42
Case report.^
(i2) Acrokerato-elastoidosis in a Somerset mother and her two sons DR C.N.A.MATTHEWS FOR DR R.R.M.HARMAN Bristol Royal Infirmary
C.S., female, aged 36 years. Hospital case no. 47 02 83. History. Abnormal thickening of the skin of the palms and soles was first noted at the age of two. This gradually increased in severity and hss never improved. It has been static for many years. She also gets red, scaly patches on the face in the summer and in windy weather. She also has had small patches of eczema at the finger tips at times. Family history. Two of the patient's three sons have a similar abnormahty of the palms and soles but as yet the lesions are less well developed.
CS.
i D.S.
R.S.
FIGURE I. Acrokerato-elastoidosis in a Somerset mother and her two sons. • , Affected female; • , affected male; • , unaffected temale; i, unaffected male.
Examination. There is a palmo-plantar keratoderma with isolated hyperkeratotic papules on the backs of the fingers and on the lateral margins of the hands, like those described in acrokeratoelastoidosis. Investigations. Histology. The epidermis is a little acanthotic and subjectively shows an increase in thickness and granularity of the granular layer. The granular-keratin junction is irregular and the grossly thickened stratum corneum is regular and homogenous, but may show more 'ghost' keratohyaline granules than expected. The dermis and its elastic content is normal.
D.S., male, aged 13 years. Weston-super-Mare. Hospital case no. 397. History. This patient first presented at age 11 years with minor red scaly patches on the face and arms. These cleared with local steroids. He was next seen at the age of 11 with raised red-yellow papules which had recently developed on the palms and soles. There was also some redness of the legs and a history of red patches on the face on exposure to wind. Examination then showed early lesions on the hands and feet very similar to those of his mother (C.S.). He also had eczema. Examination. Palmo-piantar keratoderma with knuckle pad-like lesions. Histology. The dermis and lower epidermis are normal, there is a little acanthosis and perhaps, as in
Case reports
43
; 2. Acrokerato-eiastoidosis, showing the well-dcfincd edge of the plantar kcratoderma.
the mother's case, some hypergranularity of the stratum granulosum with marked regular hyperkeratosis. R.S.) male, aged io years. Weston-super-Mare. Hospital case no. 60 39 3. History. Approximately I year ago lesions on his palms and soles appeared very like those of his mother (C.S.) and brother (D.S.). He had also developed some knuckle pad-like lesions on the fingers. Examination. Palmo-plantar keratoderma with knuckle pad-like lesions on several fingers. Follicular spiky lesions on legs and thighs. Investigations. Trichophyton interdigitale was isolated from his sole.
Comment. Do this Somerset born and bred family have acrokerato-eiastoidosis as described by Costa (1953)? REFEREN'CES COSTA, O.Ci. (1953) ALTokeraro-elastoidosis—a hitherto undescribed skin diseaso. Dermatologica, 170, 164. JUNG, E.G. (1973) Acrokerato-eiastoidosis. Humangenetik, 17, 357. JUNG, E . G . , BI;IL, F.U., ANTON LAMPRECHT, I., GRHTEN, H . & NEMETSCKHIK, T H . (1974) Acrokerato-eiastoidosis.
Der Hautarjt, 25, 127. MATTHEWS, C.N.A. (for HARMAN, R . R . M . ) (1974) Acrokerato-eiastoidosis (without elastorrhexis). Proceedings of
the Royal Society of Medicine, 67, 1237.
Case report.^
(i2) Acrokerato-elastoidosis in a Somerset mother and her two sons DR C.N.A.MATTHEWS FOR DR R.R.M.HARMAN Bristol Royal Infirmary
C.S., female, aged 36 years. Hospital case no. 47 02 83. History. Abnormal thickening of the skin of the palms and soles was first noted at the age of two. This gradually increased in severity and hss never improved. It has been static for many years. She also gets red, scaly patches on the face in the summer and in windy weather. She also has had small patches of eczema at the finger tips at times. Family history. Two of the patient's three sons have a similar abnormahty of the palms and soles but as yet the lesions are less well developed.
CS.
i D.S.
R.S.
FIGURE I. Acrokerato-elastoidosis in a Somerset mother and her two sons. • , Affected female; • , affected male; • , unaffected temale; i, unaffected male.
Examination. There is a palmo-plantar keratoderma with isolated hyperkeratotic papules on the backs of the fingers and on the lateral margins of the hands, like those described in acrokeratoelastoidosis. Investigations. Histology. The epidermis is a little acanthotic and subjectively shows an increase in thickness and granularity of the granular layer. The granular-keratin junction is irregular and the grossly thickened stratum corneum is regular and homogenous, but may show more 'ghost' keratohyaline granules than expected. The dermis and its elastic content is normal.
D.S., male, aged 13 years. Weston-super-Mare. Hospital case no. 397. History. This patient first presented at age 11 years with minor red scaly patches on the face and arms. These cleared with local steroids. He was next seen at the age of 11 with raised red-yellow papules which had recently developed on the palms and soles. There was also some redness of the legs and a history of red patches on the face on exposure to wind. Examination then showed early lesions on the hands and feet very similar to those of his mother (C.S.). He also had eczema. Examination. Palmo-piantar keratoderma with knuckle pad-like lesions. Histology. The dermis and lower epidermis are normal, there is a little acanthosis and perhaps, as in
Case reports
43
; 2. Acrokerato-eiastoidosis, showing the well-dcfincd edge of the plantar kcratoderma.
the mother's case, some hypergranularity of the stratum granulosum with marked regular hyperkeratosis. R.S.) male, aged io years. Weston-super-Mare. Hospital case no. 60 39 3. History. Approximately I year ago lesions on his palms and soles appeared very like those of his mother (C.S.) and brother (D.S.). He had also developed some knuckle pad-like lesions on the fingers. Examination. Palmo-plantar keratoderma with knuckle pad-like lesions on several fingers. Follicular spiky lesions on legs and thighs. Investigations. Trichophyton interdigitale was isolated from his sole.
Comment. Do this Somerset born and bred family have acrokerato-eiastoidosis as described by Costa (1953)? REFEREN'CES COSTA, O.Ci. (1953) ALTokeraro-elastoidosis—a hitherto undescribed skin diseaso. Dermatologica, 170, 164. JUNG, E.G. (1973) Acrokerato-eiastoidosis. Humangenetik, 17, 357. JUNG, E . G . , BI;IL, F.U., ANTON LAMPRECHT, I., GRHTEN, H . & NEMETSCKHIK, T H . (1974) Acrokerato-eiastoidosis.
Der Hautarjt, 25, 127. MATTHEWS, C.N.A. (for HARMAN, R . R . M . ) (1974) Acrokerato-eiastoidosis (without elastorrhexis). Proceedings of
the Royal Society of Medicine, 67, 1237.
