 1. The anatomy of acral skin. A: eccrine duct opening, B: furrow (sulcus superficialis), C: ridge (crista superficialis), D: benign melanocytes cluster at crista limitans and E: paired capillaries (reproduced with permission, ‘Diagnostic Dermoscopy: The Illustrated Guide’ – Wiley- Blackwell 2011).
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EJD, vol. 24, n◦ 1, January-February 2014
