British Journal of Dermatology (1977J 97, 323.
Case Reports
Generalized eczema craquele as a presenting feature of lymphoma D.J.BARKER AND J.A.COTTERILL Department of Dermatology, University of Leeds, The General Infirmary at Leeds Accepted for publication 18 March 1977
SUMMARY
Two cases of lymphoma are reported—in both, extensive eczema craquele resistant to treatment was a presenting feature.
Although eczema craquele is a common condition, there are very little data on its incidence, pathogenesis or treatment. Recently Warin (1973) discussed the history ofthe disorder and described three patients with advanced myxoedema who presented in this way. Whilst it is well recognized that acquired ichthyosis may be associated with Hodgkin's disease (StevanoviCj i960), the combination of lymphoma with eczema craquele has not been reported before. CASE REPORTS Case I
A 69'year-old man presented with a month's history of anorexia, lassitude and a generalized itchy, dry and scaly skin. The rash had not responded to treatment with oil baths and was getting rapidly more extensive. There was no previous history of skin disease except for 'industrial dermatitis' many years previously. However, he gave a 12-year history of eoeliac disease treated with a glutenfree diet to which, he stated, he adhered strictly. On examination he had almost generalized classical eczema craquele involving shins, thighs, shoulders and back (Figs i & 2). Discrete lymphadenopathy was detected in the right axillaj but there was no enlargement ofthe hver or spleen. Investigation revealed Hb 124 G/dl with a total white count of 9 6 x io''/l and a persisting eosinophiha of 25-35",,. Renal and liver function tests were initially normal. There was evidence of continuing malabsorption in that faecal fat excretion was raised; and only 13% of an oral xylose dose was excreted by 5 h. There was, however, no evidence of essential fatty acid malabsorption. Chest X-ray showed a left basal effusion with enlarged superior mediastinal glands. The patient was transferred to the Thoracic Medical Department (Dr Gordon Edwards). Sputum cytology and pleural biopsy were negative, but histological examination of a supraclavicular node revealed lymphoma, probably of Hodgkin's type. During his admission the patient received conventional treatment for his eczema. Only frequent and liberal applications of betamethasone valerate 325
324
D.J.Barker andJ.A.Cotierill
I. Eczema i;raquclc on the back of Case i,
ointment 25",, in emulsifying ointment were of any help, but even so, improvement was only partial and transiem. Sunflower oil applied topically had no beneficial effect on the skin. After a period of chemotherapy the patient died. Post mortem examination confirmed the diagnosis of Hodgkin's disease. Case 2
A 65-year-old man presented with a 3-week history of anorexia, malaise and a generalized dry, scaly.
FIGURE 2. Eczema craqueU in Case i demonstrating purpuric fissures.
Generalized eczema craquele
325
itchy rash. There was no previous history of skin disease, or any other medical problems except for a mild depressive illness. On examination he was ill and febrile with marked eczema craquele over thighs, shins and abdomen. Small, firm lymph nodes were present in both supraclavicular fossae. There was no enlargement of liver or spleen. Investigation revealed a persisting anaemia with an ESR of 135 mm/h. Hepatic enzymes were elevated (alkaline phosphatase 23-1; SGOT 24), There was biochemical evidence of hypothyroidism (Total thyroxine 50 nmol/1; free thyroxine index 0-95) but the patient was clinically euthyroid. Right axillary lymph node biopsy showed Hodgkin's disease (Dr C.K. Anderson). The patient was referred for chemotherapy, but his condition rapidly deteriorated. Autopsy confirmed widespread Hodgkin's disease of the lymphocyte depleted type. During the last few weeks of his life this patient's dermatosis persisted despite treatment with dilute topical corticosteroid ointments. DISCUSSION
The irregular, reticulate, dry fissured appearance of eczema craquele is a common sight localized mainly to the shins of elderly hospital in-patients. The dcgreasing effect on the skin of regular and methodical washing, the dry, starched sheets and low environmental humidity are probably all, in part, responsible. In a personal study of all admissions to an orthopaedic unit during a 6-month period the authors found that 20",, of patients ultimately developed a pattern of pronounced scaling and fissuring ofthe lower legs and feet. In those confined to bed for more than 2 weeks with skeletal injuries the number developing these changes was far higher, nearly 70" „, Only three ofthe 130 patients followed developed frank eczema craquele, localized to shins and thighs, all within the first month following admission. These, and all other patients affected and seen by the authors during the preceding year were over 50 years of age. This may, however, refiect only the age distribution ofthe hospital in-patient population. None of these patients was clinically hypothyroid and all responded promptly to treatment with a corticosteroid ointment diluted in emulsifying ointment. The skin changes seen in the two patients reported here were far more extensive than is usually seen in the common form of eczema craquele described above. Moreover, the patients differed in their refractory response to treatment which is usually effective. The pathogenesis of the eruption remains an enigma. Both our patients were clinically euthyroid although the second case had some biochemical evidence of hypothyroidism. Chronic malabsorption has recently been associated with essential fatty acid deficiency (E.F.A.) and a scaly dermatitis (Prottey, Hartop & Press, 1975). There was no evidence of E.F.A. malabsorption in the first case, or response to topical sunflower oil, rich in linoleic acid. Murray & Forsey {1975) described a patient with a marked reduction in sweating and eczema craquele, in an area of traumatic denervation. There was no obvious clinical evidence of peripheral neuropathy in our patients but we did not attempt to evaluate sweat gland function. In summary, the rapid onset of extensive eczema craquele, coupled with resistance to treatment, should alert the clinician to the possibility of an underlying lymphoma. This form of eczema craquele should not be confused with the localized dermatosis commonly seen in elderly and often bedridden hospital in-patients. ACKNOWLEDGMENTS We would like to thank Dr Gordon Edwards and Dr Adrian Simmons for permission to publish
326
D.J.Barker and J.A.Cotterill
details of their investigations, Dr C.Prottey for performing estimations of essential fatty acids, and Mrs V.M.Dickinson for typing the manuscript.
REFERENCES MURRAY, H.E. & FORSEY, R.R. (1975) Eczema craquele. Archives of Dermatology., 3, 1536. PROTTEY, C , HARTOP, P.J. & PRESS, M . (1975) Correction of the cutaneous manifestations of fatty acid deficiency in man by application of sunflower-seed oil. Journal of Invesiigaiive Dermaiobgy, 64, 228. STEVANOVIC, D . V . (i960) Hodgkin's disease of skin; Acquired ichthyosis preceding tumoral and ulcerating lesions for seven years. Archives of Dermatology, 82, 96. WARIN, A. (1973) Eczema craquele as the presenting feature of myxoedema. British Journal of Dermatology, 89, 289.
Case Reports
Generalized eczema craquele as a presenting feature of lymphoma D.J.BARKER AND J.A.COTTERILL Department of Dermatology, University of Leeds, The General Infirmary at Leeds Accepted for publication 18 March 1977
SUMMARY
Two cases of lymphoma are reported—in both, extensive eczema craquele resistant to treatment was a presenting feature.
Although eczema craquele is a common condition, there are very little data on its incidence, pathogenesis or treatment. Recently Warin (1973) discussed the history ofthe disorder and described three patients with advanced myxoedema who presented in this way. Whilst it is well recognized that acquired ichthyosis may be associated with Hodgkin's disease (StevanoviCj i960), the combination of lymphoma with eczema craquele has not been reported before. CASE REPORTS Case I
A 69'year-old man presented with a month's history of anorexia, lassitude and a generalized itchy, dry and scaly skin. The rash had not responded to treatment with oil baths and was getting rapidly more extensive. There was no previous history of skin disease except for 'industrial dermatitis' many years previously. However, he gave a 12-year history of eoeliac disease treated with a glutenfree diet to which, he stated, he adhered strictly. On examination he had almost generalized classical eczema craquele involving shins, thighs, shoulders and back (Figs i & 2). Discrete lymphadenopathy was detected in the right axillaj but there was no enlargement ofthe hver or spleen. Investigation revealed Hb 124 G/dl with a total white count of 9 6 x io''/l and a persisting eosinophiha of 25-35",,. Renal and liver function tests were initially normal. There was evidence of continuing malabsorption in that faecal fat excretion was raised; and only 13% of an oral xylose dose was excreted by 5 h. There was, however, no evidence of essential fatty acid malabsorption. Chest X-ray showed a left basal effusion with enlarged superior mediastinal glands. The patient was transferred to the Thoracic Medical Department (Dr Gordon Edwards). Sputum cytology and pleural biopsy were negative, but histological examination of a supraclavicular node revealed lymphoma, probably of Hodgkin's type. During his admission the patient received conventional treatment for his eczema. Only frequent and liberal applications of betamethasone valerate 325
324
D.J.Barker andJ.A.Cotierill
I. Eczema i;raquclc on the back of Case i,
ointment 25",, in emulsifying ointment were of any help, but even so, improvement was only partial and transiem. Sunflower oil applied topically had no beneficial effect on the skin. After a period of chemotherapy the patient died. Post mortem examination confirmed the diagnosis of Hodgkin's disease. Case 2
A 65-year-old man presented with a 3-week history of anorexia, malaise and a generalized dry, scaly.
FIGURE 2. Eczema craqueU in Case i demonstrating purpuric fissures.
Generalized eczema craquele
325
itchy rash. There was no previous history of skin disease, or any other medical problems except for a mild depressive illness. On examination he was ill and febrile with marked eczema craquele over thighs, shins and abdomen. Small, firm lymph nodes were present in both supraclavicular fossae. There was no enlargement of liver or spleen. Investigation revealed a persisting anaemia with an ESR of 135 mm/h. Hepatic enzymes were elevated (alkaline phosphatase 23-1; SGOT 24), There was biochemical evidence of hypothyroidism (Total thyroxine 50 nmol/1; free thyroxine index 0-95) but the patient was clinically euthyroid. Right axillary lymph node biopsy showed Hodgkin's disease (Dr C.K. Anderson). The patient was referred for chemotherapy, but his condition rapidly deteriorated. Autopsy confirmed widespread Hodgkin's disease of the lymphocyte depleted type. During the last few weeks of his life this patient's dermatosis persisted despite treatment with dilute topical corticosteroid ointments. DISCUSSION
The irregular, reticulate, dry fissured appearance of eczema craquele is a common sight localized mainly to the shins of elderly hospital in-patients. The dcgreasing effect on the skin of regular and methodical washing, the dry, starched sheets and low environmental humidity are probably all, in part, responsible. In a personal study of all admissions to an orthopaedic unit during a 6-month period the authors found that 20",, of patients ultimately developed a pattern of pronounced scaling and fissuring ofthe lower legs and feet. In those confined to bed for more than 2 weeks with skeletal injuries the number developing these changes was far higher, nearly 70" „, Only three ofthe 130 patients followed developed frank eczema craquele, localized to shins and thighs, all within the first month following admission. These, and all other patients affected and seen by the authors during the preceding year were over 50 years of age. This may, however, refiect only the age distribution ofthe hospital in-patient population. None of these patients was clinically hypothyroid and all responded promptly to treatment with a corticosteroid ointment diluted in emulsifying ointment. The skin changes seen in the two patients reported here were far more extensive than is usually seen in the common form of eczema craquele described above. Moreover, the patients differed in their refractory response to treatment which is usually effective. The pathogenesis of the eruption remains an enigma. Both our patients were clinically euthyroid although the second case had some biochemical evidence of hypothyroidism. Chronic malabsorption has recently been associated with essential fatty acid deficiency (E.F.A.) and a scaly dermatitis (Prottey, Hartop & Press, 1975). There was no evidence of E.F.A. malabsorption in the first case, or response to topical sunflower oil, rich in linoleic acid. Murray & Forsey {1975) described a patient with a marked reduction in sweating and eczema craquele, in an area of traumatic denervation. There was no obvious clinical evidence of peripheral neuropathy in our patients but we did not attempt to evaluate sweat gland function. In summary, the rapid onset of extensive eczema craquele, coupled with resistance to treatment, should alert the clinician to the possibility of an underlying lymphoma. This form of eczema craquele should not be confused with the localized dermatosis commonly seen in elderly and often bedridden hospital in-patients. ACKNOWLEDGMENTS We would like to thank Dr Gordon Edwards and Dr Adrian Simmons for permission to publish
326
D.J.Barker and J.A.Cotterill
details of their investigations, Dr C.Prottey for performing estimations of essential fatty acids, and Mrs V.M.Dickinson for typing the manuscript.
REFERENCES MURRAY, H.E. & FORSEY, R.R. (1975) Eczema craquele. Archives of Dermatology., 3, 1536. PROTTEY, C , HARTOP, P.J. & PRESS, M . (1975) Correction of the cutaneous manifestations of fatty acid deficiency in man by application of sunflower-seed oil. Journal of Invesiigaiive Dermaiobgy, 64, 228. STEVANOVIC, D . V . (i960) Hodgkin's disease of skin; Acquired ichthyosis preceding tumoral and ulcerating lesions for seven years. Archives of Dermatology, 82, 96. WARIN, A. (1973) Eczema craquele as the presenting feature of myxoedema. British Journal of Dermatology, 89, 289.
