Clinical and Experimental Dermatology (1977) 2, 171.
Clinical meeting of the St John's Hospital Dermatological Society: 3 June 1976
Poikiloderma vasculare atrophicans with vasculitis
ANDREW P. WARIN AND ROBIN A.J. EADY St John's Hospital for Diseases of the Skin, Lisle Street, Leicester Square, London V/C2H 7BJ
Accepted for publication 30 December 1976
History Mr A.P. aged 36, gave a 3 year history of a generalized eruption. At the onset he had had two attacks of an influenza-like illness followed by a purpuric rash on the lower trunk and in the groins. The clinical appearance was of widespread poikiloderma with vasculitis (Fig. i). The vasculitic element responded well to systemic corticosteroids, but there was little response of his poikiloderma. Investigations Histopathology. Pre-reticulotic poikiloderma with early mycosis fungoides in places. Fibrinoid necrosis of papillary vessels in biopsies of vasculitis lesions (Fig. 2). Lymph node: dermatopathic lymphadenopathy. Immunofluorescence. Direct LM.F. of tissue from vascuhtic lesion on lower abdomen showed staining with C3 and fibrin in and around dermal vessels (Fig. 3). Other abnormal results included: Anticomplementary activity present in a dilution of i in 32. Euglobulin lysis time: more than 7 h (control 4 h). Normal or negative results. Blood count and E.S.R.; platelets; prothrombin time; clotting time; liver function tests; immunoglobulins; C^; cryoglobulins; anti-nuclear antibodies; LE cells; rheumatoid factor; C^ esterase inhibitor; plasma viscosity; muscle enzymes; chest X-ray; bone marrow examination; lymphocyte transformation to P.H.A. and P.P.D.; routine urinalysis.
Comment The association between poikiloderma vasculare atrophicans and vascuHtis is very rare. It is not mentioned in any of the standard dermatology or dermatohistopathology text-books. 171
172
A.P,Warin and R.AJ.Eady
Figure i. Clinical appearance before treatment with steroids was started. Typical poikilodermatous changes are present on the neck, trunk, arms and thighs. Several acute vaseulitic lesions ean be seen on the lower abdomen, groins and axillary folds. Tissue necrosis has left marked scarring on the right hip.
Figure 2. Vasculitic lesion on lower abdomen. A marked lympho-histiocytie infiltrate is present in the superficial dermis. Patchy invasion of the epidermis by inflammatory cells is also evident. Some papillary vessels have undergone fibrinoid necrosis (H & E, x 150).
Poikiloderma vasculare atrophicans with vasculitis
173
Figure 3. Direct immunofluorescence staining shows presence of C3 in walls of papillary blood vessels in vaseulitie lesion ( X450).
Samman (1971) states that some elements of vasculitis can often be detected in patches of poikiloderma. He describes the association of pityriasis lichenoides with poikiloderma in seven patients, but in only one case did a 'pityriasis hchenoides-like lesion' show fibrinoid necrosis of small vessels. Although it is possible that this single case of Samman demonstrated the association of vasculitis and poikiloderma, as in our patient, it should be mentioned that fibrinoid necrosis was not a feature seen in a series of 128 cases of pityriasis hchenoides (Marks et al., 1972). We are unaware of any previous reports of poikiloderma vasculare atrophicans being associated with severe vasculitis of the type demonstrated in this patient.
References MARKS, R., BLACK, M . M .
& WILSON
JONES, E .
(1972) Pityriasis lichenoides; a reappraisal. British Journal
of Dermatology, %•], 106-1:3.
P.D. (1971) Poikiloderma with pityriasis liehenoides. Transactions of the St John's Hospital Dermatological Society, 57, 143-147.
SAMMAN,
Clinical meeting of the St John's Hospital Dermatological Society: 3 June 1976
Poikiloderma vasculare atrophicans with vasculitis
ANDREW P. WARIN AND ROBIN A.J. EADY St John's Hospital for Diseases of the Skin, Lisle Street, Leicester Square, London V/C2H 7BJ
Accepted for publication 30 December 1976
History Mr A.P. aged 36, gave a 3 year history of a generalized eruption. At the onset he had had two attacks of an influenza-like illness followed by a purpuric rash on the lower trunk and in the groins. The clinical appearance was of widespread poikiloderma with vasculitis (Fig. i). The vasculitic element responded well to systemic corticosteroids, but there was little response of his poikiloderma. Investigations Histopathology. Pre-reticulotic poikiloderma with early mycosis fungoides in places. Fibrinoid necrosis of papillary vessels in biopsies of vasculitis lesions (Fig. 2). Lymph node: dermatopathic lymphadenopathy. Immunofluorescence. Direct LM.F. of tissue from vascuhtic lesion on lower abdomen showed staining with C3 and fibrin in and around dermal vessels (Fig. 3). Other abnormal results included: Anticomplementary activity present in a dilution of i in 32. Euglobulin lysis time: more than 7 h (control 4 h). Normal or negative results. Blood count and E.S.R.; platelets; prothrombin time; clotting time; liver function tests; immunoglobulins; C^; cryoglobulins; anti-nuclear antibodies; LE cells; rheumatoid factor; C^ esterase inhibitor; plasma viscosity; muscle enzymes; chest X-ray; bone marrow examination; lymphocyte transformation to P.H.A. and P.P.D.; routine urinalysis.
Comment The association between poikiloderma vasculare atrophicans and vascuHtis is very rare. It is not mentioned in any of the standard dermatology or dermatohistopathology text-books. 171
172
A.P,Warin and R.AJ.Eady
Figure i. Clinical appearance before treatment with steroids was started. Typical poikilodermatous changes are present on the neck, trunk, arms and thighs. Several acute vaseulitic lesions ean be seen on the lower abdomen, groins and axillary folds. Tissue necrosis has left marked scarring on the right hip.
Figure 2. Vasculitic lesion on lower abdomen. A marked lympho-histiocytie infiltrate is present in the superficial dermis. Patchy invasion of the epidermis by inflammatory cells is also evident. Some papillary vessels have undergone fibrinoid necrosis (H & E, x 150).
Poikiloderma vasculare atrophicans with vasculitis
173
Figure 3. Direct immunofluorescence staining shows presence of C3 in walls of papillary blood vessels in vaseulitie lesion ( X450).
Samman (1971) states that some elements of vasculitis can often be detected in patches of poikiloderma. He describes the association of pityriasis lichenoides with poikiloderma in seven patients, but in only one case did a 'pityriasis hchenoides-like lesion' show fibrinoid necrosis of small vessels. Although it is possible that this single case of Samman demonstrated the association of vasculitis and poikiloderma, as in our patient, it should be mentioned that fibrinoid necrosis was not a feature seen in a series of 128 cases of pityriasis hchenoides (Marks et al., 1972). We are unaware of any previous reports of poikiloderma vasculare atrophicans being associated with severe vasculitis of the type demonstrated in this patient.
References MARKS, R., BLACK, M . M .
& WILSON
JONES, E .
(1972) Pityriasis lichenoides; a reappraisal. British Journal
of Dermatology, %•], 106-1:3.
P.D. (1971) Poikiloderma with pityriasis liehenoides. Transactions of the St John's Hospital Dermatological Society, 57, 143-147.
SAMMAN,
