CED
Clinical dermatology • Concise report
Clinical and Experimental Dermatology
Granulomatous pigmented purpuric dermatosis L. R. Battle,1 S. C. Shalin2 and L. Gao1 1
Department of Dermatology, and 2Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA
doi:10.1111/ced.12549
Summary
The granulomatous variant of the pigmented purpuric dermatoses (PPDs) is a rare and infrequently described condition, with a total of 16 cases published to date. We report a case of granulomatous PPD in a 59-year-old white woman who demonstrated involvement of the arms, legs, chest and back with concurrent hyperlipidaemia. Histopathological examination revealed a lymphohistiocytic infiltrate obscuring the dermoepidermal junction, and loose granuloma formation in the superficial dermis, with extravasated erythrocytes. Other conditions within the differential diagnosis such as atypical infection, papular sarcoidosis and generalized granuloma annulare were excluded on clinical and histological grounds. Our patient represents the ninth patient reported to have granulomatous PPD with coexisting hyperlipidaemia, and the fifth patient with granulomatous PPD and a lichenoid infiltrate.
The pigmented purpuric dermatoses (PPDs) are a group of chronic skin disorders characterized by a perivascular inflammatory infiltrate affecting papillary dermal capillaries, and presence of extravasated erythrocytes with haemosiderin deposition. Subtypes of PPDs include progressive pigmentary dermatosis (Schamberg disease), purpura annularis telangiectodes (Majocchi purpura), pigmented purpuric lichenoid dermatosis of Gougerot and Blum, eczematid-like purpura of Doucas and Kapetanakis, and lichen aureus (lichen purpuricus).1 One of the less well-characterized PPDs, granulomatous PPD, was first reported in 1996 by Saito and Matsuoka.1 Since that time, a total of 16 cases have appeared in the literature. Originally there was thought to be an association between hepatitis C virus, antinuclear antibodies or rheumatoid factor.1 Currently, there seems to be a positive correlation with hyperlipidaemia and granulomatous PPD, with 9 of the 17 reported patients (including the present case) having elevated cholesterol levels.2–6
Correspondence: Dr Ling Gao, Department of Dermatology, University of Arkansas for Medical Sciences, 4301 W. Markham, Slot 576, Little Rock, AR 72205, USA E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 29 April 2014
ª 2014 British Association of Dermatologists
Report A 59-year-old white woman presented with a 3-week history of a mildly pruritic rash. The rash had begun on her legs and spread to involve her trunk and arms. She had a history of presumed oral lichen planus (LP), which had resolved without treatment. She had been taking the proton pump inhibitor omeprazole for 6 years, but had been changed to dexlansoprazole 3 months prior to presentation. Her only other medication was vitamin D, which she had been taking for 5 months. On physical examination, the patient was found to have numerous, erythematous, nonblanching papules, 1–2 mm in size, distributed diffusely across her trunk and limbs (Fig. 1a,b). The clinical differential diagnosis for the leg lesions included Schamberg disease, while conditions considered for the arms and trunk included generalized granuloma annulare (GA), papular sarcoidosis, LP and drug eruption. Multiple biopsies were taken from the patient’s upper arm and back. Histological evaluation revealed vacuolar change and an infiltrate of lymphocytes that focally obscured the dermoepidermal junction. There was rare dyskeratosis and no epidermotropism. Within the superficial dermis was a vaguely nodular lymphohistiocytic infiltrate without multinucleate cells, suggestive of loosely formed granulomas (Fig. 2). Erythrocyte extravasation was present, with focal hemosiderin deposition. Eosinophils were absent.
Clinical and Experimental Dermatology (2015) 40, pp387–390
387
Granulomatous pigmented purpuric dermatosis L. R. Battle et al.
(a)
(a)
(b) (b)
Figure 2 (a) Superficial lymphohistiocytic infiltrate in the dermis, Figure 1 (a) Pinpoint erythematous papules diffusely spread over
the upper back; (b) pinpoint erythematous, nonblanching papules with a few more confluent-appearing patches over the anterior lower legs.
Laboratory findings included elevations in total cholesterol (273 mg/dL; normal < 200 mg/dL) and lowdensity lipoprotein (LDL) cholesterol (175 mg/dL; < 100 mg/dL). Tests for high-density lipoprotein (HDL) cholesterol, triglycerides, random blood glucose, and aspartate and alanine aminotransferases all gave normal values. Tests for hepatitis A and C viruses and for hepatitis B virus surface antigen were all negative. Although an infectious aetiology was considered, special stains (acid-fast bacilli–Fite, periodic-acid– Schiff) and tissue cultures failed to find any fungal, bacterial or mycobacterial organisms. Taken together, the features were felt to be consistent with the granulomatous variant of PPD. The patient was given Derma-Smoothe/FS (fluocinolone acetonide in a blend of oils containing isopropyl alcohol, isopropyl myristate, light mineral oil, oleth-2, refined peanut oil and fragrances; Hill Dermaceuticals Inc., Sanford, FL, USA) to apply to the pruritic areas. At the 10-month
388
Clinical and Experimental Dermatology (2015) 40, pp387–390
focally obscuring the basement membrane zone. Loosely formed granulomas are present; (b) collections of histiocytes forming loose granulomas, with extravasation of erythrocytes and focal haemosiderin deposition. Haematoxylin and eosin, original magnification (a) 9100; (b) 9200.
follow-up, the lesions on the patient’s lower legs were greatly improved, and the lesions on her arms and trunk showed slight improvement. The granulomatous variant of PPD was first reported in 1996 by Saito and Matsuoa.1 Since then, 16 cases have been reported in the literature. Information on these 16 reported cases of granulomatous PPD is summarized in Table 1. Like other PPDs, the lesions of granulomatous PPD are chronic and have a propensity to erupt on the legs, with the dorsa of the feet being the most common site of involvement. However, involvement of other body sites has also been reported.5,7 Our patient had granulomatous PPD lesions on her back, and is only the third case of granulomatous PPD to have involvement of areas other than the extremities. Intriguingly, 9 of the 17 patients (including ours) with granulomatous PPD had elevated cholesterol levels.2–6 In atherosclerosis, hyperlipidaemia with cholesterol deposition causes vascular inflammation, which is a proposed
ª 2014 British Association of Dermatologists
Granulomatous pigmented purpuric dermatosis L. R. Battle et al.
Table 1 Summary of reported cases of granulomatous pigmented purpuric dermatitis (PPD). Age, years
Sex
1 2 3
61 53 67
F F F
4
57
M
5 6 7
37 22 71
F M F
Right wrist Legs Legs and feet
12 6 60
8
47
M
36
9
48
F
Legs, feet, dorsum of hands Legs and dorsum of feet
Kaplan et al.5
10
60
F
Tato et al.6
11
65
Dyduch et al.7
12
MacQuarrie et al.8 Kerns et al.9 Wakusawa et al.10 Paolino et al.11
Reference Saito and Matsuoka1 Wong et al.2
Lin et al.3
Lee et al.4
Distribution of lesions
Duration, months
Additional histopathological findings*
Dorsum of feet for both Dorsum of feet for both
36 12 240
Giant cells None Thickened capillaries Thickened capillaries and giant cells All had thickened capillaries
Not reported Not reported Present but unspecified Present but unspecified Normal Remainder had elevated total and LDL cholesterol
84
None
Legs, forearms, wrists, lower back
> 180
Lichenoid infiltrate and rare eosinophils
Elevated total cholesterol and triglycerides Elevated total and LDL cholesterol, elevated triglycerides, normal HDL
M
Legs and dorsum of feet
12
Present but unspecified
17
F
Legs, arms, abdomen, dorsum of feet
96
14
53
F
Dorsum of feet
36
Lichenoid infiltrate in focal areas, multinucleate giant cells Dense, lichenoid infiltrate, single lymphocytes migrating into the epidermis, oligoclonal T-cell population Lichenoid infiltrate
13
42
F
Legs
12
15
68
F
Legs
120
Few multinucleated giant cells and rare eosinophils None
16
76
F
Legs, dorsum of feet
48
None
Patient
8
Lipid panel
Other findings/ comorbidities ANA RF and HCV COPD Hypertension – Hypertension ANA DM, hypertension, thrombocytopenia HCV, hyperuricaemia –
Nongranulomatous PPD, microscopic haematuria, obstructive sleep apnoea, hypothyroidism Hypertension
Normal
Early phase cutaneous T-cell lymphoma
Normal
Seizures
Not reported
Ulcerative colitis
Not reported
€gren syndrome Sjo
Normal
Osteoporosis
*All cases of reported granulomatous PPD displayed a superficial lymphohistiocytic to granulomatous infiltrate with extravasated erythrocytes and hemosiderin deposition. Only unusual histopathological findings are listed in the table. ANA, antinuclear antibodies; COPD, chronic obstructive pulmonary disease; DM, diabetes mellitus; GORD, gastro-oesophageal reflux disease; HCV, hepatitis C virus; HDL, high density lipoprotein; LDL, low density lipoprotein; PPD, pigmented purpuric dermatoses; RF, rheumatoid factor.
mechanism of pathogenesis in granulomatous PPD.3,6 Hyperlipidaemia is common in the US population, thus further studies are necessary to determine whether this association plays a role in the pathogenesis. The different clinical variants of PPD demonstrate variable epidermal changes and types of inflammatory infiltrates affecting the superficial papillary dermal vasculature. Presumably, perivascular inflammation leads to vascular damage and erythrocyte extravasation. Granulomatous PPD is unique in that a
ª 2014 British Association of Dermatologists
lymphohistiocytic infiltrate with granuloma formation is seen in the papillary dermis. While the presence of loosely formed granulomas is the characteristic finding of granulomatous PPD, five cases, including the present case, have also had a concomitant band-like lymphohistiocytic infiltrate at the dermoepidermal junction.5–8 Several conditions may need to be considered in the histological differential diagnosis of PPD, particularly the granulomatous variant. Typical and atypical mycobacterial or deep fungal infection must be
Clinical and Experimental Dermatology (2015) 40, pp387–390
389
Granulomatous pigmented purpuric dermatosis L. R. Battle et al.
excluded, although in infectious cases, the lymphohistiocytic infiltrate extends more deeply and granulomas often demonstrate central caseation. Sarcoidosis classically demonstrates tightly formed granulomas without a lichenoid infiltrate. Drug eruptions can show a lymphohistiocytic infiltrate with vague granulomatous zones; the presence of eosinophils and clinicopathological correlation with medication history facilitates the diagnosis, and in our case, resolution of the lesions on the legs without discontinuation of any medication excluded this possibility. Given our patient’s presumed clinical history of LP, this condition was considered, but was excluded because of the absence of hyperkeratosis, wedge-shaped granulosis and prominent dying keratinocytes along the dermoepidermal junction. Lastly, there can be histological overlap between PPD and patch stage cutaneous T-cell lymphoma (CTCL), as demonstrated by case reports of early-stage CTCL presenting as granulomatous PPD.7 Diagnostic clues that favour CTCL over PPD include the presence of large groups of lymphocytes in the upper stratum spinosum, intraepidermal lymphocytes and lymphocyte atypia. Clinical features may also be helpful, with PPD more frequently being located on the legs. Interestingly, T-cell monoclonality has been documented in a subset of patients with PPD without involvement of the legs clinically and a lichenoid infiltrate histologically.5 Moreover, some of those patients are more likely to concomitantly have or subsequently develop CTCL. Therefore, patients with PPDs may require long-term clinical and histological follow-up to monitor for progression to CTCL.
Learning points
•
Granulomatous PPD is similar to other PPDs in clinical appearance but is distinguished histologically by a lymphohistiocytic infiltrate with granuloma formation. • Like other PPDs, granulomatous PPD presents most often on the leg as red to brown macules and papules. • Other causes of granuloma formation such as fungal or mycobacterial infection, sarcoidosis and GA should be excluded. • The presence of a lichenoid infiltrate is sometimes seen in granulomatous PPD, but also raises suspicion for mycosis fungoides. • There seems to be an association between granulomatous PPD and hyperlipidaemia.
390
Clinical and Experimental Dermatology (2015) 40, pp387–390
References 1 Saito R, Matsuoka Y. Granulomatous pigmented purpuric dermatosis. J Dermatol 1996; 23: 551–5. 2 Wong WR, Kuo TT, Chen MJ et al. Granulomatous variant of chronic pigmented purpuric dermatosis: report of two cases. Br J Dermatol 2001; 145: 162–4. 3 Lin WL, Kuo TT, Shih PY et al. Granulomatous variant of chronic pigmented purpuric dermatoses: report of four new cases and an association with hyperlipidaemia. Clin Exp Dermatol 2007; 32: 513–15. 4 Lee SH, Kwon JE, Lee KG et al. Granulomatous variant of chronic pigmented purpuric dermatosis associated with hyperlipidaemia. J Eur Acad Dermatol Venereol 2010; 24: 1243–5. 5 Kaplan J, Burgin S, Sepehr A. Granulomatous pigmented purpura: report of a case and review of the literature. J Cutan Pathol 2011; 38: 984–9. 6 Tato BP, Escobedo SM, Gonz alez YCP et al. Granulomatous variant of pigmented purpuric dermatosis. Am J Dermatopathol 2012; 34: 746–8. 7 Dyduch G, Zuber Z, Turowska-Heydel D et al. Granulomatous pigmented purpura in an adolescent girl: a precursor of mycosis fungoides? Pol J Pathol 2013; 2: 157–9. 8 MacQuarrie EK, Pasternak S, Torok M et al. Persistent pigmented purpuric dermatitis: granulomatous variant. J Cutan Pathol 2011; 38: 979–83. 9 Kerns MJJ, Mallatt BD, Shamma HN. Granulomatous pigmented purpura: an unusual histological variant. Am J Dermatopathol 2009; 31: 77–80. 10 Wakusawa C, Fujimura T, Haga T et al. Granulomatous pigmented purpuric dermatitis associated with primary sj€ ogren’s syndrome. Acta Derm Venereol 2013; 93: 95–6. 11 Paolino S, Cinotti E, Merlo V et al. Progressive petechial and pigmented macules and papules on the lower extremities: challenge and answer. Am J Dermatopathol 2013; 35: 370, 388.
ª 2014 British Association of Dermatologists
Clinical dermatology • Concise report
Clinical and Experimental Dermatology
Granulomatous pigmented purpuric dermatosis L. R. Battle,1 S. C. Shalin2 and L. Gao1 1
Department of Dermatology, and 2Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA
doi:10.1111/ced.12549
Summary
The granulomatous variant of the pigmented purpuric dermatoses (PPDs) is a rare and infrequently described condition, with a total of 16 cases published to date. We report a case of granulomatous PPD in a 59-year-old white woman who demonstrated involvement of the arms, legs, chest and back with concurrent hyperlipidaemia. Histopathological examination revealed a lymphohistiocytic infiltrate obscuring the dermoepidermal junction, and loose granuloma formation in the superficial dermis, with extravasated erythrocytes. Other conditions within the differential diagnosis such as atypical infection, papular sarcoidosis and generalized granuloma annulare were excluded on clinical and histological grounds. Our patient represents the ninth patient reported to have granulomatous PPD with coexisting hyperlipidaemia, and the fifth patient with granulomatous PPD and a lichenoid infiltrate.
The pigmented purpuric dermatoses (PPDs) are a group of chronic skin disorders characterized by a perivascular inflammatory infiltrate affecting papillary dermal capillaries, and presence of extravasated erythrocytes with haemosiderin deposition. Subtypes of PPDs include progressive pigmentary dermatosis (Schamberg disease), purpura annularis telangiectodes (Majocchi purpura), pigmented purpuric lichenoid dermatosis of Gougerot and Blum, eczematid-like purpura of Doucas and Kapetanakis, and lichen aureus (lichen purpuricus).1 One of the less well-characterized PPDs, granulomatous PPD, was first reported in 1996 by Saito and Matsuoka.1 Since that time, a total of 16 cases have appeared in the literature. Originally there was thought to be an association between hepatitis C virus, antinuclear antibodies or rheumatoid factor.1 Currently, there seems to be a positive correlation with hyperlipidaemia and granulomatous PPD, with 9 of the 17 reported patients (including the present case) having elevated cholesterol levels.2–6
Correspondence: Dr Ling Gao, Department of Dermatology, University of Arkansas for Medical Sciences, 4301 W. Markham, Slot 576, Little Rock, AR 72205, USA E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 29 April 2014
ª 2014 British Association of Dermatologists
Report A 59-year-old white woman presented with a 3-week history of a mildly pruritic rash. The rash had begun on her legs and spread to involve her trunk and arms. She had a history of presumed oral lichen planus (LP), which had resolved without treatment. She had been taking the proton pump inhibitor omeprazole for 6 years, but had been changed to dexlansoprazole 3 months prior to presentation. Her only other medication was vitamin D, which she had been taking for 5 months. On physical examination, the patient was found to have numerous, erythematous, nonblanching papules, 1–2 mm in size, distributed diffusely across her trunk and limbs (Fig. 1a,b). The clinical differential diagnosis for the leg lesions included Schamberg disease, while conditions considered for the arms and trunk included generalized granuloma annulare (GA), papular sarcoidosis, LP and drug eruption. Multiple biopsies were taken from the patient’s upper arm and back. Histological evaluation revealed vacuolar change and an infiltrate of lymphocytes that focally obscured the dermoepidermal junction. There was rare dyskeratosis and no epidermotropism. Within the superficial dermis was a vaguely nodular lymphohistiocytic infiltrate without multinucleate cells, suggestive of loosely formed granulomas (Fig. 2). Erythrocyte extravasation was present, with focal hemosiderin deposition. Eosinophils were absent.
Clinical and Experimental Dermatology (2015) 40, pp387–390
387
Granulomatous pigmented purpuric dermatosis L. R. Battle et al.
(a)
(a)
(b) (b)
Figure 2 (a) Superficial lymphohistiocytic infiltrate in the dermis, Figure 1 (a) Pinpoint erythematous papules diffusely spread over
the upper back; (b) pinpoint erythematous, nonblanching papules with a few more confluent-appearing patches over the anterior lower legs.
Laboratory findings included elevations in total cholesterol (273 mg/dL; normal < 200 mg/dL) and lowdensity lipoprotein (LDL) cholesterol (175 mg/dL; < 100 mg/dL). Tests for high-density lipoprotein (HDL) cholesterol, triglycerides, random blood glucose, and aspartate and alanine aminotransferases all gave normal values. Tests for hepatitis A and C viruses and for hepatitis B virus surface antigen were all negative. Although an infectious aetiology was considered, special stains (acid-fast bacilli–Fite, periodic-acid– Schiff) and tissue cultures failed to find any fungal, bacterial or mycobacterial organisms. Taken together, the features were felt to be consistent with the granulomatous variant of PPD. The patient was given Derma-Smoothe/FS (fluocinolone acetonide in a blend of oils containing isopropyl alcohol, isopropyl myristate, light mineral oil, oleth-2, refined peanut oil and fragrances; Hill Dermaceuticals Inc., Sanford, FL, USA) to apply to the pruritic areas. At the 10-month
388
Clinical and Experimental Dermatology (2015) 40, pp387–390
focally obscuring the basement membrane zone. Loosely formed granulomas are present; (b) collections of histiocytes forming loose granulomas, with extravasation of erythrocytes and focal haemosiderin deposition. Haematoxylin and eosin, original magnification (a) 9100; (b) 9200.
follow-up, the lesions on the patient’s lower legs were greatly improved, and the lesions on her arms and trunk showed slight improvement. The granulomatous variant of PPD was first reported in 1996 by Saito and Matsuoa.1 Since then, 16 cases have been reported in the literature. Information on these 16 reported cases of granulomatous PPD is summarized in Table 1. Like other PPDs, the lesions of granulomatous PPD are chronic and have a propensity to erupt on the legs, with the dorsa of the feet being the most common site of involvement. However, involvement of other body sites has also been reported.5,7 Our patient had granulomatous PPD lesions on her back, and is only the third case of granulomatous PPD to have involvement of areas other than the extremities. Intriguingly, 9 of the 17 patients (including ours) with granulomatous PPD had elevated cholesterol levels.2–6 In atherosclerosis, hyperlipidaemia with cholesterol deposition causes vascular inflammation, which is a proposed
ª 2014 British Association of Dermatologists
Granulomatous pigmented purpuric dermatosis L. R. Battle et al.
Table 1 Summary of reported cases of granulomatous pigmented purpuric dermatitis (PPD). Age, years
Sex
1 2 3
61 53 67
F F F
4
57
M
5 6 7
37 22 71
F M F
Right wrist Legs Legs and feet
12 6 60
8
47
M
36
9
48
F
Legs, feet, dorsum of hands Legs and dorsum of feet
Kaplan et al.5
10
60
F
Tato et al.6
11
65
Dyduch et al.7
12
MacQuarrie et al.8 Kerns et al.9 Wakusawa et al.10 Paolino et al.11
Reference Saito and Matsuoka1 Wong et al.2
Lin et al.3
Lee et al.4
Distribution of lesions
Duration, months
Additional histopathological findings*
Dorsum of feet for both Dorsum of feet for both
36 12 240
Giant cells None Thickened capillaries Thickened capillaries and giant cells All had thickened capillaries
Not reported Not reported Present but unspecified Present but unspecified Normal Remainder had elevated total and LDL cholesterol
84
None
Legs, forearms, wrists, lower back
> 180
Lichenoid infiltrate and rare eosinophils
Elevated total cholesterol and triglycerides Elevated total and LDL cholesterol, elevated triglycerides, normal HDL
M
Legs and dorsum of feet
12
Present but unspecified
17
F
Legs, arms, abdomen, dorsum of feet
96
14
53
F
Dorsum of feet
36
Lichenoid infiltrate in focal areas, multinucleate giant cells Dense, lichenoid infiltrate, single lymphocytes migrating into the epidermis, oligoclonal T-cell population Lichenoid infiltrate
13
42
F
Legs
12
15
68
F
Legs
120
Few multinucleated giant cells and rare eosinophils None
16
76
F
Legs, dorsum of feet
48
None
Patient
8
Lipid panel
Other findings/ comorbidities ANA RF and HCV COPD Hypertension – Hypertension ANA DM, hypertension, thrombocytopenia HCV, hyperuricaemia –
Nongranulomatous PPD, microscopic haematuria, obstructive sleep apnoea, hypothyroidism Hypertension
Normal
Early phase cutaneous T-cell lymphoma
Normal
Seizures
Not reported
Ulcerative colitis
Not reported
€gren syndrome Sjo
Normal
Osteoporosis
*All cases of reported granulomatous PPD displayed a superficial lymphohistiocytic to granulomatous infiltrate with extravasated erythrocytes and hemosiderin deposition. Only unusual histopathological findings are listed in the table. ANA, antinuclear antibodies; COPD, chronic obstructive pulmonary disease; DM, diabetes mellitus; GORD, gastro-oesophageal reflux disease; HCV, hepatitis C virus; HDL, high density lipoprotein; LDL, low density lipoprotein; PPD, pigmented purpuric dermatoses; RF, rheumatoid factor.
mechanism of pathogenesis in granulomatous PPD.3,6 Hyperlipidaemia is common in the US population, thus further studies are necessary to determine whether this association plays a role in the pathogenesis. The different clinical variants of PPD demonstrate variable epidermal changes and types of inflammatory infiltrates affecting the superficial papillary dermal vasculature. Presumably, perivascular inflammation leads to vascular damage and erythrocyte extravasation. Granulomatous PPD is unique in that a
ª 2014 British Association of Dermatologists
lymphohistiocytic infiltrate with granuloma formation is seen in the papillary dermis. While the presence of loosely formed granulomas is the characteristic finding of granulomatous PPD, five cases, including the present case, have also had a concomitant band-like lymphohistiocytic infiltrate at the dermoepidermal junction.5–8 Several conditions may need to be considered in the histological differential diagnosis of PPD, particularly the granulomatous variant. Typical and atypical mycobacterial or deep fungal infection must be
Clinical and Experimental Dermatology (2015) 40, pp387–390
389
Granulomatous pigmented purpuric dermatosis L. R. Battle et al.
excluded, although in infectious cases, the lymphohistiocytic infiltrate extends more deeply and granulomas often demonstrate central caseation. Sarcoidosis classically demonstrates tightly formed granulomas without a lichenoid infiltrate. Drug eruptions can show a lymphohistiocytic infiltrate with vague granulomatous zones; the presence of eosinophils and clinicopathological correlation with medication history facilitates the diagnosis, and in our case, resolution of the lesions on the legs without discontinuation of any medication excluded this possibility. Given our patient’s presumed clinical history of LP, this condition was considered, but was excluded because of the absence of hyperkeratosis, wedge-shaped granulosis and prominent dying keratinocytes along the dermoepidermal junction. Lastly, there can be histological overlap between PPD and patch stage cutaneous T-cell lymphoma (CTCL), as demonstrated by case reports of early-stage CTCL presenting as granulomatous PPD.7 Diagnostic clues that favour CTCL over PPD include the presence of large groups of lymphocytes in the upper stratum spinosum, intraepidermal lymphocytes and lymphocyte atypia. Clinical features may also be helpful, with PPD more frequently being located on the legs. Interestingly, T-cell monoclonality has been documented in a subset of patients with PPD without involvement of the legs clinically and a lichenoid infiltrate histologically.5 Moreover, some of those patients are more likely to concomitantly have or subsequently develop CTCL. Therefore, patients with PPDs may require long-term clinical and histological follow-up to monitor for progression to CTCL.
Learning points
•
Granulomatous PPD is similar to other PPDs in clinical appearance but is distinguished histologically by a lymphohistiocytic infiltrate with granuloma formation. • Like other PPDs, granulomatous PPD presents most often on the leg as red to brown macules and papules. • Other causes of granuloma formation such as fungal or mycobacterial infection, sarcoidosis and GA should be excluded. • The presence of a lichenoid infiltrate is sometimes seen in granulomatous PPD, but also raises suspicion for mycosis fungoides. • There seems to be an association between granulomatous PPD and hyperlipidaemia.
390
Clinical and Experimental Dermatology (2015) 40, pp387–390
References 1 Saito R, Matsuoka Y. Granulomatous pigmented purpuric dermatosis. J Dermatol 1996; 23: 551–5. 2 Wong WR, Kuo TT, Chen MJ et al. Granulomatous variant of chronic pigmented purpuric dermatosis: report of two cases. Br J Dermatol 2001; 145: 162–4. 3 Lin WL, Kuo TT, Shih PY et al. Granulomatous variant of chronic pigmented purpuric dermatoses: report of four new cases and an association with hyperlipidaemia. Clin Exp Dermatol 2007; 32: 513–15. 4 Lee SH, Kwon JE, Lee KG et al. Granulomatous variant of chronic pigmented purpuric dermatosis associated with hyperlipidaemia. J Eur Acad Dermatol Venereol 2010; 24: 1243–5. 5 Kaplan J, Burgin S, Sepehr A. Granulomatous pigmented purpura: report of a case and review of the literature. J Cutan Pathol 2011; 38: 984–9. 6 Tato BP, Escobedo SM, Gonz alez YCP et al. Granulomatous variant of pigmented purpuric dermatosis. Am J Dermatopathol 2012; 34: 746–8. 7 Dyduch G, Zuber Z, Turowska-Heydel D et al. Granulomatous pigmented purpura in an adolescent girl: a precursor of mycosis fungoides? Pol J Pathol 2013; 2: 157–9. 8 MacQuarrie EK, Pasternak S, Torok M et al. Persistent pigmented purpuric dermatitis: granulomatous variant. J Cutan Pathol 2011; 38: 979–83. 9 Kerns MJJ, Mallatt BD, Shamma HN. Granulomatous pigmented purpura: an unusual histological variant. Am J Dermatopathol 2009; 31: 77–80. 10 Wakusawa C, Fujimura T, Haga T et al. Granulomatous pigmented purpuric dermatitis associated with primary sj€ ogren’s syndrome. Acta Derm Venereol 2013; 93: 95–6. 11 Paolino S, Cinotti E, Merlo V et al. Progressive petechial and pigmented macules and papules on the lower extremities: challenge and answer. Am J Dermatopathol 2013; 35: 370, 388.
ª 2014 British Association of Dermatologists
