# Skinbase

##### Mouse Skin and Hair Database

## Adrenocortical Dysplasia (acd) Chr8

- A 10X photomicrograph from a DW/J-acd/acd one month old female mutant mouse. Note the very dark bulbs.
- A 25X photomicrograph from a 1 month old female DW/J-acd/acd mutant mouse illustrates the marked abnormal pigmentation in the dermal papilla of the bulb.
- A 25X photomicrograph from a DW/J-acd/acd mutant mouse exhibiting follicular dystrophy with moderate epidermal hyperplasia.
- A 25X photomicrograph of interfollicular epidermis from a 1 month old DW/J-acd/acd mutant mouse. Note the stellate-shaped pigmented cells in the epidermis.
- A 10X photomicrograph of skin from a DW/J 1 month old control female mouse in which the epidermis is of normal thickness for an adult and all follicles are in late anagen.

The adrenocortical dysplasia (acd) mouse mutation arose spontaneously in 1985 in the DW/J inbred strain at The Jackson Laboratory. Mice are runted at birth with a high incidence of polydactyly of the hind feet and tail kinks. The mice have heavily pigmented skin, which is very unusual. Pigmentation of mouse skin is not due to the epidermis but rather anagen stage hair follicles that produce melanin in the bulb that is injected into keratinocytes of the developing hair fiber. When follicles enter the resting phase, telogen, pigment is lost and the skin is pink in color. Since adult mice remain in telogen for long periods of time, shaved mouse skin is usually pink in color. The acd/acd mutant mice have unusually dark skin of the nonhaired regions including the muzzle, pinnae, feet, and tail. Vibrissae are often short and curled while body hair is sparse and abnormal. Hydronephrosis occurs secondary to urethral obstruction.
Microscopically, plucked hairs are abnormal with longitudinal fissures and cuticle changes. Zigzag hairs appear to be absent. Histologic evaluation of skin sections revealed interfollicular epidermal pigmentation in mutant but not control mice. Pigment ladened macrophages were present in the dermis of mutant but not control mice. There was moderate thickening of the mutant epidermis. The adrenal cortex of mutant mice was dramatically altered and very small while the size of the cells in this region were 7.5 times larger than those in control mice.
This mutant mouse has been considered as a potential model for hypoadrenalcorticism in humans and dogs. Nonscarring coat color alopecia in blue-coated Doberman Pinschers and other breeds with blue, fawn, or red coats may have similar pigmentation abnormalities with pigment extrusion into the follicular lumen.

### References

Austin, V., Blue dog disease, Mod. Vet. Pract., 1975; 56, 31
O’Neill, CS., Hereditary skin disease in the dog and the cat, Compend. Cont. Educ., 1981; 3, 791
Muller, GH; Kirk, RW; Scott, DW, Small Animal Dermatology, 3rd ed., W.B. Saunders, Philadelphia, 1983; 567
Sweet HO; Beamer WG; Bronson RT, Adrenocortical dysplasia (acd)., Mouse News Lett 1988;81():69
Beamer WG; Sweet HO; Bronson RT; Shire JG; Orth DN; Davisson MT, Adrenocortical dysplasia: a mouse model system for adrenocortical insufficiency., J Endocrinol 1994 Apr;141(1):33-43
Sundberg JP, Orlow SJ, Sweet HO, Beamer WG, The Adrenocortical Dysplasia (acd) Mutation, Chromosome 8, In: Handbook of Mouse Mutations with Skin and Hair Abnormalities: Animal Models and Biomedical Tools. Sundberg, JP (ed.), CRC Press, Boca Raton, FL, pp 159-164, 1994.
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