R.D.G. Peachey

High dose nicotinamide in the treatment of necrobiosis lipoidica

An open study of high dose nicotinamide in the treatment of 15 patients with necrobiosis lipoidica is reported. Of 13 patients who remained on treatment for more than 1 month, eight improved. Improvement took the form of a decrease in pain and soreness, a decrease in erythema and the healing of ulcers if present, although the skin did not return completely to normal in any patient. There were no significant side‐effects, particularly with respect to diabetic control, an important finding as lesions tended to relapse if treatment was stopped.

‘Fiddler's neck’

‘Fiddlers neck’ is a condition affecting violin and viola players. Although well known to musicians it is not well recognized by dermatologists. Clinically the lesions usually consist of a localized area of lichenification on the left side of the neck—just below the angle of the jaw. Pigmentation, erythema and inflammatory papules or pustules are frequently present, while severe inflammatory induration, cyst formation and scarring occur in more severely affected subjects. The aetiology of the skin changes is probably due to a combination of factors; friction giving rise to lichenification, while local pressure, shearing stress and occlusion may play a part in producing the acne‐like changes and cyst formation. In addition, poor hygiene may predispose to local sepsis.

Minocycline-induced discolouration of the sclerae

Summary We report two patients with minocycline‐induced pigmentation of the sclerae. Cutaneous pigtnentution is a well‐recognized complication of minocycline therapy, but only live cases of pigimentation of the sclerae have been described previously. These five patients have a number of features in common with the two reported here. We propose that these patients represent the most severe end of the spectrum of minocycline‐induced cutaneous changes. Patients should be warned about the possibility of the occurrence of pigmentary changes before starting therapy.

The treatment of psoriatic nail dystrophy with intradermal steroid injections

Thirty‐seven patients with psoriatic nail dystrophy were treated with intradermal nail‐fold injections of either triamcinolone acetonide or triamcinolone hexacetonide, using the Port‐0‐Jet needleless injector.

The scleroatrophic syndrome of Huriez

We have examined 14 of 28 members of a four‐generation family. 10 of whom demonstrated the clinical features of the scleroatrophic syndrome of Huriez, a cancer‐prone dermatosis. Several members of this family demonstrated additional features, previously unrecorded in this syndrome, including poikiloderma‐like changes on the nose, flexion contractures of the little finger, a distinctive little finger nodule, and telangiectasia on the lips. Genetic linkage was excluded to distal chromosome 4q (LOD score ‐ 4.399 at θ= 0.001). This concurs with the recent reappraisal study of one of the two original families described by Huriez, in which no evidence of linkage between this syndrome and the MNSs erythrocytic system (mapped to 4q28‐q31) was found. This is the first report of a family from the U.K. with this syndrome.

Speckled hyperpigmentation, palmo-plantar punctate keratoses and childhood blistering: a clinical triad, with variable associations.: A REPORT OF TWO FAMILIES

Patients from two unrelated families, one from Bristol (two individuals) and the other from Stoke‐on‐Trent (seven individuals), who exhibited the clinical triad of speckled hyperpigmentation, palmo‐plantar punctate keratoses and a blistering tendency particularly on hands and feet in infancy, are recorded. The disorder was inherited as an autosomal trait. The relationship of this clinical triad to other recently reported inherited conditions in which it has been encountered is discussed. A plea for a unified nomenclature is made.

Pili annulati coincident with alopecia areata

Abstract: Pili annulati is a rare hair shaft abnormality of unknown pathogenesis which gives a gross beading effect. An association with alopecia areata has been reported. We present a 13‐year‐old girl who had alopecia areata and coincidentally was found to have pili annulati. Her mother also had pili annulati which had become more noticeable as her hair had become paler with age. We discuss the possible pathogenesis of pili annulati and relate that to the microscopic banding appearance of the hair shaft. We note the relevance of the condition becoming more apparent with age. We also discuss the probable coincidental association with alopecia areata.

LEG ULCERATION AND HAEMOLYTIC ANAEMIA: AN HYPOTHESIS

Leg ulceration is a well recognized complication of sickle-cell anaemia, jS-thalassaemia and hereditary spherocytosis (Wintrobe, 1974), In addition, it has also been reported rarely in hereditary elliptocytosis (Hunter & Adams, 1929), pyruvate kinase deficiency (Tanaka & Paglia, 1971), a-thalassaemia (Daneshmend & Peachey, 1978), thrombocytopenic purpura (Witts, 1942) and in patients with splenomegaly due to other conditions (Gendel, 1948), but in some of these cases it is possible that the association may have been coincidental. With the exception of sickle-cell anaemia, the cause of the leg ulceration is poorly understood. The purpose of the present review is to discuss recent advances in our understanding of the three main conditions, and in the light of these findings to put forward an hypothesis to explain the associated leg ulceration. Chronic leg ulcers occur in between 50 and 75% of older children and adults with homozygous sickle-cell anaemia. The incidence in patients with combination haemoglobinopathies is lower. In Jamaica it has been shown that the sickle-cell trait is significantly commoner in patients with leg ulcers than would be expected from the incidence of the trait in the Negro population (Serjeant & Gueri, 1970), Occasional patients with sickle-cell trait develop ulceration in the presence of associated systemic disease, such as pneumonia (Wolfort & Krizek, 1969), Adult human haemoglobin (Hb A) consists of four sub-units—a pair of a and a pair of fi polypeptide chains (Braunitzer et al., 1964)—to each of which is attached a haem group. Haemoglobin S is formed as a result of the substitution of valine for glutamic acid at the sixth residue from the N terminal end of the p polypeptide chain of haemoglobin. Cells assume the sickle shape when the haemoglobin they contain is deprived of oxygen. This change may begin within ten seconds of deoxygenation, being favoured by a lowering of pH, Under these conditions, sickle haemoglobin gels and polymerizes to give rod-like structures consisting of six monomolecular strands of haemoglobin S twisted into a spiral. The rods aggregate into bundles to form tactoids, which lie parallel to the long axis of the cell or project into the projecting spicules (White & Heagan, 1973; White, 1974), It is believed that tactoid formation within the cell causes deformity of the cell membrane and the changes of sickling, but there is also evidence to suggest that the state of the cell membrane can influence the configuration assumed by the polymers (White & Heagan, 1973), Reversion to a normal shape occurs rapidly on re-oxygenation of the cell, but after repeated sickling, cells may lose their ability to revert to normal and remain irreversibly sickled. Even though the haemoglobin in these cells may be in tlje unpolymerized state, the cell membrane—due to ATP depletion—accumulates calcium and becomfes rigid and misshapen (Bertles & Dobler, 1969), Recent work has shown that erythroycte deformability is a major factor determining blood viscosity (Chien et al., 1967a), In sickle-cell anaemia deoxygenation causes a marked rise in whole blood viscosity. This occurs as a result of increase in the internal viscosity of the individual cells, while in addition the sickled cells are relatively rigid and unable to alter their shape (Chien, Usami & Bertles, 1970), These changes may cause a gross reduction of blood flow within certain capillary beds. If blood flow through the bed is rapid, the time of exposure of the erythrocyte to hypoxia may be too short for sickling to occur, but in capillary beds subjected to venous stasis the time factor and low oxygen tensions act to the detriment of the erythrocyte (Gabuzda, 1975), In the thalassaemic syndromes it is now known that there is a deficiency of one or other of the normal polypeptide chains, which combine to form the haemoglobin molecule. Thus in ^-thalassaemia

Leg ulcers in alpha‐thalassaemia (haemoglobin H disease)

A case of recurrent leg ulceration associated with α‐thalassaemia (haemoglobin H disease) is reported. It is suggested that leg ulcers occurring in the thalassaemic syndromes may be due to structural changes in the affected red cells which result in increased cell rigidity, decreased deformability and consequent diminished blood flow in capillary beds that are subjected to venous stasis.

(8) Limb hypertrophy: associated with a solitary plexiform neurofibroma?

89 Comment. Epithelioid haemangioma of bone is a rare vascular tumour. Most cases are multicentric and there is a predilection for the lower extremities. The multicentric cases seem to have a good prognosis and metastasis to extra-osseous sites is rare. Treatment is generally by curettage and/or radiation. The differential diagnosis on plain X-ray films is wide, including metastatic carcinoma (particularly from a renal primary), aneurysmal bone cyst, giant cell tumour, primary sarcoma of bone and brown tumour, depending on site and multiplicity. The MRI appearances have not been previously described.