The histological features of 149 trichilemmal cysts removed from sixty-five patients are reviewed and related to the clinical findings. These cysts, which may be solitary or multiple, gradually increase in size and number, and may produce daughter cysts by budding. When a breach occurs in the wall, inflammatory cells pour in but do not replace the cyst wall. This event may be followed by healing from the margins of the breach, by marsupialization to the overlying epidermis and thus natural resolution, or by proliferation to produce a pseudo-epitheliomatous change which can be confused with a well differentiated squamous cell carcinoma.

SUMMARY On the basis of clinical, histological and electron microscopical findings, we are proposing that mycosis fungoides can begin in the epidermis. Early involvement of the basal layer of the epidermis is emphasized by the pigmentary changes which herald the onset of about half the cases. The formation of new fibrous tissue above the subepidermal elastic garland and the presence of PAS‐positive globules above the epidermal basement membrane, support the view that the primary change is on the epidermal side of the elastic garland and basement membrane. The predominantly epidermal distribution of the mycosis cell in many early cases suggests that it stems from a cell normally present in the epidermis. The likeliest candidate appears to be the Langerhans cell. A derivation from this cell would explain many puzzling features of mycosis fungoides and in particular the affinity of the mycosis cell for the epidermis.

Summary One hundred and fifteen individuals from 60 families are described who had trichilemmal cysts. These were solitary or multiple intradermal cysts and occurred most frequently on the scalp. A punctum leading from the skin surface to the cyst was not seen in any patient. Because they are encapsulated they are easily removed. Histologicaily they resemble the external root sheath in the region of the follicular isthmus and are quite different from epidermoid cysts. They are not associated with Gardner's syndrome or any other diseases. In 46 of the 60 families an autosomal dominant mode of inheritance was demonstrated.

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Cutaneous angiomata of the "port wine" variety are frequently associated with angiomata elsewhere in the body and particularly in the central nervous system. Retinal and nervous system involvement by angioma serpiginosum has, however, not previously been recorded. For this reason and also because of the unusual retinal abnormality in this patient, it is felt justifiable to report this single case, which may represent a new syndrome. Case reportA woman, born on March I3, 1929, was first admitted to the National Hospital, in July, I950, under the care of the late Dr. J. St C. Elkington. Her parents, four sisters, and three brothers were all alive and well and there was no family history of neurological illness or of cutaneous naevi. History At birth her skin was normal, but at the age of I year some angiomatous lesions were noted on the right leg. At 5 years these were more extensive and at the age of 7 years they were treated by cautery.At age I7 the patient underwent an appendicectomy and the right pupil was reported to be larger than the left. At the age of 21 years (I 950) she suddenly became aware of difficulty in elevating her right arm and over the next few months noticed clumsiness in the execution of fine movements with the right hand.In the following years, the right arm became progressively weaker with impaired sensation in the hand and in I962 she experienced attacks of burning pain in the upper arm lasting several hours. She also noted a sensation of fullness in the right orbit on bending down. The cutaneous angioma now involved the trunk, and new lesions appeared in the right axilla and on the adjacent medial aspect of the right arm.In I965 she first developed burning pain in the right side of the face, which persisted as did the attacks of pain in the right arm. The weakness of the right arm progressed and elevation became impossible without assistance. At about this time, she also began to experience attacks in which she would feel faint and occasionally fall to the ground. These episodes became more frequent, and occurred as often as four or five times a day, and though some were spontaneous others were precipitated by getting up suddenly or turning the head to the right.In I969, she developed nocturnal paraesthesiae in the left hand; the carpal-tunnel syndrome was diagnosed and the symptoms were relieved by surgical decompression. No change was noted in October, 1970, and she was still able to work as a hospital telephonist.The only ocular symptom reported has been of mild deterioration of vision in the right eye.

Punch biopsies of skin were taken from allogeneic marrow recipients routinely before transplantation, at 14-22 and 90-107 d after grafting and in the event of a clinical rash. Three histological appearances were encountered: graft versus host disease (GvHD), epidermal abnormalities, and normal. Graft versus host disease was characterized by epidermal basal vacuolation, spongiosis and individual cell necrosis associated with mononuclear cell infiltration of the upper dermis and lower epidermis, while epidermal abnormalities were identical to GvHD but without the mononuclear cell infiltrate. Graft versus host disease occurred only in patients receiving marrow unpurged of T-cells while epidermal abnormalities occurred with equal frequency in recipients of purged and unpurged marrow and were also noted in a high proportion of pre-transplant biopsies. Patients whose skin biopsies exhibited epidermal abnormalities showed no greater incidence of subsequent clinical or histological GvHD than those with normal biopsies. For these reasons, we conclude that epidermal abnormalities cannot be regarded as a minor manifestation of GvHD as has often been previously assumed. We also conclude that they cannot be regarded as the cause of a rash as, unlike GvHD, the incidence was not significantly different in patients with and without rashes. The cause of epidermal abnormalities is not entirely clear; cytotoxic drugs and irradiation appear to play a part but their occurrence in patients with previously normal post-transplant biopsies suggests that other factors may also be important. Some patients with strong clinical evidence of GvHD had negative biopsies; these should be regarded with caution especially within the first 24 h after the onset of a rash as the diagnostic histological picture may take time to develop. In some cases, GvHD was confined to pilosebaceous units; this seems to represent a minor form of the disease with only a limited capacity for progression. Dysplastic epidermal changes which have previously been attributed to the use of cyclosporin A were found with equal frequency in patients who did not receive this drug and must therefore have some other cause.