north british dermatological society

3
332 NORTH BRITISH DERMATOLOGICAL SOCIETY Histology.—(i) Of a fresh lesion: showed a dense inflammatory inflltrate of poly- morphs, plasma cells and monocytes in the dermis, with numerous eosinophils. (ii) Of an older lesion : showed that the stratum corneum was covered by fibrin and debris which tended to be heaped up. Clefts had appeared between the prickle cells and the basal layer of the epidermis, forming a vesicle, containing numerous inflam- matory cells—polymorphs, lymphocytes and plasma cells, while the displaced basal laj'er and dermal papillae also contained eosinophils, in addition to the inflammatory exudate. Treatmeni and progress.—On more than one occasion she was in extremis and blood transfusions were required on four occasions and mild cardiac failure due to steroid therapy responded to digoxin. The turning point in her illness seems to have been associated with the regular administration of pooled gammaglobulin 400 mg. twice weekly. Prednisone also seemed to influence the disorder in two ways, by inhibiting the evolution of fresh lesions and by accelerating healing. DisctrssiON. Dr. P. D. SAMMAN : Two or three years ago I saw a very similar case and, thinking that it was a severe herpes simplex primary infection, recommended treatment with gammaglobuhn. I did not see the patient again but was informed that complete recovery took place quite quickly. Attempts were made to demonstrate a virus but were not successful. The following cases have been published in Proc. R. Soc. Med., 58, 423. Dermatomyositis. —Dr. D. I. WILLIAMS. Thyroid Acropachy, Circumscribed Myxoedema and Exophthalmos. Dr. D. SHAEVILL. The following cases were also sho^vn. Lupus Erythematosus.—Dr. E. L. RHODES (for Dr. R. H. MAKTEN). Case for Diagnosis—Telangiectatic Erythema of the Face.—Dr. R. H. MAETEN. Dermatomyositis.—Dr. J. WABNEB (for D. I. WILLIAMS). Muttiple Cutaneous Nodules.—Dr. J. WABNEE (for Dr. R. H. MABTEN). Mycosis Fungoides with Dermatitis Herpetiformis or Pemphigoid. —Dr. M. L. JoHNSOK (for Dr. P. J. HARE). Mycosis Fungoides. —Dr. K. V. SANDEBSON. Kaposi's Haemorrhagic Sarcoma treated with Intra-Arteriat Mustine. Dr. F. BOB (for Dr. M. FEIWEL). Infiltrated Plaques of the Scalp. —Dr. A. BOWYEB (for Dr. Louis FOBMAN). Epidermolysis BuUosa Dystrophica (Hurlitz).—Dr. E. J. MOYNAHAN. Multiple Pyogenic Granulomata. —Dr. I. SAEKANY. NOETH BRITISH DERMATOLOGICAL SOCIETY. Edinburgh, 10 December 1964. Lipoid Proteinosis (Hyalinosis Cutis et Mucosae). —Dr. G. A. GRANT PETEB- KIN. A girl aged 19 years. History.—She had bronchitis at the age of six months ; since then her voice has been hoarse. Gradually her tongue became too big for her mouth and she was subject to oral bullae or ulcers. Purulent blisters appeared on her skin, leaving pitted scars (for example on the brow and the right elbow) and nodules appeared on the eyelids, at the angles of the mouth and at the aural meatuses. She has suffered from epilepsy and " rheumatic joints " and some years ago she had a parotitis. On examination.—She has a swollen, flxed tongue with some translucent nodules

Post on 15-Jul-2016

213 views

Category:

Documents


1 download

TRANSCRIPT

332 NORTH BRITISH DERMATOLOGICAL SOCIETY

Histology.—(i) Of a fresh lesion: showed a dense inflammatory inflltrate of poly-morphs, plasma cells and monocytes in the dermis, with numerous eosinophils.(ii) Of an older lesion : showed that the stratum corneum was covered by fibrin anddebris which tended to be heaped up. Clefts had appeared between the prickle cellsand the basal layer of the epidermis, forming a vesicle, containing numerous inflam-matory cells—polymorphs, lymphocytes and plasma cells, while the displaced basallaj'er and dermal papillae also contained eosinophils, in addition to the inflammatoryexudate.

Treatmeni and progress.—On more than one occasion she was in extremis and bloodtransfusions were required on four occasions and mild cardiac failure due to steroidtherapy responded to digoxin. The turning point in her illness seems to have beenassociated with the regular administration of pooled gammaglobulin 400 mg. twiceweekly. Prednisone also seemed to influence the disorder in two ways, by inhibitingthe evolution of fresh lesions and by accelerating healing.

DisctrssiON.Dr. P. D. SAMMAN : Two or three years ago I saw a very similar case and, thinking that it was a

severe herpes simplex primary infection, recommended treatment with gammaglobuhn. I did notsee the patient again but was informed that complete recovery took place quite quickly. Attemptswere made to demonstrate a virus but were not successful.

The following cases have been published in Proc. R. Soc. Med., 58, 423.Dermatomyositis.—Dr. D. I. WILLIAMS.

Thyroid Acropachy, Circumscribed Myxoedema and Exophthalmos.—Dr. D. SHAEVILL.

The following cases were also sho^vn.Lupus Erythematosus.—Dr. E. L. RHODES (for Dr. R. H. MAKTEN).Case for Diagnosis—Telangiectatic Erythema of the Face.—Dr. R. H.

MAETEN.Dermatomyositis.—Dr. J. WABNEB (for D. I. WILLIAMS).Muttiple Cutaneous Nodules.—Dr. J. WABNEE (for Dr. R. H. MABTEN).Mycosis Fungoides with Dermatitis Herpetiformis or Pemphigoid.—Dr.

M. L. JoHNSOK (for Dr. P. J. HARE).Mycosis Fungoides.—Dr. K. V. SANDEBSON.Kaposi's Haemorrhagic Sarcoma treated with Intra-Arteriat Mustine.—

Dr. F. BOB (for Dr. M. FEIWEL).Infiltrated Plaques of the Scalp.—Dr. A. BOWYEB (for Dr. Louis FOBMAN).Epidermolysis BuUosa Dystrophica (Hurlitz).—Dr. E. J. MOYNAHAN.Multiple Pyogenic Granulomata.—Dr. I. SAEKANY.

NOETH BRITISH DERMATOLOGICAL SOCIETY.Edinburgh, 10 December 1964.

Lipoid Proteinosis (Hyalinosis Cutis et Mucosae).—Dr. G. A. GRANT PETEB-KIN.

A girl aged 19 years.History.—She had bronchitis at the age of six months ; since then her voice has

been hoarse. Gradually her tongue became too big for her mouth and she was subjectto oral bullae or ulcers. Purulent blisters appeared on her skin, leaving pitted scars(for example on the brow and the right elbow) and nodules appeared on the eyelids,at the angles of the mouth and at the aural meatuses. She has suffered from epilepsyand " rheumatic joints " and some years ago she had a parotitis.

On examination.—She has a swollen, flxed tongue with some translucent nodules

NORTH BRITISH DERMATOLOGICAL SOCIETY 333

on the oral mucosae, some scamng, but no ulcers. On the eyelids, at the angles ofthe mouth and at the entrance to the aural meatuses were small grouped skin-colouredpapules and nodules. Hyperkeratosis and some nodules were found on the extensoraspect of the elbows and knees.

Investigations.—Histology of a nodule showed the characteristic features of lipoidproteinosis. Skiagram and tomograms of skull—calcification in the hippocampalgyrus of the type seen in lipoid proteinosis.

Malabsorption Syndrome with Preceding Cutaneous Manifestations.—Dr. G. A. GRANT PETEEKIN.

A man aged 30.History.—He was first seen in 1959 when he gave a history of asthma since childhood

and eczema for the previous two years. At that time he had typical Besnier'sprurigo, involving the face and the antecubital and popliteal fossae. In 1960 hereported with crusted and bullous lesions in addition to the eczema. The diagnosis ofdermatitis herpetiformis was considered and a biopsy showed a subepidermal buUa.Sulphapyridine 0-5 g. twice daily controlled the formation of bullae, but the eczemarelapsed when it was stopped. In 1961 he had a severe relapse of his Besnier's prurigowith no lesions of dermatitis herpetiformis ; this was treated with oral betamethasone.He kept well until September 1962, when the eczema and bullae again recurred butwere controlled by sulphapyridine. About this time his tonsils were removed and nofurther bullae have since appeared although he has had no more suppressive therapy.During the past year the appearance of his eruption has altered completely, showingthe features of a nodular lichen planus, mainly affecting the limbs and associated with adiffuse alopecia and brown pigmentation, mainly on the trunk. During the past fewmonths he has lost about two stones in weight and had diarrhoea for a short time.On treatment with a diluted betamethasone valerate ointment the lichenoid eruptionsoon vanished.

Investigations.—Hh 67%, M.C.H.C. 33%, W.B.C. 5400 per cu. mm., E.S.R. 10mm/hr. Film : The red cell series showed a dimorphic picture and the neutrophilsa shift to the left. Total stool fat (5 day collection) 40 g. Serum B12 assay 124/x/ig./ml. PIGLU positive. Serum iron 60 /xg./lOO ml. Maximum histamine test—14 m. eq. in post-histamine hour.

Further investigation has established that he is suffering from primary malabsorp-tion disease. He is being treated with a gluten free diet and supplements of vitamins,calcium and iron.

Lipogranulomatosis Subcutanea of Rothman and Makai treated withHydroxychloroquine Sulphate.—Dr. P. W. HANNAY.

A woman aged 58.History.—Since gastrectomy in 1961 she has had a mild iron deficiency anaemia ;

otherwise she has been in good health. She developed subcutaneous nodules on herlegs in August 1964 . They continued to appear singly on her legs and later on herforearms ; the lesions were erythematous and only slightly tender on deep pressure,their size varying from that of a hazel nut to that of a walnut. There was no systemicupset or pyrexia. All investigations gave normal findings apart from the histology(from a freshly erupted nodule) which showed a marked panniculitis associated withmany foreign body giant cells.

Treatment and comment.—She was given hydroxychloroquine sulphate 600 mg.daily, reduced to 400 mg. after a fortnight. Within three weeks the eruption offresh nodules had ceased and all lesions were subsiding, leaving wide areas of brownpigmentation. No new nodules had appeared eight weeks after starting treatment.It is impossible to be certain that this response was not a natural remission but thetime relationship was striking.